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Sample records for anemia hemolytic autoimmune

  1. [Autoimmune hemolytic anemia in children].

    Science.gov (United States)

    Becheur, M; Bouslama, B; Slama, H; Toumi, N E H

    2015-01-01

    Autoimmune hemolytic anemia is a rare condition in children which differs from the adult form. It is defined by immune-mediated destruction of red blood cells caused by autoantibodies. Characteristics of the autoantibodies are responsible for the various clinical entities. Classifications of autoimmune hemolytic anemia include warm autoimmune hemolytic anemia, cold autoimmune hemolytic anemia, and paroxysmal cold hemoglobinuria. For each classification, this review discusses the epidemiology, etiology, clinical presentation, laboratory evaluation, and treatment options. PMID:26575109

  2. Autoimmune hemolytic anemia secondary to chicken pox

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    Abraham M Ittyachen

    2013-01-01

    Full Text Available Autoimmune hemolytic anemia (AIHA is a rare complication of chicken pox. It is described mainly in children. Even in children it is a rare complication and the long-term prognosis remains to be elucidated. Herein we report an adult, a 23-year-old male who developed AIHA secondary to chicken pox.

  3. Autoimmune hemolytic anemia secondary to chicken pox

    OpenAIRE

    Abraham M Ittyachen; Mohan B Jose; Varghese Abraham

    2013-01-01

    Autoimmune hemolytic anemia (AIHA) is a rare complication of chicken pox. It is described mainly in children. Even in children it is a rare complication and the long-term prognosis remains to be elucidated. Herein we report an adult, a 23-year-old male who developed AIHA secondary to chicken pox.

  4. Autoimmune hemolytic anemia: From lab to bedside

    OpenAIRE

    Chaudhary, R. K.; Sudipta Sekhar Das

    2014-01-01

    Autoimmune hemolytic anemia (AIHA) is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct ...

  5. Hemolytic anemia

    Science.gov (United States)

    ... Jager U, Lechner K. Autoimmune hemolytic anemia. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al, ... Price EA, Schrier SS. Extrinsic nonimmune hemolytic anemias. In: Hoffman R, Benz EJ Jr, Silberstein LE, et al, ...

  6. Immunotherapy Treatments of Warm Autoimmune Hemolytic Anemia

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    Bainan Liu

    2013-01-01

    Full Text Available Warm autoimmune hemolytic anemia (WAIHA is one of four clinical types of autoimmune hemolytic anemia (AIHA, with the characteristics of autoantibodies maximally active at body temperature. It produces a variable anemia—sometimes mild and sometimes severe. With respect to the absence or presence of an underlying condition, WAIHA is either idiopathic (primary or secondary, which determines the treatment strategies in practice. Conventional treatments include immune suppression with corticosteroids and, in some cases, splenectomy. In recent years, the number of clinical studies with monoclonal antibodies and immunosuppressants in the treatment of WAIHA increased as the knowledge of autoimmunity mechanisms extended. This thread of developing new tools of treating WAIHA is well exemplified with the success in using anti-CD20 monoclonal antibody, Rituximab. Following this success, other treatment methods based on the immune mechanisms of WAIHA have emerged. We reviewed these newly developed immunotherapy treatments here in order to provide the clinicians with more options in selecting the best therapy for patients with WAIHA, hoping to stimulate researchers to find more novel immunotherapy strategies.

  7. Acute Transient Variety of Autoimmune Hemolytic Anemia Following Varicella Infection

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    N. Parmar

    2015-06-01

    Full Text Available We are reporting a case of an 11 year female presenting with Acute Transient variety of Autoimmune hemolytic anemia following chickenpox, the patient was treated with blood transfusion and prednisolone and discharged with successful rise in hemoglobin.

  8. Elderly female with Autoimmune hemolytic anemia

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    Anupam Dey

    2015-01-01

    Full Text Available Autoimmune hemolytic anemia (AIHA is a rare disease with an estimated prevalence of around 17/100,000. It is often difficult to diagnose and treat AIHA, especially in elderly. A 60-year-old female was admitted with the complaints of low grade fever, on-off for 6 months, progressive fatigue and dyspnea on exertion. She was transfused with three units of blood within these 6 months. Examination revealed pallor, edema, hemic murmur, and palpable liver. Hb was 2.9 gm%, T Bil 5.2 mg/dl, ESR 160 mm, and reticulocyte count 44.05%. Direct Coombs test was positive, anti-nuclear antibody (ANA and Anti ds DNA were positive. A diagnosis of systemic lupus erythematosus (SLE with AIHA was considered and patient was transfused with two units of packed red cells and put on steroid (prednisolone at 1 mg/kg body weight daily. After 3 weeks, her Hb had increased to 10.4 gm% with gross clinical improvement.

  9. Autoimmune hemolytic anemia: From lab to bedside.

    Science.gov (United States)

    Chaudhary, R K; Das, Sudipta Sekhar

    2014-01-01

    Autoimmune hemolytic anemia (AIHA) is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT) still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The "best match" or "least incompatible units" can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue "best match" packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services. PMID:24678166

  10. Autoimmune hemolytic anemia: From lab to bedside

    Directory of Open Access Journals (Sweden)

    R K Chaudhary

    2014-01-01

    Full Text Available Autoimmune hemolytic anemia (AIHA is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The "best match" or "least incompatible units" can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue "best match" packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services.

  11. Pure red cell aplasia following autoimmune hemolytic anemia: An enigma

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    M Saha

    2013-01-01

    Full Text Available A 26-year-old previously healthy female presented with a 6-month history of anemia. The laboratory findings revealed hemolytic anemia and direct antiglobulin test was positive. With a diagnosis of autoimmune hemolytic anemia (AIHA, prednisolone was started but was ineffective after 1 month of therapy. A bone marrow trephine biopsy revealed pure red cell aplasia (PRCA showing severe erythroid hypoplasia. The case was considered PRCA following AIHA. This combination without clear underlying disease is rare. Human parvovirus B19 infection was not detected in the marrow aspirate during reticulocytopenia. The patient received azathioprine, and PRCA improved but significant hemolysis was once again documented with a high reticulocyte count. The short time interval between AIHA and PRCA phase suggested an increased possibility of the evolution of a single disease.

  12. Autoimmune hemolytic anemia in a patient with Malaria

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    Rajesh Sonani

    2013-01-01

    Full Text Available Autoimmune Hemolytic Anemia (AIHA, a very infrequent condition which represents a group of disorders in which presence of autoantibodies directed against self-antigens leads to shortened red cell survival. Till date, a very few cases of AIHA in Malaria patients are reported worldwide but still AIHA should be considered a relatively rare cause of anemia in malaria. A 20 year male presented with intermittent fever since seven days and yellowish discoloration of urine and sclera since 5 days. He was transfused three units of blood at a private clinic before one month. On examination, pallor, icterus and spelnomegaly were present. Hemoglobin (Hb was 3.2 gm% and peripheral smear revealed ring forms of both Plasmodium vivax and Plasmodium falciparum. Serum LDH and Serum billirubin (Indirect and Direct were high. This patient′s blood group was B +ve with positive autocontrol. Indirect Antiglobulin Test (IAT, antibody screening and antibody identification were pan-positive with reaction strength of +4 against each cell. Direct Antiglobulin Test was +4 positive anti IgG and negative with anti C3. He was treated with Artesunate and methylprednisone. Least incompatible, saline washed O Neg and B neg red cells were transfused on the 2 nd day of starting treatment. Hb was raised to 6.1 gm% on 4 th day. Patient was discharged on 9th day with Hb 7.0 gm% with oral tapering dose of steroids. In the above case, patient was suffering from high grade malarial parasitemia with co-existing autoimmune RBC destruction by IgG auto-antibodies which led to sudden drop in Hb and rise in serum LDH and indirect billirubin. Least incompatible packed red cells along with antimalarials and steroids led to clinical improvement. So far, one case report each from India, Korea, Canada and Germany and one case series report of three cases from India have been reported. Under-reporting or rarity of this phenomenon may be accountable for this.

  13. Types of Hemolytic Anemia

    Science.gov (United States)

    ... from the NHLBI on Twitter. Types of Hemolytic Anemia There are many types of hemolytic anemia. The ... the condition, but you develop it. Inherited Hemolytic Anemias With inherited hemolytic anemias, one or more of ...

  14. Unusual manifestations of acute Q fever: autoimmune hemolytic anemia and tubulointerstitial nephritis

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    Korkmaz Serdal

    2012-05-01

    Full Text Available Abstract Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs’ positive autoimmune hemolytic anemia (AIHA and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease.

  15. Reticuloendothelial cell function in autoimmune hemolytic anemia (AIHA): studies on the mechanism of peripheral monocyte activation.

    OpenAIRE

    Sunada,Mitsutoshi; Suzuki, Shinya; Ota, Zensuke

    1985-01-01

    We examined the activity of peripheral blood monocytes in patients with autoimmune hemolytic anemia (AIHA) using an in vitro assay of monocyte-macrophage interaction with erythrocytes and an antibody-dependent cell-mediated cytotoxicity (ADCC) assay. The monocytes of AIHA patients in the hemolyzing period phagocytized autologous sensitized red cells and anti-D coated red cells more avidly than normal control monocytes. There was no significant relationship between phagocytic activity and ADCC...

  16. Clinical features and outcomes of autoimmune hemolytic anemia: a retrospective analysis of 32 cases

    OpenAIRE

    Baek, Seung-Woo; Lee, Myung-Won; Ryu, Hae-Won; Lee, Kyu-Seop; SONG, IK-CHAN; Lee, Hyo-Jin; Yun, Hwan-Jung; Kim, Samyong; Jo, Deog-Yeon

    2011-01-01

    Background There has been no report on the clinical features or natural history of autoimmune hemolytic anemia (AIHA) in the Korean adult population. This study retrospectively analyzed the clinical characteristics and long-term outcomes of AIHA in the Korean adults. Methods Patients newly diagnosed with AIHA between January 1994 and December 2010 at Chungnam National University Hospital were enrolled. Patient characteristics at diagnosis, response to treatment, and the natural course of the ...

  17. Ulcerative Colitis, Autoimmune Hemolytic Anemia and Primary Sclerosing Cholangitis in a Child

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    Susan M Gilmour

    1996-01-01

    Full Text Available A 15-month-old female who initially presented with autoimmune hemolytic anemia (AIHA is described. She developed bloody stools and was diagnosed with ulcerative colitis (UC. Investigations of persistent hepatomegaly revealed primary sclerosing cholangitis (PSC. The association of AIHA, UC and PSC has never been reported. All these conditions entail impaired immunoregulation. Patients with a clustering of autoimmune diseases may help to delineate the pathogenesis of UC. Autoimmune phenomena may be prominent in inflammatory bowel disease. UC, in particular, exhibits a high incidence of associated autoimmune diseases including hypothyroidism, PSC, vitiligo and alopecia areata. AIHA is well described in 0.5% to 1.0% of adult UC patients but has not been reported in children with UC.

  18. A case of autoimmune hemolytic anemia with anti-D specificity in a one-year-old

    OpenAIRE

    Bercovitz, Rachel S.; Macy, Margaret; Ambruso, Daniel R.

    2013-01-01

    While antibodies to antigens in the Rh group are common causes of warm autoimmune hemolytic anemia, specificity for only the D-antigen is rare in autoimmune hemolysis in pediatric patients. This case reports an anti-D associated with severe hemolytic anemia (Hb = 2.1 g/dL) in a previously healthy 14-month-old who presented with a three-day history of low-grade fevers and vomiting. Because of his severe anemia, on admission to the hospital he was found to have altered mental status, metabolic ...

  19. Burkitt Lymphoma Preceded by Autoimmune Hemolytic Anemia due to Anti-D Antibody.

    Science.gov (United States)

    Mizuno, Yoshimi; Shimura, Yuji; Horiike, Shigeo; Takimoto, Tomoko; Maegawa, Saori; Tanba, Kazuna; Matsumura-Kimoto, Yayoi; Sumida, Yukari; Tatekawa, Shotaro; Tsukamoto, Taku; Chinen, Yoshiaki; Mizutani, Shinsuke; Nagoshi, Hisao; Yamamoto-Sugitani, Mio; Matsumoto, Yosuke; Kobayashi, Tsutomu; Kuroda, Junya; Taniwaki, Masafumi

    2016-01-01

    We herein report a rare case of Burkitt lymphoma (BL) preceded by autoimmune hemolytic anemia (AIHA) caused by autoantibodies against D antigen. After a partial response to AIHA with prednisolone (PSL) treatment for 7 months, the patient developed BL with a t(8;22)(q24;q11.2) chromosomal translocation. Intensive immunochemotherapy, including rituximab, led to a complete response (CR) of BL; however, anti-D antibody remained detectable in the plasma and antibody-dissociated solution from erythrocytes, thus continuous therapy with PSL was necessary even after achievement of the CR. BL with AIHA is extremely rare, with only one previously reported case in the literature. PMID:27523004

  20. Direct antiglobulin ("Coombs") test-negative autoimmune hemolytic anemia: a review.

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    Segel, George B; Lichtman, Marshall A

    2014-04-01

    We have reviewed the literature to identify and characterize reports of warm-antibody type, autoimmune hemolytic anemia in which the standard direct antiglobulin reaction was negative but a confirmatory test indicated that the red cells were opsonized with antibody. Three principal reasons account for the absence of a positive direct antiglobulin test in these cases: a) IgG sensitization below the threshold of detection by the commercial antiglobulin reagent, b) low affinity IgG, removed by preparatory washes not conducted at 4°C or at low ionic strength, and c) red cell sensitization by IgA alone, or rarely (monomeric) IgM alone, but not accompanied by complement fixation, and thus not detectable by a commercial antiglobulin reagent that contains anti-IgG and anti-C3. In cases in which the phenotype is compatible with warm-antibody type, autoimmune hemolytic anemia and the direct antiglobulin test is negative, an alternative method to detect low levels of IgG sensitization, use of 4°C, low ionic strength washes to prepare the cells for the direct antiglobulin test reaction to permit retention and identification of low affinity IgG antibodies, and, if the latter are uninformative, testing for sensitization with an anti-IgA, and, if necessary, an anti-IgM reagent identifies cases of warm-antibody type, immune hemolysis not verified by a commercial reagent.

  1. [Antiphospholipid syndrome with autoimmune hemolytic anemia which mimics thrombotic thrombocytopenic purpura].

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    Karasawa, Naoki; Taniguchi, Yasuhiro; Hidaka, Tomonori; Katayose, Keiko; Kameda, Takuro; Side, Kotaro; Shimoda, Haruko; Nagata, Kenji; Kubuki, Yoko; Matsunaga, Takuya; Shimoda, Kazuya

    2010-04-01

    A 67-year-old woman was admitted to the hospital for lethargy, fever, hemolytic anemia, thrombocytopenia, and consciousness disturbance. Direct Coombs test was positive, and anti-cardiolipin beta2-glycoprotein I antibody was detected. She was diagnosed with antiphospholipid syndrome complicated with autoimmune hemolytic anemia (AIHA). She demonstrated variable consciousness disturbance, inability to distinguish right from left, dysgraphia and dyscalculia. Multiple cerebral infarctions, especially dominant cerebral hemisphere infarctions, were observed on magnetic resonance imaging. A ventilation-perfusion scan demonstrated the presence of a ventilation-perfusion mismatch in both lung fields, and multiple veinous embolisms in the right femoral, bilateral the great saphenous and popliteal veins. Therefore, pulmonary embolism and thrombophlebitis were diagnosed. Based on these findings, it was necessary to distinguish this diagnosis from thrombotic thrombocytopenic purpura (TTP). As ADAMTS-13 activity was within the normal range, TTP was denied. Thereafter, the patient was treated with 1 mg/kg of prednisolone for AIHA, 3 mg of warfarin, and 3500 units of low-molecular-weight heparin for thrombosis, and her condition improved. PMID:20467225

  2. Anti B cell targeted therapy for autoimmune hemolytic anemia in an infant

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    Darshak Makadia

    2013-01-01

    Full Text Available Autoimmune hemolytic anemia (AIHA is an immune mediated destruction of erythrocytes, which has a good prognosis in children. It is known to have chronic, remitting or relapsing course, especially in infants and adolescents. Treatment of refractory or relapsing AIHA is a challenge as the other aim of the treatment is to avoid prolonged exposure to steroids or other immunosuppressants in small children. Rituximab is used in patients who are non-responsive to conventional treatment such as steroids, intravenous immunoglobulins and transfusion therapy. It has varying therapeutic success rate. We report a case of AIHA in a 4-month-old infant who had ill-sustained response to conventional therapy, but responded to rituximab.

  3. Treatment Options for Primary Autoimmune Hemolytic Anemia: A Short Comprehensive Review.

    Science.gov (United States)

    Salama, Abdulgabar

    2015-09-01

    Until now, treatment of primary autoimmune hemolytic anemia of the warm type (wAIHA) is primarily based on immunosuppression. However, many patients do not respond adequately to treatment, and treated patients may develop severe side effects due to uncontrolled, mixed and/or long-lasting immunosuppression. Unfortunately, the newly used therapeutic monoclonal antibodies are unspecific and remain frequently ineffective. Thus, development of a specific therapy for AIHA is necessary. The ideal therapy would be the identification and elimination of the causative origin of autoimmunization and/or the correction or reprogramming of the dysregulated immune components. Blood transfusion is the most rapidly effective measure for patients who develop or may develop hypoxic anemia. Although some effort has been made to guide physicians on how to adequately treat patients with AIHA, a number of individual aspects should be considered prior to treatment. Based on my serological and clinical experience and the analysis of evidence-based studies, we remain far from any optimized therapeutic measures for all AIHA patients. Today, the old standard therapy using controlled steroid administration, with or without azathioprine or cyclophosphamide, is, when complemented with erythropoiesis-stimulating agents, still the most effective therapy in wAIHA. Rituximab or other monoclonal antibodies may be used instead of splenectomy in therapy-refractory patients.

  4. Observação de anemia hemolítica auto-imune em artrite reumatóide Observation of autoimmune hemolytic anemia in rheumatoid arthritis

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    Ricardo A. S. Souza

    2003-01-01

    Full Text Available Artrite reumatóide é uma doença difusa do tecido conjuntivo que se caracteriza pelo acometimento articular e sistêmico. Disfunções hematológicas como anemia ocorrem em até 65% dos pacientes, sendo a anemia das doenças crônicas a forma mais comum. A anemia hemolítica auto-imune pode estar associada à difusa do tecido conjuntivo, sendo classicamente associada ao lúpus eritematoso sistêmico e fazendo parte dos seus critérios de classificação. A presença de anemia hemolítica auto-imune em artrite reumatóide é relatada raramente na literatura e os mecanismos etiopatogênicos para o seu desenvolvimento ainda não estão esclarecidos. Descrevemos um caso de artrite reumatóide no adulto e outro de artrite reumatóide juvenil que desenvolveram anemia hemolítica auto-imune e discutimos os prováveis mecanismos etiopatogênicos envolvidos.Rheumatoid arthritis is a connective tissue disease characterized by articular and systemic involvement. Hematological abnormalities such as anemia may occur in up to 65% of the patients, with chronic disease anemia being the commonest form. Autoimmune hemolytic anemia can be associated with different connective tissue diseases, particularly systemic lupus erythematosus and it is part of its classification criteria. On the other hand, the presence of autoimmune hemolytic anemia in rheumatoid arthritis has rarely been described in the literature and the pathogenic mechanisms for its development remain unclear. We describe here a case of rheumatoid arthritis and another of juvenile rheumatoid arthritis that developed to autoimmune hemolytic anemia and present the probable etiopathogenic mechanisms.

  5. Life-threatening autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura: successful seletive splenic artery embolization

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    matteo molica

    2016-04-01

    Full Text Available Selective splenic artery embolization (SSAE is a nonsurgical intervention characterized by the transcatheter occlusion of the splenic artery and/or its branch vessels using metallic coils or other embolic devices. It has been applied for the management of splenic trauma, hypersplenism with portal hypertension, hereditary spherocytosis, thalassemia and splenic hemangioma. We hereby describe a case of a patient affected by idiopathic thrombocytopenic purpura (ITP and warm auto-immune hemolytic anemia (AIHA both resistant to immunosuppressive and biological therapies, not eligible for a surgical intervention because of her critical conditions. She underwent SSAE and achieved a hematologic complete response within a few days without complications. SSAE is a minimally invasive procedure to date not considered a standard option in the management of AIHA and ITP. However, following the progressive improvement of the techniques, its indications have been extended, with a reduction in morbidity and mortality compared to splenectomy in patients with critical clinical conditions. SSAE was a lifesaving therapeutic approach for our patient and it may represent a real alternative for the treatment of resistant AIHA and ITP patients not eligible for splenectomy.

  6. Reticuloendothelial cell function in autoimmune hemolytic anemia (AIHA: studies on the mechanism of peripheral monocyte activation.

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    Sunada,Mitsutoshi

    1985-10-01

    Full Text Available We examined the activity of peripheral blood monocytes in patients with autoimmune hemolytic anemia (AIHA using an in vitro assay of monocyte-macrophage interaction with erythrocytes and an antibody-dependent cell-mediated cytotoxicity (ADCC assay. The monocytes of AIHA patients in the hemolyzing period phagocytized autologous sensitized red cells and anti-D coated red cells more avidly than normal control monocytes. There was no significant relationship between phagocytic activity and ADCC activity. The activated monocytes phagocytized autologous sensitized red cells, but had no ADCC activity in a short time 51Cr release assay. Phagocytic activity of the patients' monocytes against autologous erythrocytes rapidly decreased after treatment with prednisolone even though the red cell sensitization with antibody remained almost the same as during the hemolyzing period. We postulated that the activation of monocytes in AIHA was due to the "arming" effect of anti-erythrocyte antibody, but we think that other mechanisms may also be involved in the activation of monocytes.

  7. Clinical and serological characterization of autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation

    Institute of Scientific and Technical Information of China (English)

    Yang Zhen; Wu Bangzhao; Zhou Youning; Wang Wenjuan; Chen Suning; Sun Aining; Wu Depei

    2014-01-01

    Background Autoimmune hemolytic anemia (AIHA) is an uncommon complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) which has only been reported in a few cases.We here aimed to explore its mechanism.Methods We retrospectively analyzed 296 patients who underwent allo-HSCT in our center from July 2010 to July 2012.Clinical manifestations were carefully reviewed and the response to currently available treatment approaches were evaluated.The survival and risk factors of AIHA patients after allo-HSCT were further analyzed.Results Twelve patients were diagnosed with AIHA at a median time of 100 days (15-720 days) after allo-HSCT.The incidence of AIHA after allo-HSCT was 4.1%.IgG antibody were detected in ten patients and IgM antibody in two patients.The two cold antibody AIHA patients had a better response to steroid corticoid only treatment and the ten warm antibody AIHA patients responded to corticosteroid treatment and adjustment of immunosuppressant therapy.Rituximab was shown to be effective for AIHA patients who failed conventional therapy.Survival analysis showed that the combination of AIHA in allo-HSCT patients hinted at poor survival.Cytomegalovirus (CMV) infection,graft-versus-host disease (GVHD) and histocompatibility leukocyte antigen (HLA) mismatch seemed to increase the risk of developing AIHA.Conclusions Patients who develop AIHA after allo-HSCT have poor survival compared to non-AIHA patients.Possible risk factors of AIHA are CMV infection,GVHD,and HLA mismatch.Rituximab is likely to be the effective treatment choice for the refractory patients.

  8. Autoimmune Hemolytic Anemia Triggered by Infection with Human Parvovirus B19 after Total Abdominal Colectomy for Ulcerative Colitis.

    Science.gov (United States)

    Iida, Tomoya; Satoh, Shuji; Nakagaki, Suguru; Shimizu, Haruo; Kaneto, Hiroyuki

    2016-01-01

    A 50-year-old man was admitted to our hospital for an adhesive ileus 14 years after total abdominal colectomy for ulcerative colitis (UC). The ileus decreased with conservative treatment, however, autoimmune hemolytic anemia (AIHA) was diagnosed due to worsening anemia, a positive direct Coombs test, low haptoglobin, high lactase dehydrogenase, reticulocytosis, and an increase in the erythroblastic series in a bone-marrow examination. Human parvovirus B19 (PV-B19) IgM and PV-B19 DNA were present, indicating the development of AIHA triggered by an infection with PV-B19. The patient is currently being monitored after spontaneous remission. This is the first report of UC after total abdominal colectomy complicated by AIHA triggered by PV-B19 infection. PMID:26984090

  9. Hepatocellular carcinoma with chronic B-type hepatitis complicated by autoimmune hemolytic anemia: A case report

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    A 57-year-old man consulted a local hospital because of a persistent slight fever. At the age of 37 years he was diagnosed having B-type hepatitis, but left the liver dysfunction untreated. Twenty years later, he was diagnosed having chronic hepatitis B, hepatocellular carcinoma (HCC) and macrocytic anemia, and referred to our hospital for further investigation. A HCC with a maximum diameter of 5.2 cm was detected in segment 8. Results of blood tests included 1.8 mg/dL serum total bilirubin, 0.9 mg/dL bilirubin, less than 10 mg/dL haptoglobin, 7.9 g/dL hemoglobin, 130 fL MCV, and 14.5% reticulocytes. A bone marrow sample showed erythroid hyperplasia. The direct Coombs test gave a positive result. We diagnosed the anemia as autoimmmune hemolytic anemia (AIHA), for which prednisolone could not be administered due to positivity for HBsAg and HBeAg. After preparation of washed blood cells for later transfusion, the patient underwent systematic resection of segment 8. The cut surface of the resected specimen demonstrated an encapsulated yellow-brownish tumor measuring 52 mm × 40 mmwhich was diagnosed pathologicaly as moderately differentiated HCC. On the 9th postoperative day, the patient's temperature rose to 38℃, and exacerbated hemolysis was observed. The maximum total bilirubin value was 5.8 mg/dL and minimum hemoglobin level was 4.6 g/dL. He tolerated this period without blood transfusion. Currently he is being followed up as an outpatient, and shows no signs of HCC recurrence or symptoms of anemia. AIHA associated with HBV infection has been described in only three previous cases, and the present case is the first in which surgery was performed for accompanying HCC.

  10. IDIOPATHIC AUTOIMMUNE HEMOLYTIC ANEMIA PRESENTING AS MASSIVE SPLENOMEGALY IN AN ELDERLY MALE WHICH RESPONDED TO STEROID THERAPY

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    Deepak

    2014-07-01

    Full Text Available Autoimmune hemolytic anemia is uncommon. The estimated overall (not age-adjusted annual incidence is about 1 case per 100,000 populations; after age 60 years, the annual incidence reaches 10 per 100,000. The disorder can occur at any age, but most patients are older than 40 years. About 65% of patients with primary autoimmune hemolytic anemia are women, and almost all cases that complicate systemic lupus erythematosus occur in women. A 65 years old male presented with generalized weakness, breathlessness on exertion, swelling of lower limbs and pain abdomen of 6 months duration. He was previously admitted elsewhere on several occasions (within past 3-4 months with similar complaints, and had received multiple blood transfusions. On clinical examination, patient had pallor and bilateral pitting pedal edema. Abdominal examination revealed massive Splenomegaly (12 cm below left costal margin, moderate Hepatomegaly. Investigations revealed Hb% of 8.6 g/dl, Platelet count = 1 lakh/cmm, ESR = 120 mm, retic count -2.2 %. Peripheral smear showed evidence of hemolysis. Serum LDH was high, Serum bilirubin predominantly indirect hemoglobin= 2, S. Haptoglobin below 6.63. These findings suggested hemolysis as a cause of his anemia and splenomegaly. Further evaluation was done to find out the cause of hemolysis in this elderly male. Hb Quantification using HPLC was normal. Serum G6PD activity was normal. ANA was negative. Hams test was negative. Direct & Indirect Coomb’s tests were positive. Bone marrow examination showed erythroid hyperplasia. CT Abdomen showed hepatosplenomegaly. Upper GI endoscopy and colonoscopy were normal. Based on these findings a diagnosis of warm antibody type auto immune haemolytic anaemia (AIHA, probably of idiopathic type, was made and patient was started on steroid therapy. After 2 weeks, repeat haemoglobin was 12.8 gm%, WBC count was 7020/cumm reflecting response to steroid therapy. Severe AIHA can be a medical emergency. Red

  11. Complement in hemolytic anemia.

    Science.gov (United States)

    Brodsky, Robert A

    2015-01-01

    Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributable to mutations that lead to activation of the alternative pathway of complement. For optimal therapy, it is critical, but often difficult, to distinguish aHUS from other TMAs, such as thrombotic thrombocytopenic purpura; however, novel bioassays are being developed. In cold agglutinin disease (CAD), immunoglobulin M autoantibodies fix complement on the surface of red cells, resulting in extravascular hemolysis by the reticuloendothelial system. Drugs that inhibit complement activation are increasingly being used to treat these diseases. This article discusses the pathophysiology, diagnosis, and therapy for PNH, aHUS, and CAD.

  12. Curso clínico da anemia hemolítica auto-imune: um estudo descritivo Clinical course of autoimmune hemolytic anemia: an observational study

    Directory of Open Access Journals (Sweden)

    Maria Christina L. A. Oliveira

    2006-02-01

    Full Text Available OBJETIVO: A anemia hemolítica auto-imune é caracterizada pela produção de auto-anticorpos contra antígenos de superfície das hemácias. O objetivo do estudo foi identificar as características clínicas, imunológicas e evolutivas dos pacientes com anemia hemolítica auto-imune acompanhados no serviço de hematologia pediátrica do HC-UFMG e no Hemocentro de Belo Horizonte. MÉTODOS: Foram avaliadas 17 crianças menores de 15 anos, diagnosticadas entre 1988 e 2003. O diagnóstico de anemia hemolítica auto-imune foi baseado no quadro de hemólise adquirida e confirmado por meio do teste de Coombs direto poliespecífico. Os dados clínicos, demográficos, laboratoriais e referentes à evolução dos pacientes foram obtidos retrospectivamente nos prontuários médicos. RESULTADOS: A mediana de idade ao diagnóstico foi de 10,5 meses. O teste de Coombs direto poliespecífico foi positivo em 13 pacientes e negativo em quatro. Em 14 pacientes, foi realizado o teste de Coombs direto monoespecífico. Nestes, a classe de anticorpo mais freqüente foi IgG (cinco pacientes, seguida pela IgM em dois. Em 13 (76% pacientes, a anemia foi considerada grave, o que tornou necessária a hemotransfusão. Em quatro pacientes, foi identificada uma doença de base: lúpus eritematoso sistêmico, linfoma de Hodgkin, hepatite auto-imune e histiocitose de células de Langerhans. Os demais casos foram considerados como primários. A mediana de seguimento foi de 11 meses (5 a 23 meses. Ocorreram três óbitos, sendo dois após esplenectomia e um pela doença de base. CONCLUSÃO: A anemia hemolítica auto-imune é rara em crianças e adolescentes. Apesar de apresentar resposta ao corticóide e imunoglobulina, casos fatais têm sido relatados. O prognóstico é pior na presença de uma doença crônica de base.OBJECTIVE: Autoimmune hemolytic anemia is characterized by the production of autoantibodies against erythrocyte membrane antigens. This study was carried out to

  13. IgG4-related Lung Disease Associated with Autoimmune Hemolytic Anemia: A Case Report and a Literature Review.

    Science.gov (United States)

    Noguchi, Shingo; Yatera, Kazuhiro; Jinbo, Mitsutaka; Yamada, Sohsuke; Shimabukuro, Ikuko; Yamasaki, Kei; Kido, Takashi; Ishimoto, Hiroshi; Yoshii, Chiharu; Mukae, Hiroshi

    2016-01-01

    We herein report a case of IgG4-related lung disease (IgG4-RLD) associated with autoimmune hemolytic anemia (AIHA). A 73-year-old Japanese female visited our hospital for an examination following an abnormal chest X-ray in 1999. She was diagnosed with bronchiolitis and AIHA, and treatment with prednisolone was started. After seven years, she visited our department due to a cough. Chest computed tomography (CT) demonstrated focal consolidation with ground-glass attenuations and thickened bronchial walls in the bilateral lungs. She was clinically diagnosed and treated for bronchial asthma. CT findings had shown no changes, and a lung biopsy was performed using video-assisted thoracic surgery at eleven years from the first diagnosis of AIHA. The pathological findings demonstrated the presence of peribronchovascular lymphoplasmacytic infiltrates with stromal fibrotic changes, admixed with many IgG4-positive plasma cells. Furthermore, the patient's serum IgG4 level was high, and her CT findings did not show any obvious abnormal findings in the any organs other than the lungs. She was diagnosed with IgG4-RLD based on the findings. We believe that this case report of IgG4-RLD associated with AIHA is clinically helpful for a better understanding of these diseases, although there are five reported cases of IgG4-related disease associated with AIHA. PMID:27580552

  14. Prolonged extracorporeal membrane oxygenation therapy for severe acute respiratory distress syndrome in a child affected by rituximab-resistant autoimmune hemolytic anemia: a case report

    Directory of Open Access Journals (Sweden)

    Beretta Chiara

    2009-04-01

    Full Text Available Abstract Introduction Autoimmune hemolytic anemia in children younger than 2 years of age is usually characterized by a severe course, with a mortality rate of approximately 10%. The prolonged immunosuppression following specific treatment may be associated with a high risk of developing severe infections. Recently, the use of monoclonal antibodies (rituximab has allowed sustained remissions to be obtained in the majority of pediatric patients with refractory autoimmune hemolytic anemia. Case presentation We describe the case of an 8-month-old Caucasian girl affected by a severe form of autoimmune hemolytic anemia, which required continuous steroid treatment for 16 months. Thereafter, she received 4 weekly doses of rituximab (375 mg/m2/dose associated with steroid therapy, which was then tapered over the subsequent 2 weeks. One month after the last dose of rrituximab, she presented with recurrence of severe hemolysis and received two more doses of rrituximab. The patient remained in clinical remission for 7 months, before presenting with a further relapse. An alternative heavy immunosuppressive therapy was administered combining cyclophosphamide 10 mg/kg/day for 10 days with methylprednisolone 40 mg/kg/day for 5 days, which was then tapered down over 3 weeks. While still on steroid therapy, the patient developed an interstitial pneumonia with Acute Respiratory Distress Syndrome, which required immediate admission to the intensive care unit where extracorporeal membrane oxygenation therapy was administered continuously for 37 days. At 16-month follow-up, the patient is alive and in good clinical condition, with no organ dysfunction, free from any immunosuppressive treatment and with a normal Hb level. Conclusions This case shows that aggressive combined immunosuppressive therapy may lead to a sustained complete remission in children with refractory autoimmune hemolytic anemia. However, the severe life-threatening complication presented by our

  15. Drug-induced immune hemolytic anemia

    Science.gov (United States)

    Immune hemolytic anemia secondary to drugs; Anemia - immune hemolytic - secondary to drugs ... In some cases, a drug can cause the immune system to mistake your own red blood cells for foreign substances. The body responds by making ...

  16. [Hemolytic anemias and vitamin B12 deficieny].

    Science.gov (United States)

    Dietzfelbinger, Hermann; Hubmann, Max

    2015-08-01

    Hemolytic anemias consist of corpuscular, immun-hemolytic and toxic hemolytic anemias. Within the group of corpuscular hemolytic anemias, except for the paroxysmal nocturnal hemoglobinuria (PNH), all symptoms are caused by underlying heredetiary disorders within the red blood cell membran (hereditary spherocytosis), deficiencies of red cell enzymes (G6PDH- and pyrovatkinase deficiency) or disorders in the hemoglobin molecule (thalassaemia and sickle cell disease). Immune-hemolytic anemias are acquired hemolytic anemias and hemolysis is caused by auto- or allo-antibodies which are directed against red blood cell antigens. They are classified as warm, cold, mixed type or drug-induced hemolytic anemia. Therapy consists of glucocorticoids and other immunsuppressive drugs. Pernicious anemia is the most important vitamin B12 deficiency disorder. Diagnosis relies on cobalamin deficiency and antibodies to intrinsic factor. The management should focus on a possibly life-long replacement treatment with cobalamin. PMID:26306021

  17. A CASE REPORT ON SICKLE CELL DISEASE WITH HEMOLYTIC ANEMIA, NEPHROTIC SYNDROME AND ACUTE CHEST SYNDROME

    OpenAIRE

    Putta; Yamini Devi

    2015-01-01

    Sickle cell disease is an autoimmune hemolytic anemia due to abnormal hemoglobin. Sickling of RBCs occur due to abnormal hemoglobin which leads to vaso - occlusive crisis. This disease manifests as hemolytic anemia, acute chest syndrome, stroke, ischemic leg ulcers and nephrotic syndrome. This patient presented with hemolytic anemia, nephrotic syndrome and acute chest syndrome. This case was diagnosed by electrophoresis of h emoglobin and peripheral smear. Thi...

  18. Anemia hemolítica autoinmune en un niño con hepatitis de células gigantes Autoimmune hemolytic anemia in an infant with giant cell hepatitis

    Directory of Open Access Journals (Sweden)

    Jessica Gómez

    2012-12-01

    Full Text Available La asociación de anemia hemolítica autoinmune (AHAI con hepatitis de células gigantes (HCG es un trastorno raro en la infancia. Son pocos los casos reportados y la gran mayoría fallecen a pesar de transplante hepático. La AHAI usualmente precede el desarrollo de la afección hepática. El diagnóstico temprano de esta asociación y el inicio de terapia inmunosupresora previene la progresión de la enfermedad.Autoimmune hemolytic anemia (AIHA associated with giant cell hepatitis (GCH is a rare disorder in infants. Few cases have been reported and despite receiving a liver transplant, there is high mortality among patients. AIHA usually precedes the development of liver disease. Early recognition of this association and the administration of immunosuppressive therapy prevent progression of the disease. Keywords: Autoimmune hemolytic anemia, giant cell hepatitis La asociación de anemia hemolítica autoinmune (AHAI con hepatitis de células gigantes (HCG es un trastorno raro en la infancia. Son pocos los casos reportados y la gran mayoría fallecen por fallo hepático, a pesar de trasplante. La AHAI suele preceder el desarrollo de la afección hepática. El diagnóstico temprano de esta asociación y el inicio de terapia inmunosupresora evitan la progresión de la enfermedad. Se reporta un niño de 1 año de edad al que se le diagnosticó AHAI al mes y medio de edad y luego desarrolló HCG. Para su manejo, ameritó terapia inmunosupresora con corticoesteroides, inmunoglobulina intravenosa, anticuerpo monoclonal anti-CD20 (Rituximab y, además, plasmeferésis.

  19. Graves’ Disease Causing Pancytopenia and Autoimmune Hemolytic Anemia at Different Time Intervals: A Case Report and a Review of the Literature

    Directory of Open Access Journals (Sweden)

    Peyman Naji

    2013-01-01

    Full Text Available Graves’ disease (GD is associated with various hematologic abnormalities but pancytopenia and autoimmune hemolytic anemia (AIHA are reported very rarely. Herein, we report a patient with GD who had both of these rare complications at different time intervals, along with a review of the related literature. The patient was a 70-year-old man who, during a hospitalization, was also noted to have pancytopenia and elevated thyroid hormone levels. Complete hematologic workup was unremarkable and his pancytopenia was attributed to hyperthyroidism. He was started on methimazole but unfortunately did not return for followup and stopped methimazole after a few weeks. A year later, he presented with fatigue and weight loss. Labs showed hyperthyroidism and isolated anemia (hemoglobin 7 g/dL. He had positive direct Coombs test and elevated reticulocyte index. He was diagnosed with AIHA and started on glucocorticoids. GD was confirmed with elevated levels of thyroid stimulating immunoglobulins and thyroid uptake and scan. He was treated with methimazole and radioactive iodine ablation. His hemoglobin improved to 10.7 g/dL at discharge without blood transfusion. Graves’ disease should be considered in the differential diagnosis of hematologic abnormalities. These abnormalities in the setting of GD generally respond well to antithyroid treatment.

  20. Anemia hemolítica auto-imune e outras manifestações imunes da leucemia linfocítica crônica Autoimmune hemolytic anemia and other autoimmune diseases related to chronic lymphocytic leukemia

    Directory of Open Access Journals (Sweden)

    José O. Bordin

    2005-12-01

    Full Text Available A leucemia linfocítica crônica (LLC é freqüentemente associada a manifestações auto-imunes principalmente relacionadas às células do sistema hematopoético causando anemia hemolítica auto-imune (AHAI, púrpura trombocitopênica imune (PTI, aplasia pura de série vermelha (APSV, e neutropenia imune. A LLC é diagnosticada em até 15% dos pacientes com AHAI, e em cerca de 50% dos pacientes com AHAI secundária a doença maligna. A PTI ocorre em 2%, e a APSV em 1% dos pacientes com LLC. Prednisona é o tratamento inicial de escolha para a citopenia imune associada à LLC. Para cerca de 60% dos pacientes que apresentam recidiva da manifestação auto-imune tem sido utilizada esplenectomia, imunoglobulina endovenosa, ou ciclosporina. Embora as evidências sobre fisiopatologia sejam limitadas, os mecanismos fisiopatológicos da auto-imunidade na LLC estão relacionados à atividade dos linfócitos B leucêmicos que atuam como células apresentadoras de antígeno aberrantes, e são eficientes em processar e apresentar proteínas da membrana de hemácias e de plaquetas às células TH auto-reativas. Linfócitos TH específicos para certos auto-antígenos podem escapar de mecanismos de controle de auto-tolerância, e, se ativados, podem causar doença auto-imune. O diagnóstico de AHAI contra-indica o uso de fludarabina em pacientes com LLC, pois esse análogo da purina tem sido associado ao desenvolvimento de AHAI grave e fatal, com risco consideravelmente mais alto para pacientes mais imunossuprimidos devido a vários tratamentos anteriores.Chronic lymphocytic leukemia (CLL is frequently associated with autoimmune diseases directed against hematopoietic cells, including autoimmune hemolytic anemia (AIHA, immune thrombocytopenic purpura (ITP, pure red cell aplasia (PRCA, and immune neutropenia. CLL represents the diagnosis in up to 15% of the patients with AIHA, and in 50% of the patients with AIHA secondary to malignancy. ITP occurs in 2% and

  1. Autoimmune hemolytic anemia after allogeneic hematopoietic stem cell transplantation: analysis of 533 adult patients who underwent transplantation at King's College Hospital.

    Science.gov (United States)

    Wang, Meng; Wang, Wenjia; Abeywardane, Ayesha; Adikarama, Malinthi; McLornan, Donal; Raj, Kavita; de Lavallade, Hugues; Devereux, Stephen; Mufti, Ghulam J; Pagliuca, Antonio; Potter, Victoria T; Mijovic, Aleksandar

    2015-01-01

    Autoimmune hemolytic anemia (AIHA) is a recognized complication of hematopoietic stem cell transplantation (HSCT); it is often refractory to treatment and carries a high mortality. To improve understanding of the incidence, risk factors, and clinical outcome of post-transplantation AIHA, we analyzed 533 patients who received allogeneic HSCT, and we identified 19 cases of AIHA after HSCT (overall incidence, 3.6%). The median time to onset, from HSCT to AIHA, was 202 days. AIHA was associated with HSCT from unrelated donors (hazard ratio [HR], 5.28; 95% confidence interval [CI], 1.22 to 22.9; P = .026). In the majority (14 of 19; 74%) of AIHA patients, multiple agents for treatment were required, with only 9 of 19 (47%) patients achieving complete resolution of AIHA. Patients with post-transplantation AIHA had a higher overall mortality (HR, 2.48; 95% CI, 1.33 to 4.63; P = .004), with 36% (4 of 11 cases) of deaths attributable to AIHA. PMID:25262883

  2. Development of flow cytometry for detection and quantitation of red cell bound immunoglobulin G in autoimmune hemolytic anemia with negative direct Coombs test.

    Science.gov (United States)

    Thedsawad, Anchalee; Taka, Orathai; Wanachiwanawin, Wanchai

    2011-12-01

    About 2-10% of patients with warm-antibody autoimmune hemolytic anemia (WAIHA) exhibit a negative direct Coombs test (DAT), requiring more sensitive tests, including detection of RBC-bound immunoglobulins by flow cytometry, for diagnosis. In this study, the optimal conditions for detection and quantitation of RBC-bound IgG by flow cytometry were studied using blood samples from six patients with AIHA and two healthy individuals. Quantitation of RBC-bound IgG was performed using quantum simply cellular (QSC) beads coated with goat anti-mouse IgG antibodies. For detection of RBC bound IgG, a 60-minute incubation of all blood samples with 40 microl of 1:10 dilution of FITC-conjugated mouse anti-human IgG gave mean fluorescent intensity (MFI) values comparable to experiments using larger amounts or higher concentrations of the anti-human IgG. The acquired antibody binding capacity (ABC) values (or IgG molecules) for each QSC bead level, at 40 microl of 1:5 and 1:10 dilution of anti-human IgG for 60 minutes were close to the manufacturer-assigned ABC values. The IgG molecules per RBC in all six patients with positive DAT of 4+, 3+, 2+, 1+, trace and negative DAT were 31,725, 3,823, 1,753, 524, 260 and 88 respectively and in two healthy individuals with negative DAT they were 104 and 78. PMID:22299318

  3. Transfusiones en pacientes con pruebas de compatibilidad positivas y en aquellos con anemia hemolítica autoinmune Approach to transfusion support in patients with positive compatibility tests and in those with autoimmune hemolytic anemia

    Directory of Open Access Journals (Sweden)

    2007-12-01

    Full Text Available Es frecuente la presencia de pruebas de compatibilidad positivas en pacientes que requieren soporte transfusional. Los pacientes con anemia hemolítica autoinmune presentan autoanticuerpos calientes o fríos que reaccionan contra antígenos de los eritrocitos y producen reacciones hemolíticas, que pueden ir desde leves hasta graves. Los pacientes multitransfundidos o con insuficiencia renal crónica o con anemia de células falciformes, presentan aloanticuerpos que dificultan el tratamiento con derivados de la sangre. La frecuencia de los mismos es hasta de 32% en algunas series. Dado el perfeccionamiento de las técnicas de laboratorio, en la actualidad son posibles la detección y determinación de la especificidad de los autoanticuerpos y aloanticuerpos; el enfoque integral del paciente y el conocimiento de dichas técnicas son los mejores fundamentos para un soporte transfusional seguro. Positive compatibility tests in patients who need blood transfusions are common. Patients with autoimmune hemolytic anemia may have warm or cool autoantibodies that react with red blood cell antigens and produce hemolytic transfusion reactions. Multitransfused patients, and those with either chronic renal disease or sickle cell disease, have alloantibodies that make blood transfusion difficult. In some series the frequency of such antibodies is as high as 32%. Detection and determination of specificity of autoantibodies and alloantibodies are now possible because of technical developments in laboratory. An integral approach to patients and the knowledge of these procedures are the best bases to offer secure blood transfusions.

  4. Blood Group Discrepancy-First Sign of Autoimmune Hemolytic Anemia after Hematopoietic Stem Cell Transplantation in a Child.

    Science.gov (United States)

    Datta, Suvro Sankha; Reddy, Mahua; Basu, Sabita; Krishnan, Shekhar

    2016-06-01

    A 12-year-old male child was presented in the emergency with features of anemia and mild icterus on day+67 of HSCT. The child was suffering from Fanconi anemia and undergone HSCT from ABO-matched, fully HLA matched sibling donor. The diagnosis of mixed type AIHA due to cytomegalovirus reactivation was made in the immunohematology laboratory and blood group discrepancy was the first sign of AIHA in this patient. Though the cold agglutinin titer was not significant but the clinical symptoms and laboratory evidences were suggestive of significant hemolysis due to underlying IgG autoantibody. In addition the high complement avidity of IgM autoantibody might also be a contributing factor for clinically significant hemolysis in this case. The patient was successfully treated with phenotype matched blood transfusion, rituximab and oral steroid therapy. PMID:27408394

  5. How Is Hemolytic Anemia Treated?

    Science.gov (United States)

    ... medicines rituximab and cyclosporine. If you have severe sickle cell anemia , your doctor may recommend a medicine called hydroxyurea. ... hemoglobin that newborns have. In people who have sickle cell anemia, fetal hemoglobin helps prevent red blood cells from ...

  6. MA和AIHA患者LDH和RET%检测的临床意义%Detection and clinical significance of serum LDH level and reticulocyte percentage in the megaloblastic anemia and autoimmune hemolytic anemia patients

    Institute of Scientific and Technical Information of China (English)

    马丽娜; 李兴; 杨晓阳; 崔晓红; 吴和弟

    2012-01-01

    目的 通过检测巨幼细胞贫血(MA)和自身免疫性溶血性贫血(AIHA)患者血清乳酸脱氢酶(LDH)和网织红细胞百分比(RET%),探讨LDH和RET%在MA和AIHA诊断中的作用.方法 对40例MA组、30例AIHI组以及40例正常对照组LDH、RET%的水平变化作回顾性分析比较.结果 MA组、AIHI组血清LDH水平明显高于正常组,差异有统计学意义(均P<0.01);MA组LDH明显高于AIHI组,差异有统计学意义(P<0.01); AIHI组RET%明显高于MA组和正常组,差异有统计学意义(P<0.01);MA组RET%与正常组对比无差异(P>0.05).结论 血清LDH和RET%水平对临床鉴别MA和AIHA具有临床指导意义.%Objective In older to identify the clinical diagnosis significance of serum lactate dehydrogenase (LDH) and reticulocyte percentage (RET%)in patients of megaloblastic anemia (MA)and autoimmune hemolytic anemia (AIHA). Methods The serum lactate dehydrogenase(LDH)and reticulocyte percentage(RET%)levels of the 40 MA group,30 AIHI group and 40 normal group,were retrospectively compared and analyzed. Results LDH levels were significantly higher in the MA and AIHI group than the normal group's (P<0.0l). Meanwhile,the levels of LDH were also significantly different between the MA group and the AIHA group (P<0.0l). RET% levels were significantly higher in the AIHA group than the levels of MA and normal group (P<0.01) but RET% levels were not different between the MA group and the normal group (P>0.05). Conclusion The levels of Serum LDH and RET% are important for the clinical diagnosis of MA and AIHA patients.

  7. Immune Hemolytic Anemia in a Patient with Tuberculous Lymphadenitis

    OpenAIRE

    Manjunath Nandennavar; Sanju Cyriac; Krishnakumar; Sagar, T. G.

    2011-01-01

    Anemia in tuberculosis is usually anemia of chronic disease. Severe hemolytic anemia is exceedingly rare in tuberculosis patients. We report a patient diagnosed with tubercular lymphadenitis complicated by Coomb′s positive hemolytic anemia. Patient responded well to antituberculous treatment. Hematological parameters improved after initiation of antituberculosis treatment. To the best of our knowledge, this is the first case from India of an adult patient with tuberculous lymphadenitis presen...

  8. SEVERE IMMUNE HEMOLYTIC ANEMIA AFTER LIVER TRANSPLANTATION

    Directory of Open Access Journals (Sweden)

    A. I. Sushkov

    2013-01-01

    Full Text Available Clinical case of successful treatment of severe immune hemolytic anemia after liver transplantation is represen- ted in this article. The cause of complication was so-called passenger lymphocyte syndrome (a type of graft- versus-host disease. Two plasmapheresis sessions and Ig (0.5 g/kg in combination with increased maintenance immunosuppression with a short course of oral methylprednisolone in a total dose of 150 mg during 12 days were effective. The patient was discharged from hospital 34 days after transplantation in a satisfactory condition with a stable hemoglobin level. 

  9. A CASE REPORT ON SICKLE CELL DISEASE WITH HEMOLYTIC ANEMIA, NEPHROTIC SYNDROME AND ACUTE CHEST SYNDROME

    Directory of Open Access Journals (Sweden)

    Putta

    2015-03-01

    Full Text Available Sickle cell disease is an autoimmune hemolytic anemia due to abnormal hemoglobin. Sickling of RBCs occur due to abnormal hemoglobin which leads to vaso - occlusive crisis. This disease manifests as hemolytic anemia, acute chest syndrome, stroke, ischemic leg ulcers and nephrotic syndrome. This patient presented with hemolytic anemia, nephrotic syndrome and acute chest syndrome. This case was diagnosed by electrophoresis of h emoglobin and peripheral smear. This patient recovered with blood transfusion, antibiotics, steroids, diuretics and oxygen inhalation. Sickle cell patients have a known predisposition to bacterial infection, particularly pneumococcal infection. The most si gnificant advance in the therapy of sickle cell anemia has been the introduction of hydroxyurea, but hydroxyurea should be considered in patients experiencing repeated episodes of acute chest syndrome. But in this patient as this is first episode, hydroxyu rea was not given and he recovered well.

  10. Púrpura trombocitopênica e anemia hemolítica auto-imune em pacientes internados com lúpus eritematoso sistêmico juvenil Trombocytopenic purpura and autoimmune hemolytic anemia in hospitalized patients with juvenile systemic lupus erythematosus

    Directory of Open Access Journals (Sweden)

    Jochebed Kyoung Kim

    2007-02-01

    esplenectomia. CONCLUSÕES: o CHE isolado foi uma manifestação grave em pacientes internados com LESJ, habitualmente associado a uma doença ativa e sistêmica.OBJECTIVE: to evaluate the hematological involvement (HI in hospitalized patients with juvenile systemic lupus erythematosus (JSLE. METHODS: from 1994 to 2005, 195 admissions occurred in 77 JSLE patients (American College of Rheumatology criteria and were followed by the Pediatric Rheumatology Unit of the Instituto da Criança - University of São Paulo. These admissions were evaluated according to the presence of HI at onset or during the evolution of the disease: autoimmune hemolytic anemia (AHA or thrombocytopenic purpura. All patients performed at least two complete blood counts. AHA was defined by a fall in hemoglobin levels (beyond 2 g/dl, reticulocytosis, increase in lactate dehydrogenase (LDH and indirect bilirubin levels, and a positive Coombs test. The hematologic manifestations associated with infection, neoplasia and aplastic anemia were excluded. RESULTS: HI occurred in 14 patients (18.9%, with 15 admissions. Among these patients, 11 were female, 7 had trombocytopenic purpura, 5 AHA and 2 Evans syndrome. HI as onset and single manifestation of JSLE was observed in three patients. All the patients with trombocytopenic purpura presented cutaneous bleeding (petechia and/or ecchymosis. All had disease activity and simultaneously presented other manifestations of JSLE, particularly nephritis and vasculitis. Initially, all patients received pulsetherapy with methylprednisolone and prednisone later. In three patients the treatment aimed predominantly the control of hematologic manifestations, with intravenous gammaglobulin. The most used immunossupressive therapies were intravenous cyclophosphamide, cyclosporine and azathioprine. One patient died of central nervous system bleeding. No patient needed splenectomy. CONCLUSIONS: isolated HI was a severe manifestation in hospitalized patients with JSLE, generally

  11. Sirolimus for Autoimmune Disease of Blood Cells

    Science.gov (United States)

    2016-04-22

    Autoimmune Pancytopenia; Autoimmune Lymphoproliferative Syndrome (ALPS); Evans Syndrome; Idiopathic Thrombocytopenic Purpura; Anemia, Hemolytic, Autoimmune; Autoimmune Neutropenia; Lupus Erythematosus, Systemic; Inflammatory Bowel Disease; Rheumatoid Arthritis

  12. Autoimmune hemolytic anemia in HCV/HIV coinfected patients during treatment with pegylated alpha-2a interferon plus ribavirin Anemia hemolítica auto-imune durante o tratamento da hepatite crônica com interferon peguilado alfa 2a e ribavirina, em pacientes com co-infecção HCV/HIV

    Directory of Open Access Journals (Sweden)

    Fernando Crivelenti Vilar

    2007-12-01

    Full Text Available Two cases of autoimmune hemolytic anemia that occurred during the treatment of chronic hepatitis C with pegylated alpha-2a interferon and ribavirin, in HIV coinfected patients, are presented and described. The late occurrence (after six months of therapy of this severe hemolytic anemia leads to the recommendation that hemoglobin levels should be monitored throughout the treatment period, even among patients who presented stable hemoglobin levels in the preceding months.São apresentados e discutidos dois casos de anemia hemolítica auto-imune que ocorreram durante o tratamento da hepatite crônica pelo vírus C, com interferon peguilado alfa 2a e ribavirina, em pacientes co-infectados pelo HIV. A ocorrência de anemia hemolítica grave em etapa tardia, após o sexto mês da terapêutica, recomenda que o controle dos níveis de hemoglobina deva ser feito durante todo o período do tratamento , mesmo nos pacientes que apresentam níveis estáveis de hemoglobina nos meses precedentes.

  13. Hemolytic Anemia after Aortic Valve Replacement: a Case Report

    Directory of Open Access Journals (Sweden)

    Feridoun Sabzi

    2015-10-01

    Full Text Available Hemolytic anemia is exceedingly rare and an underestimated complication after aortic valve replacement (AVR.The mechanism responsible for hemolysis most commonly involves a regurgitated flow or jet that related to paravalvar leak or turbulence of subvalvar stenosis. It appears to be independent of its severity as assessed by echocardiography. We present a case of a 24-year-old man with a history of AVR in 10 year ago that developed severe hemolytic anemia due to a mild subvalvar stenosis caused by pannus formation and mild hypertrophic septum. After exclusion of other causes of hemolytic anemia and the lack of clinical and laboratory improvement, the patient underwent redo valve surgery with pannus and subvalvar hypertrophic septum resection. Anemia and heart failure symptoms gradually resolved after surgery

  14. Vitamin B12 and Vitamin D Deficiencies: An Unusual Cause of Fever, Severe Hemolytic Anemia and Thrombocytopenia

    Science.gov (United States)

    Mishra, Vikas A.; Harbada, Rishit; Sharma, Akhilesh

    2015-01-01

    The array of diagnostic workup for pyrexia of unknown origin (PUO) generally revolves in searching for infections, inflammatory/autoimmune, and endocrine etiologies. A differential diagnosis of fever, hemolytic anemia, and thrombocytopenia can have etiologies varying from infections like malaria, dengue, cytomegalovirus, Ebstein barr virus, Parvovirus, infective endocarditis, to autoimmune disorder (systemic lupus erythromatosis), vasculitis, hemolytic uremic syndrome, thrombotic thrombocytopenic purpura (TTP), autoimmune hemolytic anemia/Evan's syndrome, paroxysmal nocturnal hemoglobinuri (PNH), or drugs. Nutritional deficiencies (especially vitamin B12 deficiency) as a cause of fever, hemolytic anemia, and thrombocytopenia are very rare and therefore rarely thought of. Severe vitamin B12 deficiency may cause fever and if accompanied by concurrent hyper-homocysteinemia and hypophosphatemia can sometimes lead to severe hemolysis mimicking the above-mentioned conditions. We present a case that highlights vitamin B12 and vitamin D deficiency as an easily treatable cause of PUO, hemolytic anemia, and thrombocytopenia, which should be actively looked for and treated before proceeding with more complicated and expensive investigation or starting empiric treatments. PMID:25811010

  15. Adult patent ductus arteriosus complicated by endocarditis and hemolytic anemia.

    Science.gov (United States)

    Sabzi, Feridoun; Faraji, Reza

    2015-01-01

    An adult with a large patent ductus arteriosus may present with fatigue, dyspnea or palpitations or in rare presentation with endocarditis. The case illustrated unique role of vegetation of endocarditis in hemolytic anemia in adult with patent ductus arteriosus (PDA). Despite treatment of endocarditis with complete course of appropriate antibiotic therapy and normality of C- reactive protein, erythrocyte sedimentation rate and leukocytosis and wellness of general condition, transthoracic echocardiography revealed large vegetation in PDA lumen, surgical closure of PDA completely relieved hemolysis, and fragmented red cell disappeared from peripheral blood smear. The 3-month follow-up revealed complete occlusion of PDA and abolishment of hemolytic anemia confirmed by clinical and laboratory examination.

  16. Incipient Coombs' Test Negative Autoimmune Hemolytic Anemia Precedes Non-Hodgkin's Lymphoma%首发症状Coombs试验阴性自身免疫性溶血性贫血的非何杰金淋巴瘤

    Institute of Scientific and Technical Information of China (English)

    万岁桂; 林阳; 夏长青; 赵弘; 徐娟

    2012-01-01

    The cases of lymphoma accompanied or preceded by Coombs' test positive autoimmune hemolytic anemia (AIHA) have been reported.However,Coombs' test negative AIHA prior to the diagnosis of lymphoma was rarely described.Herein,this article reports a case of non-Hodgkin's lymphoma (NHL) preceded about 1.5 years by Coombs test negative AIHA.A woman aged 69 was diagnosed with HA based on the history and laboratory tests.Further studies revealed that this patient was negative with Coombs' test for IgG,IgM,IgA and C3.After all possible causes of HA,especially malignancies were ruled out,the patient was diagnosed with Coombs' test negative AIHA and treated with prednisolone.The patient responded well initially to steroid treatment.Two recurrences of acute HA were presented at time of 10 months post steroid cessation,and immediately after an attempt to withdraw steroid,respectively,but the hemolysis was effectively controlled by reinstitution of prednisolone.At third recurrence,however,the patient was no longer responding to steroid,and was found with cervical lymphadenopathy.Coombs' test for IgG,IgM,IgA and C3 remained negative.B cell NHL was diagnosed by pathology.After receiving 6 cycles of CHOP chemotherapy,the patient was lymphoma free,but the hemolysis was not improved,however,which was effectively controlled by the following low dose-rituximab(RTX) therapy.The patient was still kept in a remission of lymphoma free of anemia.In conclusion,this report presented a very rare case of NHL with Coombs' test negative AIHA as initial major clinical manifestation.%非何杰金淋巴瘤(NHL)伴发自身免疫性溶血性贫血(AIHA)或AIHA发生在NHL诊断之前或治疗过程中已有不少报道,但以Coombs试验阴性AIHA为首发症状的NHL鲜有报道.在此,本文报道1例1.5年反复溶血发作的Coombs试验阴性AIHA后合并NHL的患者.患者女性,69岁,根据病史和实验室检查诊断为Coombs试验阴性AIHA,给予强的松治疗后血红蛋白恢复正

  17. 抗β2糖蛋白Ⅰ抗体对自身免疫性溶血性贫血临床结局的影响%Effect of Anti-beta2 Glycoprotein I Antibodies on Outcome of the Patients with Autoimmune Hemolytic Anemia

    Institute of Scientific and Technical Information of China (English)

    曾晓虹; 薛原; 林臖芳; 郑玲; 康日辉

    2015-01-01

    Objective To assess the relationship of anti-beta2 glycoprotein I antibodies (anti-β2GPI)and the outcome of patients with autoimmune hemolytic anemia (AIHA).Methods During December 2008 to April 2013,consecutive case series of patients with AIHA were studied in the First affiliated hospital of Fujian Medical university.Patients were classified as Primary autoimmune hemolytic anemia (Primary AIHA Group)and autoimmune hemolytic anemia secondary to SLE (SLE-AIHA Group).Levels of IgG subtype anti-β2GPI were assessed in all cases.Data were analyzed by SPSS 11.5.Results A total of 42 patients with AIHA were enrolled in the study,22 cases were primary AIHA,while 20 cases were SLE-AIHA.In the SLE-AIHA Group,patients with positive IgG subtype anti-β2GPI,revealed longer time in achieving partial remission than patients without the antibody [(18 ±7)d vs.(13 ±4)d,P 0.05].Conclusion IgG subtype anti-β2GPI antibody may be one of the factors related to the prognosis of patients with systemic lupus erythematosus complicated with autoimmune hemolytic anemia.%目的:评估抗β2糖蛋白 I 抗体(anti β2 glycoprotein Ⅰ,aβ2GPI)与自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)临床结局的相关性。方法连续收集2008年12月至2013年4月在福建医科大学附属第一医院血液风湿科门诊和病房诊治的 AIHA 患者,包括继发于 SLE 的 AIHA 患者(SLE-AIHA 组)和原发性 AIHA 患者(原发性 AIHA 组)。检测所有患者 IgG 类抗β2GPI。采用 SPSS11.5软件统计分析。结果共纳入 AIHA 患者42例,SLE-AIHA 组22例,原发性AIHA 组20例。SLE-AIHA 组 IgG 类抗β2GPI 阳性患者溶血达部分缓解所需时间显著长于该抗体阴性患者,差异有统计学意义[(18±7)d vs.(13±4)d,P <0.05]。原发性 AIHA 组 IgG 类抗β2GPI 阳性组和阴性组患者溶血达部分缓解所需时间无统计学差异[(15±5)d vs.(11±3)d,P >0.05]。结论IgG 类抗β2

  18. Rare Occurrence of 3 "H": Hypercalcemia, Hemolytic Anemia and Hodgkin's Lymphoma.

    Science.gov (United States)

    Jain, Ankur; Malhotra, Pankaj; Prakash, Gaurav; Varma, Subhash; Kumar, Narender; Das, Asim

    2016-06-01

    Clinicians in hematology practice commonly encounter anemia, hypercalcemia and renal failure, which when present in combination evoke a diagnostic workup for multiple myeloma. We report a 71-years old lady who presented to our hematology clinic with fever and easy fatiguability of 3 months duration and on investigations was found to have anemia and hypercalcemia. Direct Coomb's test characterized the anemia as complement mediated (anti-C3d) hemolysis. Biochemical investigations revealed normal 25(OH) Vitamin D3 and suppressed Parathormone levels and a negative workup for plasma cell dyscrasias, sarcoidosis and autoimmune disorders. CT scan revealed a paravertebral mass with cervical, supraclavicular and abdominal lymphadenopathy along with splenomegaly and left pleural effusion. Biopsy from the paravertebral mass confirmed the diagnosis of Hodgkin's disease (nodular sclerosis) using immunohistochemistry. Bone marrow examination suggested infiltration by lymphoma. Hypercalcemia was managed with saline and zoledronic acid. Administration of prednisolone (1 mg/kg/day) along with chemotherapy (ABVD regimen) led to normalization of calcium and hemoglobin levels. However, hemolysis recurred 2 weeks later and hence, Rituximab (375 mg/m(2)) was administered on a weekly schedule for 4 doses and ABVD (2 weekly) was continued, which brought hemolysis under control. Co-occurrence of two paraneoplastic manifestations (complement mediated hemolytic anemia and hypercalcemia) in Hodgkin's lymphoma is very unusual. Present report aims not only to highlight a rare presentation of Hodgkin's lymphoma but also focus on the role of Rituximab in controlling hemolysis associated with this disease. PMID:27408382

  19. Diagnóstico laboratorial da anemia hemolítica auto-imune: características do teste manual direto do PolybreneÒ Laboratory diagnosis of auto-immune hemolytic anemia: characteristics of the manual direct test of PolybreneTM

    Directory of Open Access Journals (Sweden)

    G.W. Braga

    1998-03-01

    Full Text Available O teste manual direto do PolybreneÒ (TDP e o teste de Coombs direto (TCD foram utilizados para a detecção de IgG na superfície de hemácias de pacientes com diagnóstico clínico e laboratorial de anemia hemolítica auto-imune (AHAI. OBJETIVO: Comparar a sensibilidade e especificidade do TPD e do TCD no diagnóstico da AHAI. MÉTODO: Foram estudados 18 pacientes com diagnóstico clínico-laboratorial de AHAI. Como indivíduos controles, foram testados 20 doadores de sangue assintomáticos e 20 pacientes com anemia falciforme. RESULTADOS: O TCD foi positivo em 14 pacientes e negativo em quatro indivíduos, enquanto o TDP foi positivo em 17 pacientes e negativo em um indivíduo que apresentava TCD positivo devido a fixação de complemento (C3d nas hemácias. Todos os eluatos positivos realizados com a técnica de diclorometano revelaram anticorpos quentes com especificidade "anti-Rh". A sensibilidade do TDP (94% para detectar fixação de IgG in vivo foi significantemente maior (pThe direct manual PolybreneTM test (DPT and the direct antiglobulin tests (DAT were employed to detect antibody sensitizing red blood cell (RCB in patients with clinical and laboratorial findings of autoimmune hemolytic anemia (AIHA. PURPOSE: To compare the sensitivity and specificity of DPT and DAT in the diagnosis of AIHA. METHODS: Eighteen consecutive patients with diagnosis of AIHA were evaluated. The control group consisted of 20 normal volunteers blood donors and 20 patients with sickle cell anemia. All patients and controls were submitted to DPT and DAT. All DAT positive samples were further tested using monospecific reagents ( anti-IgG heavy chain and anti-C3d. Positive samples for either DPT or DAT were evaluated by eluate technique using. The dichloromethane (DCM. RESULTS: The DAT was positive in 14 patients and negative in 4 subjects, while the DPT was positive in 17 patients and negative in 1 individual who had a positive DAT owing to complement (C3d. All

  20. Hemolytic anemia as first presentation of Wilson's disease with uncommon ATP7B mutation.

    Science.gov (United States)

    Ye, Xing-Nong; Mao, Li-Ping; Lou, Yin-Jun; Tong, Hong-Yan

    2015-01-01

    Wilson's disease (WD) is a rare inherited disorder of copper metabolism and the main manifestations are liver and brain disorders. Hemolytic anemia is an unusual complication of WD. We describe a 15-year-old girl who developed hemolytic anemia as the first manifestation of Wilson's disease. An Arg952Lys mutation was found in exon 12 of the ATP7B gene, which is uncommon among Chinese Han individuals. From this case and reviews, we can achieve a better understanding of WD. Besides, we may conclude that the probable diagnosis of WD should be considered in young patients with unexplained hemolytic anemia, especially in patients with hepatic and/or neurologic disorder.

  1. Auto immune hemolytic anemia in a child precipitated by chicken pox.

    Science.gov (United States)

    Billoo, Samina Shamim; Jamalvi, Syed Waseem

    2008-05-01

    Auto Immune Hemolytic Anemia (AIHA) is a rare entity in children. We report a case of an adolescent girl with AIHA, which was precipitated by chicken pox. Clinical course over 3 years, till remission is described. PMID:18541094

  2. Hemolytic Anemia as a Presenting Feature of Wilson’s Disease: A Case Report

    OpenAIRE

    Sharma, Sunita; Toppo, Anupa; B Rath; Harbhajanka, Aparna; P Lalita Jyotsna

    2010-01-01

    Wilson’s disease is a rare inherited disorder of copper metabolism causing severe damage to vital organs. Liver and brain disorders are the main manifestations. Severe hemolytic anemia is an unusual complication of Wilson’s disease. We present a case who developed spherocytic acute hemolytic anemia (Coomb’s negative) as the initial manifestation of Wilson’s disease. On examination Kayser- Fleischer ring was found. Laboratory data supported a diagnosis of Wilson’s disease.

  3. Abordagem ambulatorial do nutricionista em anemia hemolítica Nutritional ambulatory approach in hemolytic anemia

    Directory of Open Access Journals (Sweden)

    Maria Aparecida Vieira

    1999-04-01

    Full Text Available Descreve a atuação do nutricionista em ambulatório de Hematologia Pediátrica em um hospital escola e relata as condutas dietéticas necessárias na abordagem de crianças com anemia hemolítica com e sem sobrecarga de ferro, e também as atitudes mais freqüentes dos familiares em relação à alimentação desses pacientes.The Authors describe the performance of the Dietitian in a Pediatric Hematology Ambulatory. They emphasize the necessary dietetic procedures for adequate management of children with hemolytic anemia, with and without iron overload. Furthermore, they approach the family's attitude towards the patient's nutrition.

  4. Neonatal Sulfhemoglobinemia and Hemolytic Anemia Associated With Intestinal Morganella morganii.

    Science.gov (United States)

    Murphy, Kiera; Ryan, Clodagh; Dempsey, Eugene M; O'Toole, Paul W; Ross, R Paul; Stanton, Catherine; Ryan, C Anthony

    2015-12-01

    Sulfhemoglobinemia is a rare disorder characterized by the presence of sulfhemoglobin in the blood. It is typically drug-induced and may cause hypoxia, end-organ damage, and death through oxygen deprivation. We present here a case of non-drug-induced sulfhemoglobinemia in a 7-day-old preterm infant complicated by hemolytic anemia. Microbiota compositional analysis of fecal samples to investigate the origin of hydrogen sulphide revealed the presence of Morganella morganii at a relative abundance of 38% of the total fecal microbiota at the time of diagnosis. M morganii was not detected in the fecal samples of 40 age-matched control preterm infants. M morganii is an opportunistic pathogen that can cause serious infection, particularly in immunocompromised hosts such as neonates. Strains of M morganii are capable of producing hydrogen sulphide, and virulence factors include the production of a diffusible α-hemolysin. The infant in this case survived intact through empirical oral and intravenous antibiotic therapy, probiotic administration, and red blood cell transfusions. This coincided with a reduction in the relative abundance of M morganii to 3%. Neonatologists should have a high index of suspicion for intestinal pathogens in cases of non-drug-induced sulfhemoglobinemia and consider empirical treatment of the intestinal microbiota in this potentially lethal condition.

  5. Hemolytic anemia following high dose intravenous immunoglobulin in patients with chronic neurological disorders

    DEFF Research Database (Denmark)

    Markvardsen, L H; Christiansen, Ingelise; Harbo, T;

    2014-01-01

    High dose intravenous immunoglobulin (IVIG) is an established treatment for various neuromuscular disorders. Recently, cases of hemolytic anemia following IVIG have been observed. The objective of this study was to determine the extent of anemia and hemolysis after IVIG and its relationship...

  6. Recurrent Isolated Neonatal Hemolytic Anemia: Think About Glutathione Synthetase Deficiency.

    Science.gov (United States)

    Signolet, Isabelle; Chenouard, Rachel; Oca, Florine; Barth, Magalie; Reynier, Pascal; Denis, Marie-Christine; Simard, Gilles

    2016-09-01

    Hemolytic anemia (HA) of the newborn should be considered in cases of rapidly developing, severe, or persistent hyperbilirubinemia. Several causes of corpuscular hemolysis have been described, among which red blood cell enzyme defects are of particular concern. We report a rare case of red blood cell enzyme defect in a male infant, who presented during his first months of life with recurrent and isolated neonatal hemolysis. All main causes were ruled out. At 6.5 months of age, the patient presented with gastroenteritis requiring hospitalization; fortuitously, urine organic acid chromatography revealed a large peak of 5-oxoproline. Before the association between HA and 5-oxoprolinuria was noted, glutathione synthetase deficiency was suspected and confirmed by a low glutathione synthetase concentration and a collapse of glutathione synthetase activity in erythrocytes. Moreover, molecular diagnosis revealed 2 mutations in the glutathione synthetase gene: a previously reported missense mutation (c.[656A>G]; p.[Asp219Gly]) and a mutation not yet described in the binding site of the enzyme (c.[902T>C]; p.[Leu301Pro]). However, 15 days later, a control sample revealed no signs of 5-oxoprolinuria and the clinical history discovered administration of acetaminophen in the 48 hours before hospitalization. Thus, in this patient, acetaminophen exposure allowed the diagnosis of a mild form of glutathione synthetase deficiency, characterized by isolated HA. Early diagnosis is important because treatment with bicarbonate, vitamins C and E, and elimination of trigger factors are recommended to improve long-term outcomes. Glutathione synthetase deficiency should be screened for in cases of unexplained newborn HA. PMID:27581854

  7. Congenital dyserythropoietic anemia type 1 with a novel mutation in the CDAN1 gene previously diagnosed as congenital hemolytic anemia.

    Science.gov (United States)

    Fujino, Hisanori; Doisaki, Sayoko; Park, Young-Dong; Hama, Asahito; Muramatsu, Hideki; Kojima, Seiji; Sumimoto, Shinichi

    2013-05-01

    The congenital dyserythropoietic anemias (CDAs) are a heterogeneous group of genetic disorders of red cell production. They are characterized by ineffective erythropoiesis and dyserythropoiesis. Here, we present the clinical description and mutation analysis of a Japanese female with CDA type 1. She has long been diagnosed with unclassified congenital hemolytic anemia from the neonatal period. However, bone marrow morphology and genetic testing of the CDAN1 gene at the age of 12 years confirmed the afore-mentioned diagnosis. Thus, we should be aware of the possibility of CDA if the etiology of congenital anemia or jaundice cannot be clearly elucidated.

  8. Histiocytic Sarcoma Associated with Coombs Negative Acute Hemolytic Anemia: A Rare Presentation

    Science.gov (United States)

    Martin, Stephen C.; Nassiri, Mehdi; Qureshi, Amna; Markel, Troy A.

    2016-01-01

    Histiocytic sarcoma (HS) rarely involves extranodal sites, such as the spleen. We report a unique pediatric case of massive splenomegaly and refractory Coombs negative hemolytic anemia (CNHA) secondary to HS. The CNHA resolved completely after an emergent splenectomy. Next generation sequencing (NGS) revealed novel ASXL1, PTPN11, KIT, and TP53 mutations, unmasking a clonal heterogeneity within the same neoplasm. PMID:27429816

  9. A vast retroperitoneal mass and autoimmune haemolytic anemia

    Directory of Open Access Journals (Sweden)

    Fabio Di Stefano

    2013-04-01

    Full Text Available CLINICAL CASE We report a 64 year-old patient with fatigue and intermittent fever. Laboratory investigations revealed autoimmune haemolytic anemia. An abdomen CT scan showed a retroperitoneal mass near the left kidney. The CT scan guided mass biopsy was performed and its histology was diagnostic for a non Hodgkin B cell lymphoma. CONCLUSIONS The case describes autoimmume haemolytic anemia as a paraneoplastic syndrome associated with lymphoma. Autoimmume haemolytic anemia is a frequent paraneoplastic syndrome of lymphoproliferative disorders. The onset can be concomitant to the diagnosis of leukemia/lymphoma or follows the course of these neoplastic diseases or less frequently occurs years in advance.

  10. CD4+CD25+调节性T细胞在自身免疫性溶血性贫血患者中的变化及意义%Change and significance of CD4+CD25+ regulatory T cells in patients with autoimmune hemolytic anemia

    Institute of Scientific and Technical Information of China (English)

    黄东平; 何合胜

    2014-01-01

    Objective To investigate the change of regulatory T-cells (Treg) before and after therapy in patients with autoimmune hemolytic anemia (AIHA),and to study the role of Treg in AIHA.Methods Treg cells numbers was measured by flow cytometry.Results Before treatment,Treg cells in AIHA patients was (1.32 ± 0.87) %,which was significantly lower than (3.08 ± 0.96) % in the controls (t =-5.37,P < 0.01).After treatment,Treg cells in AIHA patients was significantly increased [(4.96 ± 1.13)%] (t =-16.94,P <0.01).Conclusion Treg cells decreased in AIHA patients.Glucocorticoid might play a role in AIHA treatment by up-regulating Treg cells number.%目的 探讨CD4+CD25+Foxp3+调节性T细胞(Treg)在自身免疫性溶血性贫血(AIHA)患者中的变化.方法 应用流式细胞术检测AIHA患者治疗前后外周血Treg的数量变化.结果 AIHA患者治疗前外周血中Treg细胞比例为(1.32±0.87)%,明显低于对照组(3.08±0.96)%(t=-5.37,P<0.01).治疗后Treg细胞比例为(4.96±1.13)%,明显高于治疗前(t=-16.94,P<0.01),且高于对照组(t=-4.96,P<0.01).结论 AIHA患者中Treg细胞数量下降,糖皮质激素通过上调Treg细胞数量可以控制自身免疫性溶血性贫血.

  11. A thermolabile aldolase A mutant causes fever-induced recurrent rhabdomyolysis without hemolytic anemia.

    Science.gov (United States)

    Mamoune, Asmaa; Bahuau, Michel; Hamel, Yamina; Serre, Valérie; Pelosi, Michele; Habarou, Florence; Nguyen Morel, Marie-Ange; Boisson, Bertrand; Vergnaud, Sabrina; Viou, Mai Thao; Nonnenmacher, Luc; Piraud, Monique; Nusbaum, Patrick; Vamecq, Joseph; Romero, Norma; Ottolenghi, Chris; Casanova, Jean-Laurent; de Lonlay, Pascale

    2014-11-01

    Aldolase A deficiency has been reported as a rare cause of hemolytic anemia occasionally associated with myopathy. We identified a deleterious homozygous mutation in the ALDOA gene in 3 siblings with episodic rhabdomyolysis without hemolytic anemia. Myoglobinuria was always triggered by febrile illnesses. We show that the underlying mechanism involves an exacerbation of aldolase A deficiency at high temperatures that affected myoblasts but not erythrocytes. The aldolase A deficiency was rescued by arginine supplementation in vitro but not by glycerol, betaine or benzylhydantoin, three other known chaperones, suggesting that arginine-mediated rescue operated by a mechanism other than protein chaperoning. Lipid droplets accumulated in patient myoblasts relative to control and this was increased by cytokines, and reduced by dexamethasone. Our results expand the clinical spectrum of aldolase A deficiency to isolated temperature-dependent rhabdomyolysis, and suggest that thermolability may be tissue specific. We also propose a treatment for this severe disease.

  12. A thermolabile aldolase A mutant causes fever-induced recurrent rhabdomyolysis without hemolytic anemia.

    Directory of Open Access Journals (Sweden)

    Asmaa Mamoune

    2014-11-01

    Full Text Available Aldolase A deficiency has been reported as a rare cause of hemolytic anemia occasionally associated with myopathy. We identified a deleterious homozygous mutation in the ALDOA gene in 3 siblings with episodic rhabdomyolysis without hemolytic anemia. Myoglobinuria was always triggered by febrile illnesses. We show that the underlying mechanism involves an exacerbation of aldolase A deficiency at high temperatures that affected myoblasts but not erythrocytes. The aldolase A deficiency was rescued by arginine supplementation in vitro but not by glycerol, betaine or benzylhydantoin, three other known chaperones, suggesting that arginine-mediated rescue operated by a mechanism other than protein chaperoning. Lipid droplets accumulated in patient myoblasts relative to control and this was increased by cytokines, and reduced by dexamethasone. Our results expand the clinical spectrum of aldolase A deficiency to isolated temperature-dependent rhabdomyolysis, and suggest that thermolability may be tissue specific. We also propose a treatment for this severe disease.

  13. Rituximab for the Treatment of Refractory Autoimmune Hemolytic Anemia in Children: Report of Two Cases and Literature Review%利妥昔单抗治疗儿童难治性自身免疫性溶血性贫血2例并文献复习

    Institute of Scientific and Technical Information of China (English)

    温贤浩; 管贤敏; 于洁; 宪莹

    2013-01-01

    目的:探讨利妥昔单抗治疗儿童难治性自身免疫性溶血性贫血(AIHA)的疗效、毒副作用.方法:(1)总结2例难治性A1HA患儿的临床表现、实验室检查结果及使用利妥昔单抗治疗前后的情况.(2)对2例患儿选用利妥昔单抗治疗(每次375 mg/m2,每周1次,共4次),随访利妥昔单抗使用后CD20+细胞、血红蛋白等的变化情况,探讨利妥昔单抗治疗儿童AIHA的疗效;同时观察发热、皮疹、感染等毒副作用.结果:2例患儿诊断AIHA后均首先选用糖皮质激素治疗,初期都能显效,但不能长期维持,需长期依赖激素及输血.使用利妥昔单抗治疗后,2例患儿均能显效,其中1例治愈,1例病情反复,最终死于严重贫血.结论:尽管糖皮质激素是治疗AIHA的一线药物,但激素依赖或难治性病例较常见,因此利妥昔单抗已逐渐成为儿童难治性AIHA安全有效的二线治疗药物.%Objective: To investigate the efficacy and side effect of rituximab in the treatment of refractory autoimmune hemolytic anemia (AIHA) in children. Methods: (1) Two cases of refractory AIHA were selected, discussion on the efficacy of glucocorticoid therapy in children with AIHA. Clinical manifestation, laboratory results and therapies before treated with rituximab of the two patients were summarized. (2) Rituximab was given at a dose of 375 mg/(m2 ? w) for four doses. To observe the changes of CD20* cell, hemoglobin, et al. And to investigate the efficacy of rituxumabin the treatment of refractory AIHA. Meanwhile, the side effect was regarded, such as fever, rash, infection, and so on. Results; Glucocorticoid was used as the first line therapy in the two patients who were diagnosed with AIHA. It was effective in the beginning, but it could not lead to maintenance. The two patients in our study needed persistently using steroids and transfusion to maintain the hemoglobin. The effect of rituximab was affirmed in the two cases. One patient was cured, and

  14. [Hemolytic autoimmune anemia caused by (+)-cyanidanol-3 (Catergen)].

    Science.gov (United States)

    Neftel, K; Diem, P; Gerber, H; de Weck, A L; Stucki, P

    1980-03-01

    Several episodes of acute intravascular immune hemolysis in a 68 year old patient induced by (+)-cyanidanol-3 (Catergan) are reported. Clinical and serological criteria of the so-called immune-complex mechanism of immune drug-induced hemolysis were realised. The meaning of the additional marked affinity of the drug to the erythrocytic surface remains unclear. The patient's serum was cross-reactive with rutine but not with troxerutine.

  15. [Infantile pyknocytosis: A cause of noenatal hemolytic anemia. Is recombinant erythropoietin an alternative to transfusion?].

    Science.gov (United States)

    Bagou, M; Rolland, E; Gay, C; Patural, H

    2016-01-01

    Infantile pyknocytosis is a neonatal hemolytic disorder which causes anemia and icterus and is characterized by the presence of an increased number of distorted red blood cells called pyknocytes. Resolution spontaneously occurs in the first semester of life. It has been generally described as a rare entity, with an occasional family history. We report seven cases of infantile pyknocytosis observed in our hospital in 3 years. Most of the infants presented with hemolytic icterus and profound anemia that was reaching its peak by the 3rd week of life. Three neonates received one to three red blood cell transfusions, according to former recommendations. However, the following four received a treatment with recombinant erythropoietin administered subcutaneously. Only one of these four cases required a transfusion. All of them were free of hematological disease 2-3 months after completion of treatment. Infantile pyknocytosis is a recognized cause of neonatal hemolytic anemia, which requires careful examination of red cell morphology on a peripheral blood smear. The cause of this transient disorder remains unknown. Our observations show that recombinant erythropoietin therapy is effective in treating infantile pyknocytosis and increases the reticulocyte response, thus improving the hemoglobin level. PMID:26563723

  16. Zinc-induced hemolytic anemia caused by ingestion of pennies by a pup

    International Nuclear Information System (INIS)

    A 4-month-old Pomeranian pup was examined because of anorexia, salivation, and persistent vomiting. Initial laboratory testing revealed marked hemolytic anemia with spherocytosis. Survey abdominal radiography revealed 4 metal objects which, when removed by gastrotomy, were identified as pennies. Of 4 pennies, 3 were minted since 1983 and were heavily pitted over the surface and rim. Partially digested pennies were composed of a copper-plated high zinc concentration alloy. Further laboratory testing indicated a marked increase in serum zinc concentration in the pup (28.8 mg/L), confirming metal toxicosis. Serum zinc concentrations decreased during recovery

  17. Piperacillin-Induced Immune Hemolytic Anemia in an Adult with Cystic Fibrosis

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    Mahesh Bandara

    2010-01-01

    Full Text Available We report a case of drug-induced immune hemolytic anemia (DIIHA in an adult female with cystic fibrosis (CF, complicating routine treatment of a pulmonary exacerbation with intravenous piperacillin-tazobactam. Workup revealed a positive direct antiglobulin test (DAT due to red blood cell (RBC-bound IgG and C3 and piperacillin antibodies detectable in the patient's serum. The potential influence of CF transmembrane conductance regulator mutations on the severity of DIIHA is discussed. This report illustrates the importance of early identification of DIIHA, a rare complication of a commonly utilized medication in CF.

  18. A rare case of concurrent signet-ring carcinoma of breast and microangiopathic hemolytic anemia.

    Science.gov (United States)

    Lara, Kelly; Bae, Esther; Park, Hanna; Hussain, Farabi

    2016-01-01

    Microangiopathic hemolytic anemia (MAHA) can be an uncommon presentation of an underlying malignancy, most often due to signet-ring cell carcinoma (SRCC). Additionally, pure SRCC in a breast primary-tumor comprises anemia as the first manifestation of metastatic signet-ring cell carcinoma of unknown origin: a case report and review of literature. Kor J Lab Med 2011;31:157-61). To the best of our knowledge, the combination of these two entities, pure breast primary SRCC along with MAHA, has not been reported. Here, we present such a rare case. We also evaluate the current literature regarding this and similar disease processes, of which evidence is scarce and further research is needed. PMID:27587305

  19. Maternal anti-M induced hemolytic disease of newborn followed by prolonged anemia in newborn twins

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    Satyam Arora

    2015-01-01

    Full Text Available Allo-anti-M often has an immunoglobulin G (IgG component but is rarely clinically significant. We report a case of hemolytic disease of the fetus and newborn along with prolonged anemia in newborn twins that persisted for up to 70 days postbirth. The aim was to diagnose and successfully manage hemolytic disease of newborn (HDN due to maternal alloimmunization. Direct antiglobulin test (DAT, antigen typing, irregular antibody screening and identification were done by polyspecific antihuman globulin cards and standard tube method. At presentation, the newborn twins (T1, T2 had HDN with resultant low reticulocyte count and prolonged anemia, which continued for up to 70 days of life. Blood group of the twins and the mother was O RhD positive. DAT of the both newborns at birth was negative. Anti-M was detected in mothers as well as newborns. Type of antibody in mother was IgG and IgM type whereas in twins it was IgG type only. M antigen negative blood was transfused thrice to twin-1 and twice to twin-2. Recurring reduction of the hematocrit along with low reticulocyte count and normal other cell line indicated a pure red cell aplastic state. Anti-M is capable of causing HDN as well as prolonged anemia (red cell aplasia due to its ability to destroy the erythroid precursor cells. Newborns with anemia should be evaluated for all the possible causes to establish a diagnosis and its efficient management. Mother should be closely monitored for future pregnancies as well.

  20. Therapeutic complement inhibition in complement-mediated hemolytic anemias: Past, present and future.

    Science.gov (United States)

    Risitano, Antonio M; Marotta, Serena

    2016-06-01

    The introduction in the clinic of anti-complement agents represented a major achievement which gave to physicians a novel etiologic treatment for different human diseases. Indeed, the first anti-complement agent eculizumab has changed the treatment paradigm of paroxysmal nocturnal hemoglobinuria (PNH), dramatically impacting its severe clinical course. In addition, eculizumab is the first agent approved for atypical Hemolytic Uremic Syndrome (aHUS), a life-threatening inherited thrombotic microangiopathy. Nevertheless, such remarkable milestone in medicine has brought to the fore additional challenges for the scientific community. Indeed, the list of complement-mediated anemias is not limited to PNH and aHUS, and other human diseases can be considered for anti-complement treatment. They include other thrombotic microangiopathies, as well as some antibody-mediated hemolytic anemias. Furthermore, more than ten years of experience with eculizumab led to a better understanding of the individual steps of the complement cascade involved in the pathophysiology of different human diseases. Based on this, new unmet clinical needs are emerging; a number of different strategies are currently under development to improve current anti-complement treatment, trying to address these specific clinical needs. They include: (i) alternative anti-C5 agents, which may improve the heaviness of eculizumab treatment; (ii) broad-spectrum anti-C3 agents, which may improve the efficacy of anti-C5 treatment by intercepting the complement cascade upstream (i.e., preventing C3-mediated extravascular hemolysis in PNH); (iii) targeted inhibitors of selective complement activating pathways, which may prevent early pathogenic events of specific human diseases (e.g., anti-classical pathway for antibody-mediated anemias, or anti-alternative pathway for PNH and aHUS). Here we briefly summarize the status of art of current and future complement inhibition for different complement-mediated anemias

  1. [Heme oxygenase induction in rat heart and vessels and peroxidative resistance of erythrocytes during hemolytic anemia development].

    Science.gov (United States)

    Kaliman, P A; Pavychenko, O V

    2005-01-01

    The hemolytic anemia development caused by phenylhydrazine injection (7 mg/100 g b.w.) was shown to be caused by the decreasing of both catalase activity and glutathione content in erythrocytes, and by the increasing of spontaneouse hemolysis level of these cells in blood stream. The increasing of heme oxygenase activity and TBA-active products in rat heart and vessels were revealed 24 hrs after phenylhydrazine injection. Possible mechanisms of heme oxygenase-1 induction under hypoxia as response to the hemolytic anemia development and it's role in defense of the cells from damage are discussed. PMID:16329389

  2. Complement inhibitors to treat IgM-mediated autoimmune hemolysis.

    Science.gov (United States)

    Wouters, Diana; Zeerleder, Sacha

    2015-11-01

    Complement activation in autoimmune hemolytic anemia may exacerbate extravascular hemolysis and may occasionally result in intravascular hemolysis. IgM autoantibodies as characteristically found in cold autoantibody autoimmune hemolytic anemia, in cold agglutinin disease but also in a considerable percentage of patients with warm autoantibodies are very likely to activate complement in vivo. Therapy of IgM-mediated autoimmune hemolytic anemia mainly aims to decrease autoantibody production. However, most of these treatments require time to become effective and will not stop immediate ongoing complement-mediated hemolysis nor prevent hemolysis of transfused red blood cells. Therefore pharmacological inhibition of the complement system might be a suitable approach to halt or at least attenuate ongoing hemolysis and improve the recovery of red blood cell transfusion in autoimmune hemolytic anemia. In recent years, several complement inhibitors have become available in the clinic, some of them with proven efficacy in autoimmune hemolytic anemia. In the present review, we give a short introduction on the pathogenesis of autoimmune hemolytic anemia, followed by an overview on the complement system with a special focus on its regulation. Finally, we will discuss complement inhibitors with regard to their potential efficacy to halt or attenuate hemolysis in complement-mediated autoimmune hemolytic anemia.

  3. Anemias.

    Science.gov (United States)

    Broadway-Duren, Jacqueline B; Klaassen, Hillary

    2013-12-01

    Anemias continue to present a challenge to the health care profession. Anemia is defined as a reduction in one or more of the RBC indices. Patients presenting with a mild form of anemia may be asymptomatic; however, in more serious cases the anemia can become life threatening. In many cases the clinical presentation also reflects the underlying cause. Anemia may be attributed to various causes, whereas autoimmune RBC destruction may be attributed to intrinsic and extrinsic factors. Laboratory tests are essential in facilitating early detection and differentiation of anemia.

  4. A case of asymptomatic pancytopenia with clinical features of hemolysis as a presentation of pernicious anemia.

    Science.gov (United States)

    Kollipara, Venkateswara K; Brine, Patrick L; Gemmel, David; Ingnam, Sisham

    2016-01-01

    Pernicious anemia is an autoimmune disease with a variety of clinical presentations. We describe a case of pernicious anemia presenting with pancytopenia with hemolytic features. Further workup revealed very low vitamin B12 levels and elevated methylmalonic acid. It is important for a general internist to identify pernicious anemia as one of the cause of pancytopenia and hemolytic anemia to avoid extensive workup. Pernicious anemia can present strictly with hematological abnormalities without neurological problems or vice versa as in our case. PMID:27609735

  5. Portable microsystem integrates multifunctional dielectrophoresis manipulations and a surface stress biosensor to detect red blood cells for hemolytic anemia.

    Science.gov (United States)

    Sang, Shengbo; Feng, Qiliang; Jian, Aoqun; Li, Huiming; Ji, Jianlong; Duan, Qianqian; Zhang, Wendong; Wang, Tao

    2016-01-01

    Hemolytic anemia intensity has been suggested as a vital factor for the growth of certain clinical complications of sickle cell disease. However, there is no effective and rapid diagnostic method. As a powerful platform for bio-particles testing, biosensors integrated with microfluidics offer great potential for a new generation of portable point of care systems. In this paper, we describe a novel portable microsystem consisting of a multifunctional dielectrophoresis manipulations (MDM) device and a surface stress biosensor to separate and detect red blood cells (RBCs) for diagnosis of hemolytic anemia. The peripheral circuit to power the interdigitated electrode array of the MDM device and the surface stress biosensor test platform were integrated into a portable signal system. The MDM includes a preparing region, a focusing region, and a sorting region. Simulation and experimental results show the RBCs trajectories when they are subjected to the positive DEP force, allowing the successful sorting of living/dead RBCs. Separated RBCs are then transported to the biosensor and the capacitance values resulting from the variation of surface stress were measured. The diagnosis of hemolytic anemia can be realized by detecting RBCs and the portable microsystem provides the assessment to the hemolytic anemia patient. PMID:27647457

  6. Lack of evidence of a beneficial effect of azathioprine immune-mediated hemolytic anemia: a retrospective cohort study

    NARCIS (Netherlands)

    Piek, C.J.; Spil, Van W.E.; Junius, G.; Dekker, A.

    2011-01-01

    Background Azathioprine is used as an immunosuppressant in canine immune-mediated hemolytic anemia (IMHA), but this potentially toxic and carcinogenic drug has not been proven to be beneficial. The aim of this study was to determine the difference in outcome and survival of dogs with idiopathic IMHA

  7. Adrenal failure followed by status epilepticus and hemolytic anemia in primary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Bures Vladimir

    2005-04-01

    Full Text Available Abstract We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon. Conclusion Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms.

  8. Immune-mediated hemolytic anemia - report of three cases / Anemia hemolítica imunomediada em cães - relato de três casos

    Directory of Open Access Journals (Sweden)

    Luciana Curotto Nolasco de Carvalho

    Full Text Available Immune-mediated hemolytic anemia (IMHA is a common type of anemia in dogs and cats. The disease é most common in middle-aged female dogs, especially American Cocker Spaniel. The clinical signs are associated with severe anemia. There is no pathognomonic test for IMHA, but the presence of hemolytic anemia in a young adult or middle age, autoagglutination and spherocytosis or positive results of Coombs test, elimination of any other underlying cause of anemia and an appropriate response to immunosuppressive therapy are suggestive of it. The aim of the present paper is to report of three cases of serious IMHA, and highlighting the therapeutic modalities and prognosis associated with them.A anemia hemolítica imunomediada (AHIM é um tipo comum de anemia em cães e gatos. A doença é mais comum em fêmeas caninas de meia-idade, especialmente Cocker Spaniel Americano. Os sinais clínicos estão associados com a anemia severa. Não há achados patognomônicos, mas a presença de anemia hemolítica em um cão jovem ou de meia idade, auto-aglutinação e esferócitos ou teste de Coombs positivo, eliminação de outros diagnósticos diferenciais e a resposta apropriada a terapia imunossupressora indicam AHIM. Apesar de inúmeras opções terapêuticas, os índices de mortalidade permanecem elevados. O objetivo do presente trabalho é relatar três casos graves de AHIM, ressaltando as modalidades terapêuticas e o prognóstico associado a elas.

  9. Early-onset neutropenia induced by rituximab in a patient with lupus nephritis and hemolytic anemia.

    Science.gov (United States)

    Arroyo-Ávila, Mariangelí; Fred-Jiménez, Ruth M; Vilá, Luis M

    2015-01-01

    Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE) including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 10(9)/L) after the second weekly rituximab infusion (375 mg/m(2) weekly × 4) given for nephritis and hemolytic anemia. She also had early-onset thrombocytopenia after rituximab therapy. Both hematological disorders resolved 12 days after the fourth and final dose. This case, together with few others, suggests that early-onset neutropenia may occur during rituximab therapy. Even though rituximab-induced neutropenia seems to be transient, it may predispose SLE patients to severe complications such as infections. PMID:25767732

  10. Early-Onset Neutropenia Induced by Rituximab in a Patient with Lupus Nephritis and Hemolytic Anemia

    Directory of Open Access Journals (Sweden)

    Mariangelí Arroyo-Ávila

    2015-01-01

    Full Text Available Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 109/L after the second weekly rituximab infusion (375 mg/m2 weekly × 4 given for nephritis and hemolytic anemia. She also had early-onset thrombocytopenia after rituximab therapy. Both hematological disorders resolved 12 days after the fourth and final dose. This case, together with few others, suggests that early-onset neutropenia may occur during rituximab therapy. Even though rituximab-induced neutropenia seems to be transient, it may predispose SLE patients to severe complications such as infections.

  11. Systemic neosporosis in a dog treated for immune-mediated thrombocytopenia and hemolytic anemia.

    Science.gov (United States)

    Magaña, Angie; Sánchez, Félix; Villa, Karina; Rivera, Liliana; Morales, Elizabeth

    2015-12-01

    A 4-year-old male Toy Poodle was presented to the Small Animal Veterinary Hospital of the Faculty of Veterinary Medicine of the Autonomous University of Mexico (FMVZ, UNAM) because of depression, lethargy, and hemorrhages involving several areas of the skin and around the eyes. Hematology data and a bone marrow analysis suggested hemolytic anemia and immune-mediated thrombocytopenia. The dog was treated with prednisone, and after one month the hematology variables improved. However, the dog's clinical condition inexplicably worsened and it was euthanized. On necropsy, there were no relevant findings. However, in histology, multifocal lymphoplasmacytic and histiocytic meningoencephalitis and necrosis, and a protozoan cyst in the cerebellum were identified. In addition, moderate multifocal lymphoplasmacytic and necrotizing pancreatitis, hepatitis, myocarditis, and diffuse lymphoplasmacytic enteritis were observed. Immunohistochemistry of the cerebellum, liver, pancreas, and intestine with a specific antibody against Neospora caninum confirmed the diagnosis of systemic neosporosis. The systemic neosporosis in this dog was most likely caused by reactivation of latent parasites due to prednisone administration during the one month of treatment. It should be kept in mind that in dogs being treated with immunosuppressants for immune-mediated conditions, opportunistic parasites, such as Toxoplasma gondii and N caninum, can be reactivated from a latent state, as it probably happened in the present case. PMID:26345698

  12. EPO-dependent induction of erythroferrone drives hepcidin suppression and systematic iron absorption under phenylhydrazine-induced hemolytic anemia.

    Science.gov (United States)

    Jiang, Xingkang; Gao, Ming; Chen, Yue; Liu, Jing; Qi, Shiyong; Ma, Juan; Zhang, Zhihong; Xu, Yong

    2016-05-01

    Hemolytic anemia is a common form of anemia due to hemolysis, resulting in disordered iron homeostasis. In this study, a dose of 40mg/kg phenylhydrazine (PHZ) was injected into mice to successfully establish a pronounced anemia animal model, which resulted in stress erythropoiesis and iron absorption. We found that serum erythropoietin (EPO) concentration was dramatically elevated by nearly 5000-fold for the first 2days, and then drop to the basal level on day 6 after PHZ injection. Mirrored with serum EPO concentration, the mRNA expression of erythroferrone (ERFE) was rapidly increased in the bone marrow and spleen 3days after injection of PHZ, and then gradually decreased but was still higher than baseline on day 6. In addition, we also found that the hepcidin mRNA levels were gradually reduced almost up to 8-fold on day 5, and then was ameliorated compared to the untreated control. Mechanistic investigation manifested that the increase of serum EPO essentially determined the induction of ERFE expression particular at the first 3days after PHZ treatment. Lentiviral mediated ERFE knockdown significantly restrained hepcidin suppression under PHZ treatment. Thus, our data unearthed EPO-dependent ERFE expression acts as an erythropoiesis-driven regulator of iron metabolism under PHZ-induced hemolytic anemia. PMID:27067488

  13. Severe iron overload and hyporegenerative anemia in a case with rhesus hemolytic disease: therapeutic approach to rare complications

    Directory of Open Access Journals (Sweden)

    Fatih Demircioğlu

    2010-09-01

    Full Text Available A 33 weeks’ gestation, a baby with rhesus hemolytic disease (RHD, who had received intrauterine transfusions twice, developed cholestatic hepatic disease and late hyporegenerative anemia. Her serum ferritin and bilirubin levels increased to 8842 ng/ml and 17.9 mg/dl, respectively. Liver biopsy showed cholestasis and severe iron overload. Treatment with recombinant erythropoietin (rHuEPO decreased the transfusion need, and intravenous deferoxamine resulted in a marked decreased in serum ferritin levels and normalization of liver function. In patients who have undergone intrauterine transfusions due to RHD, hyperferritinemia and late hyporegenerative anemia should be kept in mind. Chelation therapy in cases with symptomatic hyperferritinemia and rHuEPO treatment in cases with severe hyporegenerative anemia should be considered.

  14. Inborn anemias in mice. Progress report to accompany twenty-first renewal proposal, 1 May 1975--30 April 1976

    Energy Technology Data Exchange (ETDEWEB)

    Russell, E.S.; Bernstein, S.E.

    1976-05-15

    Progress is reported on studies on hereditary anemias of mice. At present under study are four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, and the autoimmune hemolytic anemia of NZB. Each of these blood dyscrasias is caused by the action of a unique mutant gene, each of which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus our wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse.

  15. Anemia hemolítica imunomediada não regenerativa em um cão Nonregenerative immune-mediated hemolytic anemia in a dog

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    Leonardo Pinto Brandão

    2004-04-01

    Full Text Available Quadros hemolíticos não eritrorregenerativos são descritos em cães e podem ser decorrentes de doença medular primária, bem como, da destruição dos precursores eritróides medulares por imunoglobulinas. Um cão macho, de três anos de idade, sem raça definida, foi atendido no Hospital Veterinário da Faculdade de Medicina Veterinária e Zootecnia da Universidade de São Paulo apresentando sinais de anemia hemolítica aguda arregenerativa. Após a instituição de terapia imunossupressora (prednisona, houve remissão da hemólise sem, no entanto, sinais de eritrorregeneração. No décimo dia de tratamento, o mielograma demonstrou discreta hipoplasia e displasia eritróide, descartando a possibilidade de aplasia medular. Associou-se ciclofosfamida e azatioprina ao tratamento, tendo havido resposta eritrorregenerativa e recuperação dos valores hematológicos. A ocorrência deste caso de anemia hemolítica não eritrorregenerativa deve servir como alerta para a ocorrência desta condição mórbida, como também, da importância da utilização do mielograma como método auxiliar no diagnóstico de anemias arregenerativas.Nonregenerative forms of immune-mediated hemolytic anemia has been describe in dogs and are attributed to bone marrow diseases or immune-mediated destruction of erythroid progenitors. A 3-year-old, male mongrel dog was received at the Veterinary Hospital of the Faculdade of Medicina Veterinária e Zootecnia da Universidade de São Paulo (USP, Brazil showing signs of acute hemolytic anemia without erythroregeneration. After immunosuppressive therapy (prednisone the hemolysis was suppressed but the dog didn’t show hematologic signs of erythroid response. On the 10th day after treatment a bone marrow aspiration was performed and signs of hypoplasia and mild erythroid cell dysplasia were the main features observed, which could exclude the suspicious of marrow aplasia. After the addiction of cyclophosphamide and

  16. A case of aggravation of hemolytic anemia, elevated liver enzymes and low platelet count syndrome after delivery

    Institute of Scientific and Technical Information of China (English)

    JIANG Yuan-hui; WANG Yong-qing; WANG Jing; YE Rong-hua

    2011-01-01

    Background Hemolytic anemia, elevated liver enzymes and low platelet count (HELLP) syndrome is a severe obstetric complication which usually resolves in most patients after delivery.Methods We report a rare case of aggravation of HELLP syndrome after delivery.Results The patient underwent the treatment for HELLP syndrome,.including glucocorticoid therapy. The symptoms of HELLP syndrome reappeared and became more severe than before the termination of pregnancy. The patient also had severe and persistent hypoproteinemia, hyponatremia and hypocalcemia.Conclusions HELLP syndrome is an acute and critical obstetric syndrome which can have heterogeneous presentations and variable prognosis. We should be fully aware of the diverse clinical characteristics of this condition.

  17. Recombinant Human Erythropoietin Therapy for a Jehovah's Witness Child With Severe Anemia due to Hemolytic-Uremic Syndrome.

    Science.gov (United States)

    Woo, Da Eun; Lee, Jae Min; Kim, Yu Kyung; Park, Yong Hoon

    2016-02-01

    Patients with hemolytic-uremic syndrome (HUS) can rapidly develop profound anemia as the disease progresses, as a consequence of red blood cell (RBC) hemolysis and inadequate erythropoietin synthesis. Therefore, RBC transfusion should be considered in HUS patients with severe anemia to avoid cardiac or pulmonary complications. Most patients who are Jehovah's Witnesses refuse blood transfusion, even in the face of life-threatening medical conditions due to their religious convictions. These patients require management alternatives to blood transfusions. Erythropoietin is a glycopeptide that enhances endogenous erythropoiesis in the bone marrow. With the availability of recombinant human erythropoietin (rHuEPO), several authors have reported its successful use in patients refusing blood transfusion. However, the optimal dose and duration of treatment with rHuEPO are not established. We report a case of a 2-year-old boy with diarrhea-associated HUS whose family members are Jehovah's Witnesses. He had severe anemia with acute kidney injury. His lowest hemoglobin level was 3.6 g/dL, but his parents refused treatment with packed RBC transfusion due to their religious beliefs. Therefore, we treated him with high-dose rHuEPO (300 IU/kg/day) as well as folic acid, vitamin B12, and intravenous iron. The hemoglobin level increased steadily to 7.4 g/dL after 10 days of treatment and his renal function improved without any complications. To our knowledge, this is the first case of successful rHuEPO treatment in a Jehovah's Witness child with severe anemia due to HUS. PMID:26958070

  18. Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia

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    Sarfraz Saleemi

    2014-01-01

    Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO group I to group V PH diseases. Treatment strategies are also unique and include blood transfusion, iron chelation, hydroxyurea, and oxygen therapy. The role of PH-specific agents has not been established.

  19. The hemolytic component of cancer anemia: effects of osmotic and metabolic stress on the erythrocytes of rats bearing multifocal inoculations of the Walker 256 tumor

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    Vido A.A.

    2000-01-01

    Full Text Available Cancer anemia is classified as an anemia of chronic diseases, although it is sometimes the first symptom of cancer. Cancer anemia includes a hemolytic component, important in the terminal stage when even transfused cells are rapidly destroyed. The presence of a chronic component and the terminal complications of the illness limit studies of the hemolytic component. A multifocal model of tumor growth was used here to simulate the terminal metastatic dissemination stage (several simultaneous inoculations of Walker 256 cells. The hemolytic component of anemia began 3-4 days after inoculation in 100% of the rats and progressed rapidly thereafter: Hb levels dropped from 14.9 ± 0.02 to 8.7 ± 0.06 from days 7 to 11 (~5 times the physiologically normal rate in rats in the absence of bleeding. The development of anemia was correlated (r2 = 0.86 with the development of other systemic effects such as anorexia. There was a significant decrease in the osmotic fragility of circulating erythrocytes: the NaCl concentration causing 50% lysis was reduced from 4.52 ± 0.06 to 4.10 ± 0.01 (P<0.01 on day 7, indicating a reduction in erythrocyte volume. However, with mild metabolic stress (4-h incubation at 37oC, the erythrocytes showed a greater increase in osmotic fragility than the controls, suggesting marked alteration of erythrocyte homeostasis. These effects may be due to primary plasma membrane alterations (transport and/or permeability and/or may be secondary to metabolic changes. This multifocal model is adequate for studying the hemolytic component of cancer anemia since it is rapid, highly reproducible and causes minimal animal suffering.

  20. Anemia hemolítica microangiopática induzida por tacrolimus e ciclosporina A Microangiopathic hemolytic anemia induced by tacrolimus and ciclosporine A

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    Rafael S. Vasconcelos

    2008-12-01

    Full Text Available Relatamos caso raro de anemia hemolítica microangiopática em paciente após transplante hepático por cirrose Child B causada por deficiência de alfa1-antitripsina. Após análise clínica e laboratorial extensa, concluiu-se que o quadro de hemólise foi causado pela medicação imunossupressora utilizada após transplante. Inicialmente foi utilizado tacrolimus que, posteriormente, foi substituído por ciclosporina A, entretanto não houve melhora da hemólise. Após suspensão das duas drogas, houve melhora importante clínica do paciente, com normalização dos parâmetros hematológicos.We report a rare case of acute hemolysis in a patient after liver transplant for Child B cirrhosis caused by alfa1-antitripsine deficiency. History and laboratorial examinations indicated that the most probable cause of the hemolysis was the use of the immunosupressor tacrolimus and later Cyclosporine A. After the discontinuation of these drugs, there was improvement in the clinical condition of the patient, with a compensated hemolytic anemia.

  1. Anemia hemolítica causada por Ditaxis desertorum (Euphorbiaceae em bovinos Hemolytic anemia caused by Ditaxis desertorum (Euphorbiaceae in cattle

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    Carlos Hubinger Tokarnia

    1997-07-01

    Full Text Available Ditaxis desertorum Pax et K. Hoffm., planta herbácea da família Euphorbiaceae, causou experimentalmente em bovinos um quadro caracterizado por hemoglobinúria em virtude de sua ação hemolítica, quando administrada por via oral em doses diárias de 1,0 a 2,5 g/kg (planta fresca, a partir do 4° ao 8° dia do experimento. Após um período de 3 a 5 dias em que os animais tiveram hemoglobinúria e anemia acentuadas, apesar de continuarem a receber a planta (durante um total de 12 a 14 dias, em três dos quatro animais esses sintomas desapareceram. Verificou-se nesses casos uma rápida recuperação dos valores hemáticos logo que cessou a hemoglobinúria. O quarto bovino, que recebeu 2,5 g/kg/dia durante 5 dias, morreu no 8° dia, tendo apresentado durante os últimos 4 dias de vida hemoglobinúria e anemia acentuadas. À necropsia e nos exames histopatológicos deste animal foram verificadas nefrose hemoglobinúrica e distrofia hepática com necrose centrolobular do parênquima. Dose de 7,7 g/kg única ou quantidades de 2,5 e 3 g/kg/dia administradas durante 2 dias seguidos, causaram em três outros bovinos quadro clínico de cólica, com morte em questão de horas, verificando-se à necropsia acentuado edema da parede do rúmen e do retículo. Pelos históricos obtidos somente ocorre, sob condições naturais, a intoxicação caracterizada pelo quadro da anemia hemolítica, indicando que possivelmente a ingestão de D. desertorum em quantidades necessárias para causar o quadro com lesões dos proventrículos ser, apesar de sua boa palatabilidade, autolimitada pelo efeito cáustico da planta.Ditaxis desertorum Pax et K. Hoffm., a herbaceous plant of the Euphorbiaceae family, caused hemoglobinuria due to its hemolytic properties, when force-fed fresh to bovines in daily doses of 1.0 to 2.5 g/kg; the hemoglobinuria appeared from the 4th to 8th day of the experiment. After a period of 3 to 5 days of severe hemoglobinuria and anemia, these

  2. A case of asymptomatic pancytopenia with clinical features of hemolysis as a presentation of pernicious anemia

    Directory of Open Access Journals (Sweden)

    Venkateswara K. Kollipara

    2016-09-01

    Full Text Available Pernicious anemia is an autoimmune disease with a variety of clinical presentations. We describe a case of pernicious anemia presenting with pancytopenia with hemolytic features. Further workup revealed very low vitamin B12 levels and elevated methylmalonic acid. It is important for a general internist to identify pernicious anemia as one of the cause of pancytopenia and hemolytic anemia to avoid extensive workup. Pernicious anemia can present strictly with hematological abnormalities without neurological problems or vice versa as in our case.

  3. Clostridium Perfringens Infection in a Febrile Patient with Severe Hemolytic Anemia

    OpenAIRE

    Hashiba, Masamitsu; Tomino, Atsutoshi; Takenaka, Nobuyoshi; Hattori, Tomonori; Kano, Hideki; Tsuda, Masanobu; Takeyama, Naoshi

    2016-01-01

    Patient: Male, 82 Final Diagnosis: Clostridium perfringens infection Symptoms: Anemia • fever • shock Medication: — Clinical Procedure: Antimicrobial chemotherapy Specialty: Infectious Diseases Objective: Rare disease Background: Clostridium perfringens (C. perfringens) can cause various infections, including gas gangrene, crepitant cellulitis, and fasciitis. While C. perfringens sepsis is uncommon, it is often rapidly fatal because the alpha toxin of this bacterium induces massive intravascu...

  4. Who Is at Risk for Anemia?

    Science.gov (United States)

    ... Aplastic Anemia Hemolytic Anemia Iron-Deficiency Anemia Pernicious Anemia Sickle Cell Disease Send a link to NHLBI to someone ... A family history of inherited anemia, such as sickle cell anemia or thalassemia Rate This Content: NEXT >> Updated: May ...

  5. Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases

    Science.gov (United States)

    2005-06-23

    Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

  6. Gene disruption of dematin causes precipitous loss of erythrocyte membrane stability and severe hemolytic anemia.

    Science.gov (United States)

    Lu, Yunzhe; Hanada, Toshihiko; Fujiwara, Yuko; Nwankwo, Jennifer O; Wieschhaus, Adam J; Hartwig, John; Huang, Sha; Han, Jongyoon; Chishti, Athar H

    2016-07-01

    Dematin is a relatively low abundance actin binding and bundling protein associated with the spectrin-actin junctions of mature erythrocytes. Primary structure of dematin includes a loosely folded core domain and a compact headpiece domain that was originally identified in villin. Dematin's actin binding properties are regulated by phosphorylation of its headpiece domain by cyclic adenosine monophosphate-dependent protein kinase. Here, we used a novel gene disruption strategy to generate the whole body dematin gene knockout mouse model (FLKO). FLKO mice, while born at a normal Mendelian ratio, developed severe anemia and exhibited profound aberrations of erythrocyte morphology and membrane stability. Having no apparent effect on primitive erythropoiesis, FLKO mice show significant enhancement of erythroblast enucleation during definitive erythropoiesis. Using membrane protein analysis, domain mapping, electron microscopy, and dynamic deformability measurements, we investigated the mechanism of membrane instability in FLKO erythrocytes. Although many membrane and cytoskeletal proteins remained at their normal levels, the major peripheral membrane proteins spectrin, adducin, and actin were greatly reduced in FLKO erythrocytes. Our results demonstrate that dematin plays a critical role in maintaining the fundamental properties of the membrane cytoskeleton complex. PMID:27073223

  7. Liver cirrhosis as a consequence of iron overload caused by hereditary nonspherocytic hemolytic anemia

    Institute of Scientific and Technical Information of China (English)

    Philip Hilgard; Guido Gerken

    2005-01-01

    Nonspherocytic hereditary anemias are occasionally accompanied by significant iron overload but the significance for the development of chronic liver disease is not clear. We described two cases of patients with chronic liver d isease and severeiron overload due to chronic hereditary hemolysis. Both patients have had signs of liver cirrhosis and severe hemolysis since childhood. A hereditary pyruvate kinase deficiency (PKD) was discovered as the underlying reason for the hemolysis.Sequencing of the pyruvate kinase gene showed a mutation within exon 11. Liver histology in both patients revealed cirrhosis and a severe iron overload but primary hemochromatosis was excluded by HFE-gene analysis.An iron reduction therapy with desferrioxamine led to significant decrease of serum ferritin and sustained clinical improvement. PKD-induced hemolysis may cause severe iron overload even in the absence of HFE-genotype abnormalities. This secondary iron overload can lead to chronic liver disease and cirrhosis. Therefore, the iron metabolism of PKD patients has to be closely monitored and iron overload should be consequently treated.

  8. Men with Sickle Cell Anemia and Priapism Exhibit Increased Hemolytic Rate, Decreased Red Blood Cell Deformability and Increased Red Blood Cell Aggregate Strength.

    Directory of Open Access Journals (Sweden)

    Kizzy-Clara Cita

    Full Text Available To investigate the association between priapism in men with sickle cell anemia (SCA and hemorheological and hemolytical parameters.Fifty-eight men with SCA (median age: 38 years were included; 28 who had experienced priapism at least once during their life (priapism group and 30 who never experienced this complication (control group. Twenty-two patients were treated with hydroxycarbamide, 11 in each group. All patients were at steady state at the time of inclusion. Hematological and biochemical parameters were obtained through routine procedures. The Laser-assisted Optical Rotational Cell Analyzer was used to measure red blood cell (RBC deformability at 30 Pa (ektacytometry and RBC aggregation properties (laser backscatter versus time. Blood viscosity was measured at a shear rate of 225 s-1 using a cone/plate viscometer. A principal component analysis was performed on 4 hemolytic markers (i.e., lactate dehydrogenase (LDH, aspartate aminotransferase (ASAT, total bilirubin (BIL levels and reticulocyte (RET percentage to calculate a hemolytic index.Compared to the control group, patients with priapism exhibited higher ASAT (p = 0.01, LDH (p = 0.03, RET (p = 0.03 levels and hemolytic indices (p = 0.02. Higher RBC aggregates strength (p = 0.01 and lower RBC deformability (p = 0.005 were observed in patients with priapism compared to controls. After removing the hydroxycarbamide-treated patients, RBC deformability (p = 0.01 and RBC aggregate strength (p = 0.03 were still different between the two groups, and patients with priapism exhibited significantly higher hemolytic indices (p = 0.01 than controls.Our results confirm that priapism in SCA is associated with higher hemolytic rates and show for the first time that this complication is also associated with higher RBC aggregate strength and lower RBC deformability.

  9. Anemia hemolítica causada por Indigofera suffruticosa (Leg. Papilionoideae em bovinos Hemolytic anemia caused by Indigofera suffruticosa (Leg. Papilionoideae in cattle

    Directory of Open Access Journals (Sweden)

    José Diomedes Barbosa Neto

    2001-03-01

    animals had hemoglobinuria, which was transitory, inspite continuation of the administration of the plant. Two animals had no further manifestations, a third animal showed only slight other manifestations, and the other three had additional symptoms of moderate intensity. These were apathy, whitish visible mucous membranes, rough hair coat, anorexia, descrease in frequency and intensity of the ruminal movements, tachycardia, positive venous pulse and dispnoea. Before the occurrence of the hemolytic crisis the urine had a bluish-green colour. None of the experimental animals died, but one was euthanized whilst showing hemoglobinuria. Post-mortem findings were anemia, the bladder containing wine-red urine, swollen dark-brown kidneys, liver on the outside and on the cut-surface bluish and with perceptible lobular design. The main histological changes were found in liver and kidney. In the liver there was coagulative necrosis and cloudy swelling and/or cytoplasmatic microvacuolization of the hepatocytes; in the kidney there was severe nephrosis, associated with large amounts of filtrate and/or hemoglobine in the Bowman spaces, in the tubules and also in the cytoplasm of the epithelial cells.

  10. Hereditary nonspherocytic hemolytic anemia caused by red cell glucose-6-phosphate isomerase (GPI) deficiency in two Portuguese patients: Clinical features and molecular study.

    Science.gov (United States)

    Manco, Licínio; Bento, Celeste; Victor, Bruno L; Pereira, Janet; Relvas, Luís; Brito, Rui M; Seabra, Carlos; Maia, Tabita M; Ribeiro, M Letícia

    2016-09-01

    Glucose-6-phosphate isomerase (GPI) deficiency cause hereditary nonspherocytic hemolytic anemia (HNSHA) of variable severity in individuals homozygous or compound heterozygous for mutations in GPI gene. This work presents clinical features and genotypic results of two patients of Portuguese origin with GPI deficiency. The patients suffer from a mild hemolytic anemia (Hb levels ranging from 10 to 12.7g/mL) associated with macrocytosis, reticulocytosis, hyperbilirubinemia, hyperferritinemia and slight splenomegaly. Genomic DNA sequencing revealed in one patient homozygosity for a new missense mutation in exon 3, c.260G>C (p.Gly87Ala), and in the second patient compound heterozygosity for the same missense mutation (p.Gly87Ala), along with a frameshift mutation resulting from a single nucleotide deletion in exon 14, c.1238delA (p.Gln413Arg fs*24). Mutation p.Gln413Arg fs*24 is the first frameshift null mutation to be described in GPI deficiency. Molecular modeling suggests that the structural change induced by the p.Gly87Ala pathogenic variant has direct impact in the structural arrangement of the region close to the active site of the enzyme. PMID:27519939

  11. 化学物理与生物毒素所致的溶血性贫血%Hemolytic anemia resulting from chemical and physical agents and biotoxins

    Institute of Scientific and Technical Information of China (English)

    许小平

    2012-01-01

    Non-immune hemolytic anemia induced by chemical, physical factors and biological toxins is associated with external factors, unrelated to erythrocytes themselves. Hemolysis can be the only one of clinical features, also coexist with features of other organs. This paper will introduce the mechanism, diagnosis and treatment of hemolytic anemia which caused by chemical, physical factors and biological toxins which happens accidentally in our normal life.%化学、物理与生物毒素所致的溶血性贫血属于红细胞外在因素导致的非免疫性溶血性贫血.溶血可以是患者唯一的临床表现,也可以与其他脏器的症状合并存在.文章重点介绍日常生活中较为多见的化学、物理与生物毒素引起溶血性贫血的发生机制以及诊治原则.

  12. Inheritance of the Bantu/Benin haplotype causes less severe hemolytic and oxidative stress in sickle cell anemia patients treated with hydroxycarbamide.

    Science.gov (United States)

    Okumura, Jéssika V; Silva, Danilo G H; Torres, Lidiane S; Belini-Junior, Edis; Barberino, Willian M; Oliveira, Renan G; Carrocini, Gisele C S; Gelaleti, Gabriela B; Lobo, Clarisse L C; Bonini-Domingos, Claudia R

    2016-07-01

    Beta S-globin gene cluster haplotypes (β(S)-haplotypes) can modulate the response to hydroxycarbamide (HC) treatment in sickle cell anemia (SCA) patients. In Brazil, the most common haplotypes are Bantu and Benin, and both confer a poor prognosis for patients when untreated with HC. We evaluated oxidative and hemolytic biomarkers in 48 SCA patients undergoing HC treatment separated in three subgroups: Bantu/Bantu, Bantu/Benin and Benin/Benin haplotype. On the basis of reduced haptoglobin (HP) levels, patients with Bantu/Bantu haplotypes had 3.0% higher hemolysis degree when compared with those with Bantu/Benin haplotypes (P=0.01). The Benin/Benin patients had 53.6% greater lipid peroxidation index than the Bantu/Bantu patients (P=0.01) because of evaluated thiobarbituric acid reactive species levels. The Bantu/Benin subgroup had intermediate levels of hemolytic and oxidative stress markers compared with the homozygous subgroups. Through strict inclusion criteria adopted, as well as consolidated and well-described hemolytic and the oxidative parameters evaluated, we suggest a haplotype-interaction response to HC treatment mediated by a 'balance' between the genetic factors of each haplotype studied. PMID:26961071

  13. Favism, the commonest form of severe hemolytic anemia in Palestinian children, varies in severity with three different variants of G6PD deficiency within the same community.

    Science.gov (United States)

    Reading, N Scott; Sirdah, Mahmoud M; Shubair, Mohammad E; Nelson, Benjamin E; Al-Kahlout, Mustafa S; Al-Tayeb, Jamal M; Aboud, Lina N; Shaban, Maysaa Abu; Luzzatto, Lucio; Prchal, Josef T

    2016-09-01

    Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common genetic abnormality known to predispose to acute hemolytic anemia (AHA), which can be triggered by certain drugs or infection. However, the commonest trigger is fava beans (Vicia faba) ingestion, causing AHA (favism), which may be life-threatening especially in children. G6PD deficiency is genetically highly heterogeneous, as nearly 200 different mutations have been observed. We have investigated the hematological features of acute favism in the Palestinian Gaza community that is characterized by the polymorphic coexistence of three different G6PD deficiency genes (G6PD A-, G6PD Cairo, G6PD Med). We have found by comparison to the general population (485 adults and 466 newborns) that children with favism, in terms of relative frequency, G6PD A- was under-represented, whereas G6PD Med was over-represented. We also found that the severity of anemia was significantly greater with G6PD Med and G6PD Cairo than with G6PD A-; and with G6PD Cairo, compared to the other two variants, there was greater hyperbilirubinemia, as well as persistence of mild anemia and reticulocytosis for as long as 4months after recovery from favism. This is the first report determining a differential impact of different G6PD mutations on the clinical features of favism in the same population and the same environment. PMID:27519946

  14. Multidisciplinary approach to anemia

    Directory of Open Access Journals (Sweden)

    Anca Ghiațău

    2015-08-01

    Full Text Available Introduction: We present the case of a 65 years- old woman who was admitted with a severe macrocytic anemia Hb= 5.7g/dl and diffuse bone pain. Biologically she has moderate thrombocytopenia 35 000/µl, a hepatic cytolysis and cholestatic syndrome. Material and method: The patient was extensively evaluated before presentation for a mild iron - deficiency anemia for which she underwent endoscopic examination of the upper and lower gastrointestinal tract- normal. The bone marrow aspiration on admission revealed a marked hyperplasia of the erythroblastic line with ~50% basophilic erythroblasts suggesting a regenerative erythroid hyperplasia. These changes along with the marked reticulocytosis on the peripheral blood smear oriented us towards a hemolytic anemia; Folic acid, vitamin B12, autoimmune tests and hemolytic tests were all normal. We continued the investigations with a thoraco-abdominopelvic computed tomography which identified diffuse demineralization, vertebral compactation and pelvic stress fractures. The breast examination revealed a right breast nodule, but the breast ultrasonography pleaded for benignity. Lacking a clear definitive diagnosis we decided to perform a bone marrow biopsy. Results: The osteo- medullary biopsy pointed towards a medullar invasion from a lobular mammary carcinoma; In these circumstances we performed an ultrasound guided biopsy of the right mammary lump thus histologically confirming a tumoral invasion of the bone marrow with subsequent anemia. The patient started chemotherapy in the Oncology ward. Conclusion: The particularity of this case consists in the pattern of anemia, which initially seemed iron deficient and afterwards macrocytic – apparently hemolytic and was actually due to the tumoral medullar invasion and also the nonspecific ultrasonographic appearance of the breast tumor.

  15. Microangiopathic Hemolytic Anemia in 57-year-old woman with Borderline Serous Tumor of the Ovary:Real-Time Management of Common Pathways of Hemostatic Failure

    Directory of Open Access Journals (Sweden)

    Gloria Joan Morris

    2012-05-01

    Full Text Available We present a case of a 57-year-old woman who underwent surgery for the removal of an ovarian mass but subsequently experienced microangioathic hemolytic anemia post-operatively, associated with fevers, renal insufficiency, hypertension, and hemolysis. While her clinical situations was initially suspicious for thrombotic thrombocytopenic purpura (TTP, further sorting of clinical information led to other explanations of these findings, including a systemic inflammatory response. Multiple triggers of the coagulation system which can lead to a common pathway of hemostatic failure were considered, and specific criteria seen in disseminated intravascular coagulation (DIC, TTP, heparin-induced thrombocytopenia (HIT, catastrophic antiphospholipid anitbody syndrom (APS, all of which can seem to overlap when a physician is faced with distinguishing the diagnosis clinically. We propose a chronologic and strategic approach for the clinician to consider when approaching this diagnostic dilemma.

  16. Diagnosis and management of autoimmune cytopenias in childhood.

    Science.gov (United States)

    Teachey, David T; Lambert, Michele P

    2013-12-01

    The diagnosis and management of children with autoimmune cytopenias can be challenging. Children can present with immune-mediated destruction of a single-cell lineage or multiple cell lineages, including platelets (immune thrombocytopenia [ITP]), erythrocytes (autoimmune hemolytic anemia), and neutrophils (autoimmune neutropenia). Immune-mediated destruction can be primary or secondary to a comorbid immunodeficiency, malignancy, rheumatologic condition, or lymphoproliferative disorder. Treatment options generally consist of nonspecific immune suppression or modulation. This nonspecific approach is changing as recent insights into disease biology have led to targeted therapies, including the use of thrombopoietin mimetics in ITP and sirolimus for cytopenias associated with autoimmune lymphoproliferative syndrome. PMID:24237984

  17. Hemolytic anemia caused by hereditary pyruvate kinase deficiency in a West Highland White Terrier dog Anemia hemolítica causada por la deficiencia de piruvato quinasa hereditaria en un perro West Highland White Terrier

    Directory of Open Access Journals (Sweden)

    NRC Hlavac

    2012-01-01

    Full Text Available Inherited erythrocytic pyruvate kinase (PK deficiency is an autosomal recessive hemolytic disorder described in dogs and cats. Pyruvate kinase is one of two key-regulatory enzymes in the anaerobic glycolytic pathway, and its deficiency causes premature destruction of the ATP-depleted red cells. This case study reports the clinical and laboratory findings in a West Highland White Terrier (WHWT dog with a life-long history of weakness and exercise intolerance from Brazil. The dog was found to have persistently profound pallor, a highly regenerative hemolytic anemia and osteosclerosis. PK deficiency was confirmed by a breed-specific DNA test for the previously described 6 bp insertion at 3' end of exon 10 in red blood cell (RBC R- PK gene sequence. The dog was euthanized at 20 months of age due to the deterioration of its clinical condition including anemia and blood incompatibility. Other PK-deficient WHWTs have lived as long as 9 years. Hereditary red cell defects are important differential diagnoses for chronic hemolytic anemias in younger animals after excluding immune-mediated and infectious causes. Furthermore, purebred dogs for which DNA tests for hereditary diseases are available should be screened prior to breeding in order to limit the spread of the mutant allele and to avoid future production of PK-deficient animals.La deficiencia de piruvato quinasa (PK es un desorden hemolítico autosómico recesivo descrito en perros y gatos. La piruvato quinasa es una de las enzimas regulatorias esenciales de la glicólisis anaeróbica, la deficiencia de esta enzima causa una destrucción prematura de los eritrocitos. El presente es un estudio de caso y relata los hallazgos clínicos y paraclínicos en un perro brasileño de la raza West Highland White Terrier (WHWT con historia de debilidad e intolerancia al ejercicio. El paciente presentaba mucosas pálidas, anemia hemolítica bastante regenerativa y osteoclerosis. La deficiencia de PK fue

  18. Study on histogenesis of enterochromaffin-like carcinoid in autoimmune atrophic gastritis associated with pernicious anemia

    Directory of Open Access Journals (Sweden)

    Mačukanović-Golubović Lana

    2007-01-01

    Full Text Available Background/Aim. Autoimmune atrophic fundic gastritis induces the pernicious anemia (PA, as well as the changes in both epithelium and endocrine cells of gastric mucosa. The most important complications are: achlorhydria, hypergastrinemia, gastric cancer and enterochromaffin-like ( ECL carcinoid. The aim of this study was to examine ECL carcinoid histogenesis in A-gastritis associated with PA. Methods. During the period from 2000−2006, 65 patients with PA and 30 patients of the control group were examined. Histopathological examination was done in endoscopical biopsies of gastric mucosa fixed in 10% formaldehyde. Paraffin sections were stained with classic hematoxylin-eosin (HE; histochemical AB-PAS (pH 2.5, cytochemical argyrophilic Servier-Munger′s and immunocytochemical PAP methods for G cell identification and chromogranin A antibodies - specific marker for neuroendocrine ECL cells. Both G and ECL cells were counted per 20 fields, of surface 0.0245312 mm2 by a field. Basal gastrin serum levels were also examined by using radioimmunoassay (RIA method. The obtained results were statisticaly calculated by using Student΄s t test. Results. Marked antral G cell hyperplasia associated with corporal ECL hyperplasia was found. ECL cell hyperplasia was of simplex, linear, adenomatoid type to the pattern of intramucous ECL cell carcinoid. An average number of G cells was statistically significant in the patients with PA as compared to the control group (p < 0.05 as well as an average number of ECL cells. Conclusion. We concluded that antral G cell hyperplasia accompanied by gastrinemia induces ECL hyperplasia and ECL corporal carcinoid in A-gastritis and that their histogenesis develops trough simple, linear and adenomatoide hyperplasia. .

  19. Anemia

    Science.gov (United States)

    ... Physician October 01, 2002, http://www.aafp.org/afp/20021001/1217.html) Normocytic Anemia by JR Brill, ... Physician November 15, 2000, http://www.aafp.org/afp/20001115/2255.html) Written by familydoctor.org editorial ...

  20. Microangiopathic Hemolytic Anemia in 57-year-old woman with Borderline Serous Tumor of the Ovary:Real-Time Management of Common Pathways of Hemostatic Failure

    Directory of Open Access Journals (Sweden)

    Gloria Joan Morris

    2012-01-01

    Full Text Available

    We present a case of a 57-year-old woman who underwent surgery for the removal of an ovarian mass but subsequently experienced microangioathic hemolytic anemia post-operatively, associated with fevers, renal insufficiency, hypertension, and hemolysis. While her clinical situations was initially suspicious for thrombotic thrombocytopenic purpura (TTP, further sorting of clinical information led to other explanations of these findings, including a systemic inflammatory response. Multiple triggers of the coagulation system which can lead to a common pathway of hemostatic failure were considered, and specific criteria seen in disseminated intravascular coagulation (DIC, TTP, heparin-induced thrombocytopenia (HIT, catastrophic antiphospholipid anitbody syndrom (APS, all of which can seem to overlap when a physician is faced with distinguishing the diagnosis clinically. We propose a chronologic and strategic approach for the clinician to consider when approaching this diagnostic dilemma.

  1. [Activity of key enzymes of heme metabolism and cytochrome P-450 content in the rat liver in experimental rhabdomyolysis and hemolytic anemia].

    Science.gov (United States)

    Kaliman, P A; Inshina, N N; Strel'chenko, E V

    2003-01-01

    The 5-aminolevulinate synthase, heme oxygenase, tryptophan-2,3-dioxygenase activities, the content of total heme and cytochrome P-450 content in the rat liver and absorption spectrum of blood serum in Soret region under glycerol model of rhabdomiolisis and hemolytic anemia caused by single phenylhydrazine injection have been investigated. The glycerol injection caused a considerable accumulation of heme-containing products in the serum and the increase of the total heme content, holoenzyme, total activity and heme saturation of tryptophan-2,3-dioxygenase, as well as the increase of the 5-aminolevulinate synthase and heme oxygenase activities in the liver during the first hours of its action and the decrease of cytochrome P-450 content in 24 h. Administration of phenylhydrazine lead to the increasing of hemolysis products content in blood serum too, although it was less expressed. The phenylhydrazine injection caused the increase of activities of 5-aminolevulinate synthase, holoenzyme, total activity and heme saturation of tryptophan-2,3-dioxygenase, as well as decrease of cytochrome P-450 content in the rat liver in 2 h. The increase of the total heme content and heme oxygenase activity has been observed in 24 h. The effect of heme arrival from the blood stream, as well as a direct influence of glycerol and phenylhydrazine on the investigated parameters are discussed. PMID:14577161

  2. Quadro seroproteico como auxílio diagnóstico na anemia hemolítica imunomediada em cães Serum proteic profile as diagnosis aids in immune-mediated hemolytic anemia in dogs

    Directory of Open Access Journals (Sweden)

    Patrícia Mendes Pereira

    2010-04-01

    maior nestes. Tais achados analisados em conjunto agregam informações adicionais úteis à elucidação das AHIMs em cães.This assay aimed to determine the serum protein - via polyacrylamide gel electrophoresis, which contained duodecil sodium sulfate (SDS-PAGE - in 120 dogs, with different breeds and ages, seen by the Veterinary Hospital "Governor Laudo Natel." These animals were grouped into five experimental groups: Group 1 - group control with 20 dogs, group 2 - 28 dogs with regenerative anemia; group 3 - 27 dogs with arregenarative; anemia group 4 - 10 dogs with primary immune-mediated hemolytic anemia (AHIM 1.rd; group 5 - 35 dogs with secondary immune-mediated hemolytic anemia (AHIM 2.rd. The technique allowed the SDS-PAGE fractionation of 24 protein, whose molecular weights (PM ranged from 18,000 to 165,000 daltons (Da. The dogs with 1st and 2nd AHIM showed 24 protein fractions in their tracks electrophoretic, while other groups of dogs showed 23 fractions of protein, whose molecular protein weight of 68,000Da was not found. Thus, twenty-three proteins were common to proteinograms of the five experimental groups. From these, it was possible to identify eleven protein fractions nominally, and others were identified by their molecular weights. For control dogs, the anemic (groups 2, 3, 4 and 5 showed higher concentrations of serum transferrin and between them, the animals carrying the primary IMHA. All groups of dogs showed anemic levels of serum haptoglobin and phosphorylase significantly higher than the control dogs, while the serum ceruloplasmin was lower in anemic dogs. These findings provide additional information to the elucidation of the AHIMs in dogs.

  3. Autoimmune Cytopenias in Chronic Lymphocytic Leukemia

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    Giovanni D'Arena

    2013-01-01

    Full Text Available The clinical course of chronic lymphocytic leukemia (CLL may be complicated at any time by autoimmune phenomena.The most common ones are hematologic disorders, such as autoimmune hemolytic anemia (AIHA and immune thrombocytopenia (ITP. Pure red cell aplasia (PRCA and autoimmune agranulocytosis (AG are, indeed, more rarely seen. However, they are probably underestimated due to the possible misleading presence of cytopenias secondary to leukemic bone marrow involvement or to chemotherapy cytotoxicity. The source of autoantibodies is still uncertain, despite the most convincing data are in favor of the involvement of resting normal B-cells. In general, excluding the specific treatment of underlying CLL, the managementof these complications is not different from that of idiopathic autoimmune cytopenias or of those associated to other causes. Among different therapeutic approaches, monoclonal antibody rituximab, given alone or in combination, has shown to be very effective.

  4. Unusual cause of childhood anemia: Imerslund grasbeck syndrome

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    Kishan Prasad Hosapatna Laxminarayana

    2011-01-01

    Full Text Available Imerslund Grasbeck syndrome (IGS is a rare autosomal recessive childhood disorder characterized by selective Vitamin (vit B 12 malabsorption with asymptomatic proteinuria without any structural renal pathology. The patients stay healthy for decades with life-long parenteral vit B12. We report a case of young female who presented with pancytopenia and proteinuria, evaluated in local hospitals as chronic hemolytic anemia (autoimmune cause, finally diagnosed as IGS on complete evaluation. She was treated with injectable vit B12 (1000 μg cyanocobalalmin and showed drastic recovery. IGS should be considered in patients with megaloblastic anemia not responding to oral vit B12 and associated proteinuria.

  5. Investigation of risk factors of Hepatitis E-induced hemolytic anemia%戊型肝炎发生溶血性贫血的危险因素调查

    Institute of Scientific and Technical Information of China (English)

    牛心文

    2013-01-01

    OBJECTIVE To explore risk factors of hepatitis E-induced hemolytic anemia and to provide references for prevention and treatment. METHODS This study included 175 patients with hepatitis E, including 85 patients with hemolytic anemia in group A and 90 patients without hemolytic anemia in group B. Patients' medical history and basic information were recorded and multivariate regression analysis was conducted. RESULTS Univariate analysis results showed there was statistically significant difference in age of 45 or older, enlarged liver and spleen, G-6-PD deficiency, taking anticoagulants, bleeding history, hyperlipidemia, cholelithiasis, IBIL, DBIL, PTA, prothrombin time, ALT and AST between the two groups (P 0.05). Multivariate analysis showed that G-6-PD deficiency, hyperlipidemia, increased PTA, and extended prothrombin time were independent risk factors of hepatitis E-induced hemolytic anemia (P< 0.05). CONCLUSION G-6-PD deficiency, hyperlipidemia, increased PTA, and extended prothrombin time are risk factors of hepatitis E-induced hemolytic anemia. It is necessary to address those risk factors to reduce the occurrence of hemolytic anemia in future intervention.%目的 探讨戊型肝炎发生溶血性贫血的危险因素,为其防治提供参考.方法 共纳入175例戊型肝炎患者,其中85例患者发生溶血性贫血作为A组,90例患者未发生溶血性贫血作为B组.记录两组患者的病史及基本信息,应用多因素回归分析进行多因素分析.结果 单因素分析结果显示:两组间年龄≥45岁、肝脾大、G-6PD缺陷、服用抗凝药、既往出血史、高脂血症、胆石症、IBIL、DBIL、PTA、凝血酶原时间、ALT、AST比较差异有统计学意义(P<0.05);而性别、ALP、Ch、TP、A/G差异无统计学意义(P>0.05).进一步行多因素分析结果表明:G-6PD缺陷、高脂血症、PTA增加、凝血酶原时间延长是戊型肝炎患者发生溶血性贫血的独立危险因素(P<0.05).结论 G-6PD

  6. Clinical indications and biological mechanisms of splenic irradiation in autoimmune diseases

    International Nuclear Information System (INIS)

    Background: Splenic irradiation (SI) is a fairly unknown treatment modality in autoimmune disorders like autoimmune thrombocytopenia (AIT) or autoimmune hemolytic anemia (AIHA), which may provide an effective, low toxic and cost-effective treatment for selected patients. Patients, Materials and Methods: This article reviews the limited experiences on splenic irradiation in autoimmune thrombocytopenia by analyzing the current studies including 71 patients and some preliminary reports on splenic irradiation in autoimmune hemolytic anemia. Results: In autoimmune thrombocytopenia between 40 and 90% of all patients responded, but most of them relapsed within 4 to 6 months after splenic irradiation. Between 10 and 20% of all patients had a sustained response. The efficacy of splenic irradiation in HIV-associated cases of thrombocytopenia is probably lower than in other forms of autoimmune thrombocytopenia, but especially in this group immunosuppressive drug treatment of autoimmune thrombocytopenia exposes some problems. In autoimmune hemolytic anemia there are some case reports about efficacy of splenic irradiation. Toxicity of splenic irradiation in both diseases was very moderate. Conclusions: For HIV patients, for elderly patients or patients at high risk for complications following splenectomy splenic irradiation might be a treatment option. Splenic irradiation as preoperative treatment in patients not responding to or not suitable for immunosuppressive drugs prior to splenectomy may be a promising new application of splenic irradiation to reduce adverse effects of splenectomy in thrombocytopenic patients. A further analysis of the biological mechanisms underlying splenic irradiation may help to improve patient selection, to optimize dose concepts and treatment schedules and will improve understanding of radiotherapy as an immunomodulatory treatment modality. (orig.)

  7. Autoimmune lymphoproliferative syndrome presenting with glomerulonephritis.

    Science.gov (United States)

    Kanegane, Hirokazu; Vilela, Maria Marluce dos Santos; Wang, Yue; Futatani, Takeshi; Matsukura, Hiroyoshi; Miyawaki, Toshio

    2003-05-01

    Autoimmune lymphoproliferative syndrome (ALPS) is characterized clinically by chronic non-malignant lymphoproliferation and autoimmunity and is caused by a genetic defect in programmed cell death (apoptosis). Most patients with ALPS have heterozygous mutations in the Fas gene. We describe an 11-year-old Brazilian boy with hepatosplenomegaly, lymphadenopathy, hemolytic anemia, and hypergammaglobulinemia since early infancy. T cell lines from the patient were defective in Fas-mediated apoptosis. He was diagnosed as having ALPS and found to have a novel Fas gene mutation (IVS4+1G>A). In addition, he presented with glomerulonephritis in infancy. An aunt and uncle who had the same Fas mutations also had histories of glomerulonephritis. Although glomerulonephritis is common in Fas-deficient mice, it is infrequent in human ALPS. Corticosteroid therapy ameliorated the glomerulonephritis in our patient, as well as his lymphoproliferation, anemia, and hypergammaglobulinemia. This study suggests that glomerulonephritis is one of the characteristic features of ALPS. PMID:12736807

  8. A case of successful management with splenectomy of intractable ascites due to congenital dyserythropoietic anemia type Ⅱ-induced cirrhosis

    Institute of Scientific and Technical Information of China (English)

    Themistoklis Vassiliadis; Ioannis Tsitouridis; Antonios Antoniadis; Panagiotis Semertzidis; Anna Kioumi; Evangelos Premetis; Nikolaos Eugenidis; Vassilia Garipidou; Vassilios Perifanis; Konstantinos Tziomalos; Olga Giouleme; Kalliopi Patsiaoura; Michalis Avramidis; Nikolaos Nikolaidis; Sofia Vakalopoulou

    2006-01-01

    The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis,characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type Ⅱ is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type Ⅱ. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.

  9. 头孢唑肟钠致急性溶血性贫血%Acute hemolytic anemia due to ceftizoxime sodium

    Institute of Scientific and Technical Information of China (English)

    张寒钰

    2015-01-01

    A 70-year-old man with pneumonia received an Ⅳ infusion of ceftizoxime sodium 3 g in 0.9% sodium chloride 250 ml twice daily.Twenty minutes after the first infusion, he developed low back pain, chills and confusion.He presented with moderately jaundiced skin and sclera.Laboratory tests revealed the following results : white blood cell (WBC) count 11 × 109/L, neutrophil 0.8, red blood cell (RBC) count 3.2 × 1012/L, hemoglobin 91 g/L, reticulocytes 0.081, total bilirubin 98 μmol/L, indirect bilirubin 80 μmol/L.Direct Coomb's test(+).He developed strong tea-colored urine.Routine urine tests showed the following results: urine bilirubin (+) , occult blood (+ + +), 0-2 red cells per high power field.Ceftizoxime sodium-induced acute hemolytic anemia was considered.He received hydration and alkalizing of urine.Ceftizoxime sodium was switched to fosfomycin.On day 5, the jaundice disappeared and the color of urine returned to normal.Laboratory tests showed the following results: WBC count 4 × 109/L,RBC count 4.2 × 1012/L, hemoglobin 131 g/L, reticulocytes 0.015, total bilirubin 17 μmol/L, indirect bilirubin 10 μmol/L, urine bilirubin (-), occult blood (-), 0-1 red cells per high power field.%1例70男性患者因肺炎给予头孢唑肟钠3 g加入0.9%氯化钠注射液250 ml静脉滴注,2次/d.首次给药后约20 min,患者突发腰痛、寒战、意识模糊.体格检查见皮肤巩膜中度黄染.实验室检查:外周血白细胞计数11×109/L,中性粒细胞0.8,红细胞计数3.2×1012/L,血红蛋白91 g/L,网织红细胞计数0.081,总胆红素98 μmol/L,间接胆红素80 μmol/L,直接抗人球蛋白试验阳性;尿呈浓茶色,胆红素(+),潜血(+++),红细胞0~2个/HP.考虑头孢唑肟钠致急性药物性溶血性贫血.给予水化及碱化尿液治疗.停用头孢唑肟钠,改用磷霉素抗感染.第5天复查,患者黄染消失、尿色恢复正常,白细胞计数4×109/L,红细胞计数4.2 ×1012/L,血红蛋白131 g

  10. Inborn anemias in mice

    International Nuclear Information System (INIS)

    hereditary anemias of mice have been the chief objects of investigation. At present under study are four macrocytic anemias, five hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an α-thalassemia, and a new target-cell anemia. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus our wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values, (b) determinations of radiosensitivity under a variety of conditions, (c) measurements of iron metabolism and heme synthesis, (d) histological and biochemical study of blood-forming tissue, (e) functional tests of the stem cell component, (f) examination of responses to erythroid stimuli, and (g) transplantation of tissue between individuals of differently affected genotypes

  11. Advances of the studies on lymphoid neoplasma associated anemia%淋巴系统肿瘤并发贫血的研究进展

    Institute of Scientific and Technical Information of China (English)

    王婷; 邵宗鸿

    2010-01-01

    Anemia is one of the most common complication and a negative prognostic factor of lymphoid neoplasma. Different cases with lymphoid neoplasma associated anemia (LNAA) have various pathogenesis such as hemorrhagic anemia, anemia of chronic disease, autoimmune hemolytic anemia and treatment related anemia, though they all have the same symptoms. So different regimens are given to different patients according to their pathogenesis of LNAA.%贫血是淋巴系统肿瘤(LN)常见的并发症之一,影响疾病预后.尽管贫血表象相同,但不同病例可能单独或同时存在失血性贫血、慢性病贫血、自身免疫性溶血性贫血和治疗相关性贫血等.机制各异,治疗方法及侧重点也不尽相同.

  12. Protective Effects of Royal Jelly and Vitamin C against Experimental Hemolytic Anemia on Sex Hormones and Histochemical Testicle Tissue Histochemistry of Adult Mice

    OpenAIRE

    H Anbara; R Shahrooz; H Malekinejad; S Saadati

    2016-01-01

    Introduction: Phenylhydrazine (PHZ) is a well-known hemolytic compound inducing intoxication in erythrocytes. Therefore, the present study aimed to evaluate the protective effects of royal jelly and vitamin C against phenylhydrazine-induced damages in mouse testicles. Methods: In this study, 64 adult male mice were randomly and equally assigned to eight groups. The first group received normal saline (0.1ml) intraperitoneally. The second group received PHZ (6 mg/100 gr) intraperitoneally i...

  13. The application of hematopoietic stem cell transplantation in treatment of congenital hemolytic anemia%造血干细胞移植在先天性溶血性贫血治疗中的应用

    Institute of Scientific and Technical Information of China (English)

    赖永榕

    2012-01-01

    造血干细胞移植(HSCT)是目前惟一能治愈先天性溶血性贫血(如重型地中海贫血和镰状细胞贫血)的治疗方法.HLA相合同胞供者移植治疗儿童患者的总生存(OS)率超过90%,无事件生存(EFS)率超过80%.无关供者移植使更多无HLA相合同胞供者的患者获得移植机会.%Hematopoietic stem cell transplantation (HSCT) remains the only treatment method that can cure congenital hemolytic anemia such as severe thalassemia and sickle cell anemia (SCA) at present. The overall survival ( OS) is over90% in children treated with HLA-identical sibling donor transplantation, while event-free survival (EFS) is over 80%. The e-vent-free donor transplantation enables more patients without HLA-identical sibling donors to obtain transplantation chances.

  14. Reação transfusional hiper-hemolítica em pacientes portadores de anemia falciforme: relato de dois casos Hyper-hemolytic transfusional reaction in sickle cell patients: two case reports

    Directory of Open Access Journals (Sweden)

    Cláudia C.S. Naufel

    2002-12-01

    increased frequency of transfusions to which these patients are submitted, knowledge of the main risks and an adequate diagnosis of the complications caused by transfusional therapy are of fundamental importance. An atypical form of transfusional reaction, denominated hyperhemolytic transfusional reaction was recently described in sickle cell anemia patients after the transfusion of apparently compatible hemacias. In this case, previous conditions can exacerbate the hemolytic condition and put the life of the patient at risk. The pathophysiological conditions of this disease are not yet understood well and the treatment consists of suspending transfusions, corticoid therapy and / or administration of immunoglobulin. The aim of this work is o present two case reports of hyperhemolytic transfusional reaction in sickle cell anemia patients.

  15. The IgG-specific endoglycosidase EndoS inhibits both cellular and complement-mediated autoimmune hemolysis

    OpenAIRE

    Allhorn, Maria; Briceño, Juana G.; Baudino, Lucie; Lood, Christian; Olsson, Martin L.; Izui, Shozo; Collin, Mattias

    2010-01-01

    EndoS from Streptococcus pyogenes is an immunomodulating enzyme that specifically hydrolyzes glycans from human immunoglobulin G and thereby affects antibody effector functions. Autoimmune hemolytic anemia is caused by antibody-mediated red blood cell (RBC) destruction and often resists treatment with corticosteroids that also cause frequent adverse effects. We show here that anti-RhD (anti-D) and rabbit anti–human-RBC antibodies (anti-RBC) mediated destruction of RBC, ie, phagocytosis, compl...

  16. Development of Hemolytic Anemia in a Nivolumab-Treated Patient with Refractory Metastatic Squamous Cell Skin Cancer and Chronic Lymphatic Leukemia.

    Science.gov (United States)

    Schwab, K S; Heine, A; Weimann, T; Kristiansen, G; Brossart, P

    2016-01-01

    Management of patients with metastatic squamous cell skin cancer, refractory to initial therapy with standard chemotherapy and radiation protocols, remains difficult with poor overall prognosis and limited therapeutic options. Recently, promising response rates with nivolumab, a programmed death receptor-1-blocking antibody, in squamous cancer of the head and neck have been demonstrated. Considering the similar histological patterns of squamous cell cancer of the skin and squamous cell cancer of the head and neck, we assumed that nivolumab could also be effective in our patients with refractory metastatic squamous cell cancer of the skin. So far, there have been no clinical data on the therapeutic efficacy of nivolumab in squamous cell skin cancer. We here present a case of a patient with metastatic squamous cell skin cancer refractory to previous therapies, who showed a good response to nivolumab over a period of 5 months, but developed a serious hemolytic crisis under nivolumab treatment after eight applications. PMID:27462240

  17. The major autoantibody epitope on factor H in atypical hemolytic uremic syndrome is structurally different from its homologous site in factor H-related protein 1, supporting a novel model for induction of autoimmunity in this disease.

    Science.gov (United States)

    Bhattacharjee, Arnab; Reuter, Stefanie; Trojnár, Eszter; Kolodziejczyk, Robert; Seeberger, Harald; Hyvärinen, Satu; Uzonyi, Barbara; Szilágyi, Ágnes; Prohászka, Zoltán; Goldman, Adrian; Józsi, Mihály; Jokiranta, T Sakari

    2015-04-10

    Atypical hemolytic uremic syndrome (aHUS) is characterized by complement attack against host cells due to mutations in complement proteins or autoantibodies against complement factor H (CFH). It is unknown why nearly all patients with autoimmune aHUS lack CFHR1 (CFH-related protein-1). These patients have autoantibodies against CFH domains 19 and 20 (CFH19-20), which are nearly identical to CFHR1 domains 4 and 5 (CFHR14-5). Here, binding site mapping of autoantibodies from 17 patients using mutant CFH19-20 constructs revealed an autoantibody epitope cluster within a loop on domain 20, next to the two buried residues that are different in CFH19-20 and CFHR14-5. The crystal structure of CFHR14-5 revealed a difference in conformation of the autoantigenic loop in the C-terminal domains of CFH and CFHR1, explaining the variation in binding of autoantibodies from some aHUS patients to CFH19-20 and CFHR14-5. The autoantigenic loop on CFH seems to be generally flexible, as its conformation in previously published structures of CFH19-20 bound to the microbial protein OspE and a sialic acid glycan is somewhat altered. Cumulatively, our data suggest that association of CFHR1 deficiency with autoimmune aHUS could be due to the structural difference between CFHR1 and the autoantigenic CFH epitope, suggesting a novel explanation for CFHR1 deficiency in the pathogenesis of autoimmune aHUS. PMID:25659429

  18. Evaluation of stem cell reserve using serial bone marrow transplantation and competitive repopulation in a murine model of chronic hemolytic anemia

    International Nuclear Information System (INIS)

    Serial transplantation and competitive repopulation were used to evaluate any loss of self-replicative capacity of bone marrow stem cells in a mouse model with increased and persistent hemopoietic demands. Congenic marrows from old control and from young and old mice with hereditary spherocytic anemia (sphha/sphha) were serially transplanted at 35-day intervals into normal irradiated recipients. Old anemic marrow failed or reverted to recipient karyotype at a mean of 3.5 transplants, and young anemic marrow reverted at a mean of 4.0 transplants, whereas controls did so at a mean of 5.0 transplants. In a competitive assay in which a mixture of anemic and control marrow was transplanted, the anemic marrow persisted to 10 months following transplantation; anemic marrow repopulation was greater if anemic marrow sex matched with the host. It is possible that lifelong stress of severe anemia decreases stem cell reserve in the anemic sphha/sphha mouse marrow. However, marginal differences in serial transplantation number and the maintenance of anemic marrow in a competition assay would suggest that marrow stem cells, under prolonged stress, are capable of exhibiting good repopulating and self-replicating abilities

  19. Evaluation of stem cell reserve using serial bone marrow transplantation and competitive repopulation in a murine model of chronic hemolytic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Maggio-Price, L.; Wolf, N.S.; Priestley, G.V.; Pietrzyk, M.E.; Bernstein, S.E.

    1988-09-01

    Serial transplantation and competitive repopulation were used to evaluate any loss of self-replicative capacity of bone marrow stem cells in a mouse model with increased and persistent hemopoietic demands. Congenic marrows from old control and from young and old mice with hereditary spherocytic anemia (sphha/sphha) were serially transplanted at 35-day intervals into normal irradiated recipients. Old anemic marrow failed or reverted to recipient karyotype at a mean of 3.5 transplants, and young anemic marrow reverted at a mean of 4.0 transplants, whereas controls did so at a mean of 5.0 transplants. In a competitive assay in which a mixture of anemic and control marrow was transplanted, the anemic marrow persisted to 10 months following transplantation; anemic marrow repopulation was greater if anemic marrow sex matched with the host. It is possible that lifelong stress of severe anemia decreases stem cell reserve in the anemic sphha/sphha mouse marrow. However, marginal differences in serial transplantation number and the maintenance of anemic marrow in a competition assay would suggest that marrow stem cells, under prolonged stress, are capable of exhibiting good repopulating and self-replicating abilities.

  20. Anemia hemolítica autoinmune postinfección por virus de la hepatitis A. Informe de caso; Autoimmune haemolytic anaemia associated to hepatitis A. Case report

    Directory of Open Access Journals (Sweden)

    Claudia Lucía Sossa Melo, MD

    2010-01-01

    Full Text Available La anemia hemolítica autoinmune se asocia con una variedad de virus hepatotrópicos, en particular citomegalovirus (CMV, virus del Epstein-Barr y de la hepatitis B. No es frecuente dentro de la historia natural de la hepatitis A, la aparición de anemia hemolítica, y cuando se presenta, generalmente se asocia a deficiencia de glucosa-6-fosfato deshidrogenasa. Presentamos el caso de un paciente de sexo masculino sin hemólisis previa, con astenia e ictericia de dos meses de evolución y hepatomegalia 14 cm por debajo del reborde costal derecho. Los hallazgos en los exámenes de laboratorios mostraron anemia hemolítica con Coombs directo positivo, anticuerpos tipo inmunoglobulina M contra el virus de la hepatitis A positivos, niveles de bilirrubinas 20 veces y aminotrasferasas cuatro veces por arriba del rango normal; con estos datos el paciente fue diagnosticado como hepatitis A complicada con anemia hemolítica y probable hepatitis autoinmune asociada, por lo que se inició manejo con corticoides, alcanzándose mejoría clínica. Resaltamos la importancia de descartar la infección por el virus de la hepatitis A como posible etiología de anemia hemolítica autoinmune.______________________________________________________________________ Acute auto inmune haemolytic anaemia is associated with a variety of hepatotropic viruses, in particular cytomegalovirus, Epstein Barr virus and hepatitis B. The typical course of hepatitis A is rarely complicated with glucose-6-phosphate dehydrogenase deficiency. Wepresent the case of a man without previous haemolysis, he had been unwell for two months with fatigue and jaundice, the liver edge was palpable and tender 14 cm below the costal margin. Clinical chemistry showed haemolytic anaemia with positive direct coombs test, immunoglobulin M antibodies to hepatitis A virus were detected, the total bilirrubin concentration 20 times the upper and transaminase 4 times upper limit for normal levels; with this

  1. Protective Effects of Royal Jelly and Vitamin C against Experimental Hemolytic Anemia on Sex Hormones and Histochemical Testicle Tissue Histochemistry of Adult Mice

    Directory of Open Access Journals (Sweden)

    H Anbara

    2016-03-01

    Full Text Available Introduction: Phenylhydrazine (PHZ is a well-known hemolytic compound inducing intoxication in erythrocytes. Therefore, the present study aimed to evaluate the protective effects of royal jelly and vitamin C against phenylhydrazine-induced damages in mouse testicles. Methods: In this study, 64 adult male mice were randomly and equally assigned to eight groups. The first group received normal saline (0.1ml intraperitoneally. The second group received PHZ (6 mg/100 gr intraperitoneally in 48-hour intervals. The third group received vitamin C (250 mg/kg/day intraperitoneally perday a long with PHZ. The fourth group received royal jelly (100 mg/kg/day through gavage. The fifth group received PHZ along with vitamin C and royal jelly in similar doses to the previous groups. The sixth group received only vitamin C, the seventh group recieved only royal jelly, and finally the eighth group received similar doses of vitamin C and royal jelly. After 35 days, serum and tissue samples were taken and used for histochemical (Mallory-Azan, Alkaline phosphatase, Oil red-O and PAS, and serum analyses (Testosterone, LH, FSH. Results: The study results revealed the histochemical changes in testicular tissue of the phenylhydrazine group, in which vitamin C and royal jelly partly improved the changes. Furthermore, serum analyses demonstrated a significant decrease in testosterone, FSH and LH levels, which this decrease was diminished by royal jelly and vitamin C. Conclusions: Royal jelly and vitamin C seem to have the potential to decrease serum and tissue damages induced by phenylhydrazine via restraining free radicals.

  2. [Hemolytic uremic syndrome. Clinical manifestations. Treatment].

    Science.gov (United States)

    Exeni, Ramón A

    2006-01-01

    Clinical manifestation are described in children with epidemic HUS. The intestinal involvement in the prodromic period, is outlined and the most common disturbances such acute renal failure, thrombocytopenia, hemolytic anemia, leucocitosis hypertension, neurological, pancreatic and cardiac manifestations are described. We discuss the acid-base and electrolyte disturbances, metabolic acidosis, hyponatremia, hyperkalemia. The etiopathogenic treatment and the control of renal sequelae are also discussed.

  3. The incidence of autoimmune hemolytic anemia in pediatric hematopoietic stem cell recipients post-first and post-second hematopoietic stem cell transplant.

    Science.gov (United States)

    Ahmed, Ibrahim; Teruya, Jun; Murray-Krezan, Cristina; Krance, Robert

    2015-06-01

    The reported incidence of post-allogeneic HSCT AIHA was between 4.4% and 6% following a single transplant. Cord blood transplantation, T-cell depletion, and chronic GvHD are significantly associated with post-transplant AIHA. During an 11-yr period, data for 500 pediatric HSCT recipients were eligible for evaluation of the incidence of AIHA post-first and post-second transplants. Demographic, transplant, and post-transplant-related variables were analyzed. Twelve of 500 (2.4%) recipients at a median of 273 days and seven of 72 (9.7%) recipients at a median of 157 days developed AIHA post-first and post-second HSCT, respectively. Post-first HSCT, none of the MRD recipients developed AIHA (0/175 MRD vs. 12/325 other donors, p = 0.04). Four of 12 required a second HSCT to control the AIHA. After the second HSCT, MUD was significantly associated with the development of AIHA. No other variables were associated with the post-second transplant AIHA. The incidence of AIHA post-first and post-second HSCT was less than the reported. The increased incidence of AIHA among recipients of second HSCT is most likely due to the profound immune dysregulation. A much larger, prospective study would be needed to evaluate the incidence, complications, and management of post-transplant AIHA.

  4. Autosomal Recessive Chronic Granulomatous Disease, IgA Deficiency and Refractory Autoimmune Thrombocytopenia Responding to Anti-CD20 Monoclonal Antibody

    Directory of Open Access Journals (Sweden)

    Shahin Shamsian Bibi

    2008-09-01

    Full Text Available Immunodeficiency and autoimmune disease may occur concomitantly in the same individual. Some of the immunodeficiency syndromes, especially humoral defects are associated with autoimmune disorders. Hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. Persistent antigen stimulation due to an inherent defect in the ability of the immune system to eradicate pathogens is the primary cause leading to autoimmunity in patients with primary immunodeficiency states.We describe a 10 year old Iranian girl with chronic granulomatous disease -the autosomal recessive type with mutation of NCF1 gene P47- associated with selective IgA deficiency, refractory immune thrombocytopenia that showed an excellent response to Rituximab (Anti-CD20 monoclonal antibody.Patients with primary immunodeficiencies may have variable autoimmune manifestations. So for early detection and appropriate treatment, autoimmune diseases should always be suspected in such patients.

  5. 坏死性淋巴结炎合并桥本甲状腺炎及自身免疫性溶血性贫血1例%Necrotizing lymphadenitis accompanying Hashimoto's thyroiditis and autoimmune hemolytic anemia:A case report

    Institute of Scientific and Technical Information of China (English)

    赵春洪; 张志强; 肖红菊; 刘刚

    2013-01-01

    Objective To study the diagnosis and treatment of histiocytic necrotizing lymphadenitis (HNL) accompanying Hashimoto's thyroiditis (HT) and autoimmune hemolytic anemia (AIHA) by reporting a case of unknown origin fever and reviewing its related literature.Methods The clinical data about a patient with HNL accompanying HT and AIHA admitted to the Department of Fever-Related Diseases in our hospital asa case of unknown origin fever were retrospectively analyzed with its related literature reviewed.Results The patient was a 56-year-old female manifested as unknown origin fever accompanying arthralgia, myalgia, sore-throat, transient skin rash, thyroid enlargement, axillary and inguinal lymph node enlargement, anemia, and was diagnosed as HNL accompanying HT and AIHA according to its clinical manifestations, pathological examination and bone marrow biopsy. Conclusion HNL accompanying HT and AIHA is rarely encountered in clinical practice. HNL and autoimmune diseases should be considered in patients with fever of unknown origin. Its diagnosis depends mainly on its pathological examination and its treatment depends mainly on glucocorticoid.%目的:通过对以不明原因发热就诊的组织细胞坏死性淋巴结炎合并桥本甲状腺炎及自身免疫性溶血性贫血的病例报道并文献复习,探讨诊治方法。方法分析本院发热疾病科收治的1例以不明原因发热就诊的坏死性淋巴结炎合并桥本甲状腺炎及自身免疫性溶血性贫血患者的临床资料并复习相关文献,总结诊治经验。结果患者56岁女性,临床表现为不明原因发热伴关节肌肉痛、咽痛、一过性皮疹、甲状腺肿大、腋窝及腹股沟淋巴结肿大、贫血,根据其临床表现、病理及骨髓穿刺结果确诊为坏死性淋巴结炎合并桥本甲状腺炎及自身免疫性溶血性贫血。结论此例病症临床罕见,对不明原因发热的患者应想到坏死性淋巴结炎及多种自身免疫性疾

  6. Atypical Hemolytic Uremic Syndrome Secondary to Lupus Nephritis, Responsive to Eculizumab

    Science.gov (United States)

    Raufi, Alexander G.; Scott, Shruti; Darwish, Omar; Harley, Kevin; Kahlon, Kanwarpal; Desai, Sheetal; Lu, Yuxin; Tran, Minh-Ha

    2016-01-01

    Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) – a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage. Although plasma exchange is considered first-line therapy for thrombotic thrombocytopenic purpura – a TMA classically associated with autoimmune depletion of ADAMTS-13 – aHUS demonstrates less reliable responsiveness to this modality. Instead, use of the late complement inhibitor Eculizumab has emerged as an effective modality for the management of such patients. Diagnosis of aHUS, however, is largely clinically based, relying heavily upon a multidisciplinary approach. Herein we present the case of a patient with atypical HUS successfully treated with Eculizumab in the setting of Class IV-G (A) lupus nephritis and hypocomplementemia.

  7. Hemolytic Uremic Syndrome in Children

    Science.gov (United States)

    ... KB)​​​​​ Alternate Language URL Hemolytic Uremic Syndrome in Children Page Content On this page: What is hemolytic ... spine. [ Top ] What causes hemolytic uremic syndrome in children? The most common cause of hemolytic uremic syndrome ...

  8. Autoimmunity in Wiskott-Aldrich Syndrome: an unsolved enigma

    Directory of Open Access Journals (Sweden)

    Marco eCatucci

    2012-07-01

    Full Text Available Wiskott-Aldrich Syndrome (WAS is a severe X-linked Primary Immunodeficiency (PID that affects 1 to 10 out of 1 million male individuals. WAS is caused by mutations in the WAS Protein (WASP expressing gene that leads to the absent or reduced expression of the protein. WASP is a cytoplasmic protein that regulates the formation of actin filaments in hematopoietic cells. WASP deficiency causes many immune cell defects both in humans and in the WAS murine model, the Was-/- mouse. Both cellular and humoral immune defects in WAS patients contribute to the onset of severe clinical manifestations, in particular microthrombocytopenia, eczema, recurrent infections and a high susceptibility to develop autoimmunity and malignancies. Autoimmune diseases affect from 22% to 72% of WAS patients and the most common manifestation is autoimmune hemolytic anemia, followed by vasculitis, arthritis, neutropenia, inflammatory bowel disease and IgA nephropathy. Many groups have widely explored immune cell functionality in WAS partially explaining how cellular defects may lead to pathology. However, the mechanisms underlying the occurrence of autoimmune manifestations have not been clearly described yet. In the present review, we report the most recent progresses in the study of immune cell function in WAS that have started to unveil the mechanisms contributing to autoimmune complications in WAS patients.

  9. Two Cases of Primary Cold Agglutinin Disease Associated with Megaloblastic Anemia

    Directory of Open Access Journals (Sweden)

    Shinsaku Imashuku

    2015-01-01

    Full Text Available We report two cases of primary cold agglutinin disease (CAD associated with megaloblastic anemia in Japanese elderly patients. Case 1 was a 67-year-old male and Case 2 was a 55-year-old male. Both patients were diagnosed with primary CAD, with continuously high cold agglutinin titers (1 : >8,192 and 1 : 16,834, resp., monoclonal IgM-kappa light chains, and no underlying disease. In addition, both patients had megaloblastic anemia due to vitamin B12 deficiency. One patient received rituximab and both received vitamin 12 supplementation. To date, no cooccurrence of primary CAD and megaloblastic anemia has been emphasized. Thus, the association of these hematological diseases may be incidental; however, given that CAD is an autoimmune disease which may show antibodies against intrinsic factor and gastric parietal cells, this association was thought to be probably not a coincidence. Clinicians should be aware of the possible simultaneous presence of autoimmune hemolytic/megaloblastic anemia in patients with primary CAD.

  10. Two cases of primary cold agglutinin disease associated with megaloblastic anemia.

    Science.gov (United States)

    Imashuku, Shinsaku; Kudo, Naoko; Takagishi, Katsushige; Saigo, Katsuyasu

    2015-01-01

    We report two cases of primary cold agglutinin disease (CAD) associated with megaloblastic anemia in Japanese elderly patients. Case 1 was a 67-year-old male and Case 2 was a 55-year-old male. Both patients were diagnosed with primary CAD, with continuously high cold agglutinin titers (1 : >8,192 and 1 : 16,834, resp.), monoclonal IgM-kappa light chains, and no underlying disease. In addition, both patients had megaloblastic anemia due to vitamin B12 deficiency. One patient received rituximab and both received vitamin 12 supplementation. To date, no cooccurrence of primary CAD and megaloblastic anemia has been emphasized. Thus, the association of these hematological diseases may be incidental; however, given that CAD is an autoimmune disease which may show antibodies against intrinsic factor and gastric parietal cells, this association was thought to be probably not a coincidence. Clinicians should be aware of the possible simultaneous presence of autoimmune hemolytic/megaloblastic anemia in patients with primary CAD. PMID:25918651

  11. Clinical indications and biological mechanisms of splenic irradiation in autoimmune diseases

    Energy Technology Data Exchange (ETDEWEB)

    Weinmann, M.; Becker, G. [Tuebingen Univ. (Germany). Abt. fuer Strahlenonkologie; Einsele, H.; Bamberg, M. [Tuebingen Univ. (Germany). Abt. fuer Innere Medizin 2

    2001-02-01

    Background: Splenic irradiation (SI) is a fairly unknown treatment modality in autoimmune disorders like autoimmune thrombocytopenia (AIT) or autoimmune hemolytic anemia (AIHA), which may provide an effective, low toxic and cost-effective treatment for selected patients. Patients, Materials and Methods: This article reviews the limited experiences on splenic irradiation in autoimmune thrombocytopenia by analyzing the current studies including 71 patients and some preliminary reports on splenic irradiation in autoimmune hemolytic anemia. Results: In autoimmune thrombocytopenia between 40 and 90% of all patients responded, but most of them relapsed within 4 to 6 months after splenic irradiation. Between 10 and 20% of all patients had a sustained response. The efficacy of splenic irradiation in HIV-associated cases of thrombocytopenia is probably lower than in other forms of autoimmune thrombocytopenia, but especially in this group immunosuppressive drug treatment of autoimmune thrombocytopenia exposes some problems. In autoimmune hemolytic anemia there are some case reports about efficacy of splenic irradiation. Toxicity of splenic irradiation in both diseases was very moderate. Conclusions: For HIV patients, for elderly patients or patients at high risk for complications following splenectomy splenic irradiation might be a treatment option. Splenic irradiation as preoperative treatment in patients not responding to or not suitable for immunosuppressive drugs prior to splenectomy may be a promising new application of splenic irradiation to reduce adverse effects of splenectomy in thrombocytopenic patients. A further analysis of the biological mechanisms underlying splenic irradiation may help to improve patient selection, to optimize dose concepts and treatment schedules and will improve understanding of radiotherapy as an immunomodulatory treatment modality. (orig.) [German] Hintergrund: Die Bestrahlung der Milz zur Behandlung von haematologischen

  12. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation

    Directory of Open Access Journals (Sweden)

    Eiji Matsukuma

    2014-01-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS can be distinguished from typical or Shiga-like toxin-induced HUS. The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase. Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions. aHUS in the nontransplant postsurgical period, however, is rare. An 8-month-old boy underwent surgical repair of tetralogy of Fallot. Neurological disturbances, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia developed 25 days later, and aHUS was diagnosed. Further evaluation revealed that his complement factor H (CFH level was normal and that anti-FH antibodies were not detected in his plasma. Sequencing of his CFH, complement factor I, membrane cofactor protein, complement factor B, and thrombomodulin genes was normal. His ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin-1 repeats 13 activity was also normal. However, he had a potentially causative mutation (R425C in complement component C3. Restriction fragment length polymorphism analysis revealed that his father and aunt also had this mutation; however, they had no symptoms of aHUS. We herein report a case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation.

  13. Postoperative atypical hemolytic uremic syndrome associated with complement c3 mutation.

    Science.gov (United States)

    Matsukuma, Eiji; Imamura, Atsushi; Iwata, Yusuke; Takeuchi, Takamasa; Yoshida, Yoko; Fujimura, Yoshihiro; Fan, Xinping; Miyata, Toshiyuki; Kuwahara, Takashi

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) can be distinguished from typical or Shiga-like toxin-induced HUS. The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase. Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions. aHUS in the nontransplant postsurgical period, however, is rare. An 8-month-old boy underwent surgical repair of tetralogy of Fallot. Neurological disturbances, acute renal failure, thrombocytopenia, and microangiopathic hemolytic anemia developed 25 days later, and aHUS was diagnosed. Further evaluation revealed that his complement factor H (CFH) level was normal and that anti-FH antibodies were not detected in his plasma. Sequencing of his CFH, complement factor I, membrane cofactor protein, complement factor B, and thrombomodulin genes was normal. His ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin-1 repeats 13) activity was also normal. However, he had a potentially causative mutation (R425C) in complement component C3. Restriction fragment length polymorphism analysis revealed that his father and aunt also had this mutation; however, they had no symptoms of aHUS. We herein report a case of aHUS that developed after cardiovascular surgery and was caused by a complement C3 mutation. PMID:25431709

  14. Diagnosis and management of rare congenital nonimmune hemolytic disease.

    Science.gov (United States)

    Gallagher, Patrick G

    2015-01-01

    Rare, congenital nonimmune hemolytic disorders of the erythrocyte, although uncommon, are important causes of anemia in the child and adult. These are a heterogeneous group of diseases that disrupt normal erythrocyte structure and function in varying ways. Predominant are abnormalities of hemoglobin stability, defects of erythrocyte metabolism, and disorders of erythrocyte hydration. Unstable hemoglobinopathies may lead to chronic or episodic hemolysis. Perturbation of critical enzymes of the Embden-Meyerhof pathway lead to altered erythrocyte metabolism and chronic hemolysis. Disorders of erythrocyte hydration are an under-recognized cause of hemolytic anemia. Beyond pathophysiologic mechanisms of disease, clinical, laboratory, and genetic heterogeneity characterize this group of disorders. Often, they are underdiagnosed or misdiagnosed. This review discusses pathophysiology, inheritance, clinical findings, laboratory manifestations, and management considerations in several rare nonimmune hemolytic diseases including the unstable hemoglobins, disorders of erythrocyte metabolism, and abnormalities of erythrocyte hydration. PMID:26637748

  15. Tissue Factor and Thrombin in Sickle Cell Anemia

    OpenAIRE

    Chantrathammachart, Pichika; Pawlinski, Rafal

    2012-01-01

    Sickle cell anemia is an inherited hematologic disorder associated with hemolytic and vaso-occlusive complications. An activation of coagulation is also a prominent feature of sickle cell anemia. Growing evidence indicates that coagulation may contribute to the inflammation and vascular injury in sickle cell anemia. This review focuses on tissue factor expression and its contribution to the activation of coagulation, thrombosis and vascular inflammation in sickle cell anemia.

  16. AUTOIMMUNE CYTOPENIAS IN CHRONIC LYMPHOCYTIC LEUKEMIA, FACTS AND MYTHS

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    Pavankumar Tandra

    2013-11-01

    Full Text Available CLL has been defined as presence of more than 5000 small mature appearing monoclonal B lymphocytes with a specific immunophenotype in peripheral blood. It is a well-known fact that CLL is associated with autoimmune cytopenias. CLL cells are CD5+ B lymphocytes, and usually are not the “guilty” cells which produce autoantibodies. T cell defect is another characteristic of CLL and the total number of T cells is increased, and there is inversion of the CD4/CD8 ratio. Autoimmune hemolytic anemia (AIHA is the most common autoimmune complication of CLL and has been reported in 10-25% of CLL patients. However, the stage-adjusted estimated rate of AIHA in CLL is about 5%. Conversely, CLL is three times more common in patients who present with AIHA. Direct agglutinin test (DAT is positive in 7-14% of CLL patients but AIHA may also occur in DAT negative patients. Autoimmune thrombocytopenia (AIT is the second most common complication of CLL and has been reported in 2-3% of patients. DAT is positive in AIT but presence of antiplatelet antibodies is neither diagnostic nor reliable. Autoimmune neutropenia (AIN and pure red cell aplasia (PRCA are very rare complications of CLL and like other autoimmune complications of CLL may occur at any clinical stage. It is believed that most case reports of AIN and PRCA in CLL actually belong to large granular lymphocytic leukemia (LGL. Non-hematologic autoimmune complications of CLL including cold agglutinin disease (CAD, paraneoplastic pemphigus (PNP, acquired angioedema, and anti-myelin associated globulin are rare. Before starting any treatment, clinicians should distinguish between autoimmune cytopenias and massive bone marrow infiltration since autoimmune complications of CLL are not necessarily equal to advanced disease with poor prognosis. According to IWCLL guideline, steroids are the mainstay of treatment of simple autoimmunity. Intravenous immunoglobulin (IVIg, cyclosporine, and rituximab are used in

  17. [Autoimmune lymphoproliferative syndrome: a case report and literature review].

    Science.gov (United States)

    Sun, Jia-peng; Lu, Xin-tian; Zhao, Wei-hong; Hua, Ying

    2015-12-18

    We described 1 case of autoimmune lymphoproliferative syndrome (ALPS), first diagnosed in our hospital, and reviewed the recent literature. The 11-month old male patient presented with a history of splenomegaly and hepatomegaly since 1 month after birth. He suffered recurrent infectious diseases including cytomegalovirus infection, parvovirus B19 infection and chronic diarrhea disease. Besides, his symptoms included hemolytic anemia and thrombocytopenia. The laboratory abnormality indicated an expanded population of alpha/beta double-negative T cells (DNTs) (27.18% of lymphocytes, 35.16% of CD3+ T lymphocytes) in peripheral blood, and autoantibodies including antinuclear antibody, double-stranded DNA and rheumatic factor were positive. Hyper gamma globulinemia and positive direct Coombs tests were seen in the patient. His parents were both healthy and denied autoimmune diseases. We identified a heterozygous point mutation in exon 3 of the FAS gene carrying c.309 A>C, resulting in a single base pair substitution in exon 3 of FAS gene which changed the codon of Arg103 to Ser103. Unfortunately, we were unable to obtain the gene results of the child's parents. The patient was treated with glucocorticoids in our hospital and with mycophenolatemofetil in other hospital. And we were informed that his anemia condition relieved through the telephone follow-up, but he still suffered recurrent infections, hepatomegaly and splenomegaly still existed. As we all know ALPS is characterized by defective lymphocyte apoptosis, and thus cause lymphoproliferative disease and autoimmune disease, and increase the risk of lymphoma. It is more likely to be misdiagnosed as other diseases. ALPS should be suspected in the case of chronic lymphadenopathy, splenomegaly and autoimmune features. Flow cytometry approach is helpful for the diagnosis. Immunosuppressive drugs are the necessary treatment. PMID:26679669

  18. 头孢曲松钠致急性溶血性贫血伴多器官功能衰竭%Ceftriaxone sodium-induced acute hemolytic anemia with multiorgan failure

    Institute of Scientific and Technical Information of China (English)

    黄玉玲; 杨辉; 许沧海

    2012-01-01

    A 68-year-old woman with paronychia received an IV infusion of ceftriaxone sodium 4. 0 g in 0. 9% sodium chloride 250 ml once daily. She developed low back pain during drug therapy. On day 3, she was hospitalized with transient syncope and hematuria. On admission, she presented with severely jaundiced skin and sclera. Laboratory tests revealed the following results: white blood cell count 39.4 × 109/L, neutrophil 0. 97, red blood cell ( RBC ) count 1. 9 × 1012/L, hemoglobin ( Hb ) 42 g/L, reticulocytes 0.03, blood urea nitrogen 15.0 mmol/1, serum creatinine ( SCr ) 336 μmol/L, alanine aminotransferase ( ALT) 380 U/L, aspartate aminotransferase ( AST ) 930 U/L, gamma-glutamyltransferase ( γ-GT ) 60 U/L, total bilirubin ( TBil ) 235. 1 μmol/L, direct bilirubin ( DBil ) 130. 6 μmol/L, and indirect bilirubin ( IBil ) 104. 5 μmol/L, lactate dehydrogenase 2198 U/L, creatine kinase 211 U/L, α-hydroxybutyric dehydrogenase 1538 U/L, creatine kinase-MB 22 U/L, prothrombin time 21. 3 seconds, activated partial thromboplastin time 50. 5 seconds, thrombin time 29. 6 seconds, fibrinogen 1.03 g/L, D-dimer 10. 4 mg/L, and Coomb' s C3 ( + ). Routine urine tests showed the following results: urine bilirubin( + + ), occult blood ( + + + ), protein( + + ) and 3 RBC/high power field. Ceftriaxone sodium-induced acute hemolytic anemia with multiorgan failure was considered. An IV injection of human immunoglobulin and glucocorticoid was given. She received continuous renal replacement therapy, hemodialysis, plasma exchange, repeated transfusions of washed red blood cells, fresh frozen plasma and cryoprecipitate, urinary alkalinization, liver protection, and other symptomatic treatment. On day 57, laboratory testing showed the following levels: RBC count 4. 0 × 1012/L,Hb 112 g/L. SCr 110 μmol/L, ALT 13 U/L, AST 20 U/L, TBil 11.7 μmol/L, DBil 4. 5 μmol/L, IBil 7. 2 μmol/L. Her symptoms improved and she was discharged.%1例68岁女性患者因甲沟炎给予头孢曲松钠4 g

  19. Anemia hemolítica autoinmune postinfección por virus de la hepatitis A. Informe de caso; Autoimmune haemolytic anaemia associated to hepatitis A. Case report

    OpenAIRE

    Claudia Lucía Sossa Melo, MD; Sara Inés Jiménez Sanguino, MD; Carlos Andrés Pérez Martínez, MD; Amaury Alexis Amaris Vergara, MD; Luis Antonio Salazar Montaña, MD; Ángela Peña Castellanos, MD; Jesica Liliana Pinto Ramírez; Laura Andrea Rincón Arenas

    2010-01-01

    La anemia hemolítica autoinmune se asocia con una variedad de virus hepatotrópicos, en particular citomegalovirus (CMV), virus del Epstein-Barr y de la hepatitis B. No es frecuente dentro de la historia natural de la hepatitis A, la aparición de anemia hemolítica, y cuando se presenta, generalmente se asocia a deficiencia de glucosa-6-fosfato deshidrogenasa. Presentamos el caso de un paciente de sexo masculino sin hemólisis previa, con astenia e ictericia de dos meses de evolución y hepatomeg...

  20. Understanding anemia of chronic disease.

    Science.gov (United States)

    Fraenkel, Paula G

    2015-01-01

    The anemia of chronic disease is an old disease concept, but contemporary research in the role of proinflammatory cytokines and iron biology has shed new light on the pathophysiology of the condition. Recent epidemiologic studies have connected the anemia of chronic disease with critical illness, obesity, aging, and kidney failure, as well as with the well-established associations of cancer, chronic infection, and autoimmune disease. Functional iron deficiency, mediated principally by the interaction of interleukin-6, the iron regulatory hormone hepcidin, and the iron exporter ferroportin, is a major contributor to the anemia of chronic disease. Although anemia is associated with adverse outcomes, experimental models suggest that iron sequestration is desirable in the setting of severe infection. Experimental therapeutic approaches targeting interleukin-6 or the ferroportin-hepcidin axis have shown efficacy in reversing anemia in either animal models or human patients, although these agents have not yet been approved for the treatment of the anemia of chronic disease.

  1. Hemolytic-Uremic Syndrome Associated with Gemcitabine: A Case Report and Review of Literature

    Directory of Open Access Journals (Sweden)

    McGee PJ

    2005-07-01

    Full Text Available CONTEXT: Hemolytic uremic syndrome is a rare condition compromising the clinical triad of acute renal failure, microangiopathic hemolytic anemia, and thrombocytopenia. Hemolytic uremic syndrome may be associated with a variety of etiologies, and chemotherapeutic agents have also been reported to be associated with hemolytic uremic syndrome, including mitomycin, cisplatin, bleomycin, and most recently gemcitabine. CASE REPORT: A 72-year-old Caucasian male treated with four cycles of gemcitabine at 1,000 mg/m2 developed clinical and laboratory findings compatible with hemolytic uremic syndrome. He developed microangiopathic hemolysis, rapidly declining renal function with proteinuria and hematuria, and renal biopsy revealed thrombotic microangiopathy. Hemodialysis, plasmapheresis, and corticosteroid therapy were utilized but the process ultimately was irreversible. CONCLUSION: With multiple reports of hemolytic uremic syndrome complicating gemcitabine therapy, it is imperative that clinicians heighten their awareness of this potentially lethal complication.

  2. ANEMIA IN INFLAMMATORY BOWEL DISEASE MORE THAN AN EXTRAINTESTINAL COMPLICATION.

    Science.gov (United States)

    Nemeş, Roxana Maria; Pop, Corina Silvia; Calagiu, Dorina; Dobrin, Denisa; Chetroiu, Diana; Jantea, Petruta; Postolache, Paraschiva

    2016-01-01

    The most common hematologic complication of inflammatory bowel disease (IBD)--ulcerative colitis and Crohn's Disease is anemia. Anemia in patients with IBD may be a result of iron, vitamin B12 or folate deficiency; anemia of chronic disease and hemolytic anemia are other causes in these patients. Factors contributing to the development of anemia include chronic gastrointestinal blood loss, vitamin B12 malabsorption secondary to terminal ileitis, folate deficiency as a result of sulfasalazine therapy. Approximately 30% of patients with IBD have hemoglobin levels below 12 g/dl. The risk of developing anemia relates to disease activity, given that blood loss and inflammatory anemia are triggered by intestinal inflammation. In the management strategy of IBD patients with anemia it is important to distinguish between the different types of anemia in order to decide an appropriate manner of treatment.

  3. Cold agglutinin disease (CADwith autoimmune haemolytic anaemia: a case report of a coronary artery disease patient Doença por aglutininas a frio (DAC com anemia hemolítica auto-imune: relato de caso de um coronariopata

    Directory of Open Access Journals (Sweden)

    Leandro A. Barbosa

    2008-02-01

    Full Text Available Cold agglutinin disease (CAD with autoimmune haemolytic anemia is characterized by the production of harmful cold autoantibodies associated with increased red cell destruction during exposure to cold. The treatment of CAD is very difficult and a great effort is required to obtain therapeutic success. Cyclophosphamide is a potent immunosuppressive agent which is widely used in all bone marrow transplantation conditioning regimens for patients with acquired severe aplastic anemia. In this report, we describe the case of a coronary artery disease patient with severe CAD, but without lymphoproliferative disease, in which general measures and immunosuppressive therapies were adopted, there by avoiding blood transfusions.A doença por aglutininas a frio (CAD cursando com anemia hemolítica auto-imune (AHAI é decorrente da produção de autoanticorpos que reagem muito bem a baixas temperaturas, dirigidos contra hemácias autólogas. A habilidade desses anticorpos em destruir as hemácias encontra-se diretamente relacionada à sua capacidade em fixar complemento durante a exposição do paciente a baixas temperaturas. A AHAI por anticorpos frios pode ser idiopática - ausência de doença de base - ou secundária, geralmente associada a desordens linfoproliferativas de células B ou determinados processos infecciosos. A hemólise é intravascular, através de aglutininas da classe IgM, com teste direto da antiglobulina humana positivo para complemento. O tratamento da CAD é difícil, exigindo um esforço contínuo, necessário para se obter sucesso terapêutico. A ciclofosfamida é um agente imunossupressor potente, amplamente utilizado em transplantes de medula óssea, particularmente nos portadores de anemia aplástica. Descrevemos o caso de um coronariopata portador de CAD severa, cuja exploração diagnóstica excluiu doença linfoproliferativa. Adotamos medidas gerais de suporte e terapia imunossupressora, coibindo o uso de hemotransfusões.

  4. Anemia (For Parents)

    Science.gov (United States)

    ... Story" 5 Things to Know About Zika & Pregnancy Anemia KidsHealth > For Parents > Anemia Print A A A ... With Anemia Preventing Anemia en español Anemia About Anemia Anemia, one of the more common blood disorders, ...

  5. An overlap syndrome involving autoimmune hepatitis and systemic lupus erythematosus in childhood

    Institute of Scientific and Technical Information of China (English)

    Yusuf Usta; Figen Gurakan; Zuhal Akcoren; Seza Ozen

    2007-01-01

    We report a 12 years old female patient with an overlap syndrome involving autoimmune hepatitis (ALM) and systemic lupus erythematosus (SLE). The patient presented with jaundice, hepatosplenomegaly, malAlse, polyarthralgia, arthritis and butterfly rash on the face. Laboratory tests revealed severe liver dysfunction, Coombs positive hemolytic anemia and a positive ANA/ anti-dsDNA test. Renal biopsy showed class IIA kidney disease, while liver biopsy showed chronic hepatitis with severe inflammatory activity. The patient satisfied the international criteria for both SLE and ALM. Clinical symptoms and laboratory findings of SLE improved with high dose treatment with corticosteroids and azathioprine, however, remission of the liver disease could not be achieved. Repeat biopsy of the liver after three years of therapy revealed ongoing chronic hepatitis with high level of inflammatory activity. The present case indicates that children with liver dysfunction and SLE should be investigated for ALM. There is much diagnostic and therapeutic dilemma in patients with ALH-SLE overlap syndrome.

  6. Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus.

    Science.gov (United States)

    Bermea, Rene S; Sharma, Niharika; Cohen, Kenneth; Liarski, Vladimir M

    2016-09-01

    Atypical hemolytic uremic syndrome is characterized by the presence of thrombocytopenia, microangiopathic hemolytic anemia, and end-organ injury. In this report, we describe two patients with systemic lupus erythematosus who presented with findings compatible with atypical hemolytic uremic syndrome, complicated by acute kidney injury that was refractory to conventional therapies. Both patients exhibited a response to eculizumab, a monoclonal antibody to complement protein C5, with stabilization of their platelet count. On 1-year follow-up from their initial presentation, their hematologic disease remained in remission without recurrence. PMID:27556240

  7. Aplastic Anemia

    Science.gov (United States)

    Aplastic anemia is a rare but serious blood disorder. If you have it, your bone marrow doesn't make ... blood cells. There are different types, including Fanconi anemia. Causes include Toxic substances, such as pesticides, arsenic, ...

  8. Aplastic crisis due to human parvovirus B19 infection in hereditary hemolytic anaemia Crise aplástica devido à infecção por parvovirus humano B19 em anemia hemolítica hereditária

    Directory of Open Access Journals (Sweden)

    R. C. N. Cubel

    1992-10-01

    Full Text Available Specific anti-B19 IgM was demonstrated in sera from three children showing transient aplastic crisis. A two years-old boy living in Rio de Janeiro suffering from sickle-cell anaemia showed the crisis during August, 1990. Two siblings living in Santa Maria, RS, developed aplastic crisis during May, 1991, when they were also diagnosed for hereditary spherocytosis. For a third child from this same family, who first developed aplastic crisis no IgM anti-B19 was detected in her sera.IgM específica anti-B19 foi demonstrada nos soros de três crianças apresentando aplasia transitória de medula. Um menino de dois anos de idade vivendo no Rio de Janeiro e sendo portador de anemia falciforme, apresentou a crise durante Agosto de 1990. Dois irmãos vivendo em Santa Maria - RS, desenvolveram crise de aplasia em Maio de 1991, quando foram também diagnosticados como portadores de microesferocitose. IgM anti-B19 não foi detectada no soro de uma terceira criança, desta mesma família, a qual primeiramente apresentou crise de aplasia.

  9. Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological properties of sickle erythrocytes.

    OpenAIRE

    Embury, S H; Clark, M R; Monroy, G; Mohandas, N

    1984-01-01

    The concurrence of sickle cell anemia and alpha-thalassemia results in less severe hemolytic anemia apparently as a result of reduced intraerythrocytic concentration of hemoglobin S and its retarded polymerization. We have evaluated the effect of alpha-globin gene number on several interrelated properties of sickle erythrocytes (RBC) that are expected to correlate with the hemolytic and rheologic consequences of sickle cell disease. The irreversibly sickled cell number, proportion of very den...

  10. Cytotoxic T lymphocyte antigen-4 (CTLA-4 A49G polymorphism and autoimmune blood diseases

    Directory of Open Access Journals (Sweden)

    Faruk Aktürk

    2010-06-01

    Full Text Available Objective: The cytotoxic T lymphocyte associated antigen-4 (CTLA-4 is expressed on T lymphocytes, and inhibits the T-cell responses. In animal models, it has been shown that complete CTLA-4 deficiency was lethal due to massive infiltration of tissues by polyclonally proliferating lymphocytes. CTLA-4 A49G polymorphism, which has been suggested to reduce the inhibitory function of the CTLA-4 molecule, was found to be associated with various autoimmune diseases in recent studies. Material and Methods: In this study, we evaluated the frequency of CTLA-4 A49G polymorphism in 46 patients with autoimmune hemolytic anemia (AIHA, 62 patients with immune thrombocytopenic purpura (ITP, and 150 healthy individuals. Results: Allele frequencies and genotype distributions were similar in both ITP and AIHA patients compared to healthy individuals. In subgroup analysis, however, we found that in chronic lymphocytic leukemia (CLL patients with AIHA (n=4, all patients had CTLA-4 A49G polymorphism (3 had AG, 1 had GG. There was no significant statistical association between G allele and systemic lupus erythematosus (SLE or AIHA.Conclusion: These data suggest that CTLA-4 A49G polymorphism does not contribute to the pathogenesis of lymphoproliferative diseases itself, nor does it increase the risk of autoimmune complications in patients with lymphoproliferative disease.

  11. 老年贫血517例的临床分析%Clinical Analysis of 517 Cases with Senile Anemia

    Institute of Scientific and Technical Information of China (English)

    李晓莉

    2015-01-01

    目的:探讨老年人贫血的原因及种类特点。方法回顾性分析517例临床确诊的老年贫血患者的临床资料。结果老年贫血的种类前三位分别是缺铁性贫血161例(31.1%),慢病性贫血76例(14.7%),巨细胞性贫血74例(14.3%),骨髓增生异常综合征48例(9.3%),再生障碍性贫血46例(8.9%),自身免疫性溶血性贫血46例(8.9%),淋巴瘤35例(6.8%),白血病31例(6.0%)。结论老年人贫血病因复杂,很大程度上影响到老年人生活质量,同时也影响到老年人基础疾病的预后,因此防治老年贫血应引起临床工作者的高度重视。%Objective To Discuss on the causes and characteristics types of elderly. Methods Retrospective analysis clinical data of 517 cases elderly anemia clinical y diagnosed. Results The first three type of anemia in the elderly are iron deficiency anemia(161 cases, 31.1%), anemia of chronic disease (76 cases, 14.7%), sickle cel anemia (74 cases,14.3%) respectively, myelodysplastic syndrome was 48 cases ( 9.3%), aplastic anemia was 46 cases (8.9%), autoimmune hemolytic anemia anemia was 46 cases (8.9%), lymphoma was 35 cases (6.7%), leukemia was 31 patients(6.0%). Conclusion The complex etiology of anemia in the elderly, to a great extent af ect the quality of life of the elderly, at the same time, anemia af ects the elderly based disease prognosis, Thus the prevention of anemia in the elderly should be paid high at ention.

  12. Clinical Analysis of 517 Cases with Senile Anemia%老年贫血517例的临床分析

    Institute of Scientific and Technical Information of China (English)

    李晓莉

    2015-01-01

    Objective To Discuss on the causes and characteristics types of elderly. Methods Retrospective analysis clinical data of 517 cases elderly anemia clinical y diagnosed. Results The first three type of anemia in the elderly are iron deficiency anemia(161 cases, 31.1%), anemia of chronic disease (76 cases, 14.7%), sickle cel anemia (74 cases,14.3%) respectively, myelodysplastic syndrome was 48 cases ( 9.3%), aplastic anemia was 46 cases (8.9%), autoimmune hemolytic anemia anemia was 46 cases (8.9%), lymphoma was 35 cases (6.7%), leukemia was 31 patients(6.0%). Conclusion The complex etiology of anemia in the elderly, to a great extent af ect the quality of life of the elderly, at the same time, anemia af ects the elderly based disease prognosis, Thus the prevention of anemia in the elderly should be paid high at ention.%目的:探讨老年人贫血的原因及种类特点。方法回顾性分析517例临床确诊的老年贫血患者的临床资料。结果老年贫血的种类前三位分别是缺铁性贫血161例(31.1%),慢病性贫血76例(14.7%),巨细胞性贫血74例(14.3%),骨髓增生异常综合征48例(9.3%),再生障碍性贫血46例(8.9%),自身免疫性溶血性贫血46例(8.9%),淋巴瘤35例(6.8%),白血病31例(6.0%)。结论老年人贫血病因复杂,很大程度上影响到老年人生活质量,同时也影响到老年人基础疾病的预后,因此防治老年贫血应引起临床工作者的高度重视。

  13. Clinical analysis on 28 cases of primary sjogren's syndrome complicated with anemia%原发性干燥综合征并发贫血28例临床研究

    Institute of Scientific and Technical Information of China (English)

    章赛芜; 武洵

    2013-01-01

    Objective:To observe the clinical features of primary sjogren's syndrome (pSS) with anemia. Method: Collect the clinical data of primary sjogren's syndrome with anemia from 28 patients,and analyze the types of a-nemia and its correlation with autoantibody in these patients. Result:28(44%) out of 64 pSS patients were primary sjogren's syndrome with anemia,of which 18(64%) were anemia of chronic disease (ACD),3(10%) were iron deficiency anemia(IDA) ,3( 10%) were autoimmune hemolytic anemia (AIHA),2(7%) were megaloblastic anemia (MA) and other 2(7%)cases were (aplastic anemia) A A. Patients with positive ANA,anti-SSA,anti-SSB antibody were more common in pSS with immunity anemia( AIHA, AA) ,P<0. 05. Conclusion: ACD was the most common type of anemia in pSS complicated with anemia;There was some kind of inherent association between the disorder of immune function and anemia.%目的:观察原发性干燥综合征(pSS)并发贫血的特征.方法:回顾性分析28例pSS并发贫血患者临床资料,并对贫血的类型及与自身抗体相关性进行分析.结果:pSS并发贫血占所有住院pSS患者的44%.其中慢性病贫血(ACD) 18例(64.%),缺铁性贫血(IDA)3例(10%),巨幼红细胞性贫血(MA)2例(7%),自身免疫性溶血性贫血(AIHA)3例(10%),再生障碍性贫血(AA)2例(7%).ANA、抗SSA、SSB抗体阳性在pSS伴免疫性贫血(AIHA,AA)中更常见(P<0.05).结论:pSS并发贫血以ACD最多见;免疫功能紊乱与贫血存在内在的关联.

  14. Reduced 25-OH vitamin D in patients with autoimmune cytopenias, clinical correlations and literature review.

    Science.gov (United States)

    Fattizzo, Bruno; Zaninoni, Anna; Giannotta, Juri A; Binda, Francesca; Cortelezzi, Agostino; Barcellini, Wilma

    2016-07-01

    Vitamin D deficiency is widespread in Western Countries and has been found related to autoimmune and hematologic disease incidence and clinical course. We evaluated vitamin D levels, vitamin D receptor (VDR) and T helper (Th)1, Th2 and Th17 immunomodulatory cytokines in patients with immune thrombocytopenic purpura (ITP, N=44), primary autoimmune hemolytic anemia (AIHA, n=35), Evans' syndrome (n=5) and chronic idiopathic neutropenia (CIN, n=19) and also tested vitamin D effect on the in vitro production of anti-erythrocyte autoantibodies. 25-OH-vitamin D levels were significantly lower and vitamin D receptor higher in patients than in controls. Among ITP cases, those with very low vitamin D levels displayed reduced platelet counts, irrespective of the bleeding history. In AIHA patients, LDH values negatively correlated with vitamin D levels in mixed forms, and reticulocyte counts were positively related with vitamin D. Considering treatment, AIHA patients who had been treated with 2 therapy lines or more showed lower mean 25-OH-vitamin D levels than those untreated or treated with one line of therapy only. IL-6, IL-10, IL-17 and IFN-γ levels were higher in patients versus controls, whereas TNF-α was significantly reduced. Finally, vitamin D at concentrations of 10, 20, and 40ng/mL reduced the in vitro production of anti-erythrocyte autoantibodies both in pokeweed-stimulated and unstimulated cultures. In conclusion, vitamin D is reduced in autoimmune cytopenias and correlate with disease severity, supporting its possible protective role against the development of autoimmunity. Literature review showed vitamin D deficiency reports both in onco- and in non onco-hematologic diseases with a relationship with disease severity/activity in myeloid and lymphoid neoplasms, as well as in sickle cell disease. Supplementation has produced weak results in autoimmune and hematologic diseases, and further studies are needed.

  15. Pregnancy Complications: Anemia

    Science.gov (United States)

    ... Close X Home > Complications & Loss > Pregnancy complications > Anemia Anemia E-mail to a friend Please fill in ... anemia at a prenatal care visit . What causes anemia? Usually, a woman becomes anemic (has anemia) because ...

  16. Alcohol-induced severe acute pancreatitis followed by hemolytic uremic syndrome managed with continuous renal replacement therapy

    OpenAIRE

    Fu, Peng; Yuan, Ai-hong; Wang, Chun-Hua; LI, XIN; Wu, Hai-yang

    2014-01-01

    Background Acute kidney injury in patients with acute pancreatitis carries a poor prognosis. Hemolytic uremic syndrome (HUS) is characterized by non-immune hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney, and though rare in adults it is associated with high mortality and a high rate of chronic renal failure. Case presentation Herein, we report a case of alcohol-induced acute pancreatitis in a 38-year-old Chinese female com...

  17. 中国人先天性溶血性贫血红细胞膜骨架4.1蛋白电泳变异型%Electrophorestic variants of erythrocyte membrane cytoskeletal protein band 4.1 in Chinese with hereditary hemolytic anemia

    Institute of Scientific and Technical Information of China (English)

    李津婴; 叶煦亭; 黄正霞; 许燕群; 韩凤来; 万树栋

    2002-01-01

    Objective:To identify the electrophorestic variants of band 4.1 protein(one of the cytoskeletons in erythrocyte membrane) in Chinese with hereditary hemolytic anemia (HHA). Methods:Twenty-five hemolytic anemia(HA) individuals from unrelated kindreds with abnormal red cell morphology were screened by a protocol on red cell morphology (phase contrast microscope,scanning electron microscope) and red cell membrane proteins (4%-15% sodium dodecylsulfate polyacrylamide gel electrophoresis).Results: The band 4.1 defect in quantity or quality was found in 11 patients(44%).The electrophorestic variants were divided into 4 types:band 4.1 deficiency in both a and b subunits,deficiency only in band 4.1a,partial band 4.1a deficiency,and complete band 4.1a deficiency.The variants showed no correspondence to cell morphology except the type of complete band 4.1a absence, which was only seen in the red cells with screenmes-like membrane surface.Conclusion:The band 4.1 defect is associateds not only with hereditary spherocytosis (HS) and hereditary elliptocytosis, but also with other morphologic changes.In addition to the dificiency in spectrin and band 3,another major cause of HS is the defect of band 4.1 protein in Chinese,while ankyrin in European and North American and band 4.2 more frequently in Japanese.These findings imply the causes of HS and the band 4.1 electrophrestic variants may be different among the different race.%目的:鉴定中国人先天性溶血性贫血(溶贫)红细胞膜4.1蛋白(band 4.1)电泳变异类型.方法:以普通显微镜、相差镜和扫描电镜观察25例溶贫患者红细胞形态学变化.以4%~15% 梯度聚丙烯酰胺凝胶电泳作红细胞膜蛋白定性定量分析.结果:(1)25例红细胞形态学异常溶贫患者中,band 4.1定性定量改变者11例(44%).(2)Band 4.1电泳变异型:①4.1a/b亚基同时缺陷;②4.1a亚基缺陷,a/b亚基相对含量比值倒置(患者0.65~1.20,正常对照1.87±0.22);③4.1a亚基部分缺失

  18. Hemolytic Transfusion Reactions

    Directory of Open Access Journals (Sweden)

    Fatih Mehmet Azık

    2011-12-01

    Full Text Available The prevalence of fatal hemolytic transfusion reactions (HTRs is approximately 1:200000 per unit. Acute HTRs occur during or within 24 h after administration of a blood product. Transfusion of incompatible red blood cells (RBCs, and, more rarely, of a large volume of incompatible plasma usually are the causative agents. Delayed HTRs are caused by a secondary immune response to an antigen on the donor’s RBCs. Different mechanisms lead to intra- and extravascular hemolysis, such as complete complement activation, phagocytosis of RBCs covered with C3b by macrophages after incomplete complement activation, or destruction of RBCs covered only with IgG by direct cell to cell contact with K cells. The clinical consequences of HTRs are triggered via several pathophysiological pathways. Formation of anaphylatoxins, release of cytokines causing a systemic inflammatory response syndrome, activation of the kinin system, the intrinsic clotting cascade and fibrinolysis result in hypotension, disseminated intravascular coagulation, diffuse bleeding, and disruption of microcirculation leading to renal failure and shock. In this review, the symptoms of HTR are introduced, laboratory investigations and treatment are described, and some recommendations for prevention are given. (Journal of Current Pediatrics 2011; 9: 127-32

  19. Autoimmune hepatitis

    Science.gov (United States)

    ... Sjogren syndrome Systemic lupus erythematosus Thyroiditis Type 1 diabetes Ulcerative colitis Autoimmune hepatitis may occur in family members of people with autoimmune diseases. There may be a genetic cause. This disease is most common in young girls ...

  20. Autoimmune dysregulation and purine metabolism in adenosine deaminase (ADA-deficiency

    Directory of Open Access Journals (Sweden)

    Aisha Vanessa Sauer

    2012-08-01

    Full Text Available Genetic defects in the adenosine deaminase (ADA gene are among the most common causes for severe combined immunodeficiency (SCID. ADA-SCID patients suffer from lymphopenia, severely impaired cellular and humoral immunity, failure to thrive and recurrent infections. Currently available therapeutic options for this otherwise fatal disorder include bone marrow transplantation (BMT, enzyme replacement therapy with bovine ADA (PEG-ADA or hematopoietic stem cell gene therapy (HSC-GT. Although varying degrees of immune reconstitution can be achieved by these treatments, breakdown of tolerance is a major concern in ADA-SCID. Immune dysregulation such as autoimmune hypothyroidism, diabetes mellitus, hemolytic anemia, and immune thrombocytopenia are frequently observed in milder forms of the disease. However, several reports document similar complications also in patients on long-term PEG-ADA and after BMT or GT treatment.A skewed repertoire and decreased immune functions have been implicated in autoimmunity observed in certain B-cell and/or T-cell immunodeficiencies, but it remains unclear to what extent specific mechanisms of tolerance are affected in ADA deficiency. Herein we provide an overview about ADA-SCID and the autoimmune manifestations reported in these patients before and after treatment. We also assess the value of the ADA-deficient mouse model as a useful tool to study both immune and metabolic disease mechanisms. With focus on regulatory T and B cells we discuss the lymphocyte subpopulations particularly prone to contribute to the loss of self-tolerance and onset of autoimmunity in ADA deficiency. Moreover we address which aspects of immune dysregulation are specifically related to alterations in purine metabolism caused by the lack of ADA and the subsequent accumulation of metabolites with immunomodulatory properties.

  1. What Is Aplastic Anemia?

    Science.gov (United States)

    ... from the NHLBI on Twitter. What Is Aplastic Anemia? Aplastic anemia (a-PLAS-tik uh-NEE-me-uh) is ... heart, heart failure , infections, and bleeding. Severe aplastic anemia can even cause death. Overview Aplastic anemia is ...

  2. What Causes Anemia?

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Causes Anemia? The three main causes of anemia are: Blood ... the blood and can lead to anemia. Aplastic Anemia Some infants are born without the ability to ...

  3. About Anemia (For Kids)

    Science.gov (United States)

    ... Homework? Here's Help White House Lunch Recipes About Anemia KidsHealth > For Kids > About Anemia Print A A ... to every cell in your body. What Is Anemia? Anemia occurs when a person doesn't have ...

  4. Inborn anemias in mice. Progress report, 1 August 1979-15 July 1980

    Energy Technology Data Exchange (ETDEWEB)

    Bernstein, S.E.; Russell, E.S.

    1980-08-01

    Four macrocytic anemias, four hemolytic anemias, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, an ..cap alpha..-thalassemia, and a new target-cell anemia are under investigation in mice. Each of these blood dyscrasias is caused by the action of a unique mutant gene, which determines the structure of different intracellular molecules, and thus controls a different metabolic process. Thus the wide range of different hereditary anemias has considerable potential for uncovering many different aspects of hemopoietic homeostatic mechanisms in the mouse. Each anemia is studied through: (a) characterization of peripheral blood values; (b) determinations of radiosensitivity under a variety of conditions; (c) measurements of iron metabolism and heme synthesis; (d) histological and biochemical study of blood-forming tissue; (e) functional tests of the stem cell component; (f) examination of responses to erythroid stimuli; and (g) transplantation of tissue between individuals of differently affected genotypes.

  5. Type 1 diabetes associated autoimmunity.

    Science.gov (United States)

    Kahaly, George J; Hansen, Martin P

    2016-07-01

    Diabetes mellitus is increasing in prevalence worldwide. The economic costs are considerable given the cardiovascular complications and co-morbidities that it may entail. Type 1 diabetes (T1D) is a chronic autoimmune disease characterized by the loss of insulin-producing pancreatic β-cells. The pathogenesis of T1D is complex and multifactorial and involves a genetic susceptibility that predisposes to abnormal immune responses in the presence of ill-defined environmental insults to the pancreatic islets. Genetic background may affect the risk for autoimmune disease and patients with T1D exhibit an increased risk of other autoimmune disorders such as autoimmune thyroid disease, Addison's disease, autoimmune gastritis, coeliac disease and vitiligo. Approximately 20%-25% of patients with T1D have thyroid antibodies, and up to 50% of such patients progress to clinical autoimmune thyroid disease. Approximately 0.5% of diabetic patients have concomitant Addison's disease and 4% have coeliac disease. The prevalence of autoimmune gastritis and pernicious anemia is 5% to 10% and 2.6% to 4%, respectively. Early detection of antibodies and latent organ-specific dysfunction is advocated to alert physicians to take appropriate action in order to prevent full-blown disease. Patients and family members should be educated to be able to recognize signs and symptoms of underlying disease.

  6. Assesment, treatment and prevention of atypical hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Azar Nickavar

    2013-01-01

    Full Text Available Hemolytic uremic syndrome (HUS is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1 D+ HUS: Presentation with a preceding diarrhea; (2 typical HUS: D+ HUS with a single and self-limited episode; (3 atypical HUS (aHUS: Indicated those with complement dysregulation; (4 recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA after improvement of hematologic abnormalities; and (5 familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.

  7. Iron deficiency anemia in celiac disease.

    Science.gov (United States)

    Freeman, Hugh James

    2015-08-21

    Iron is an important micronutrient that may be depleted in celiac disease. Iron deficiency and anemia may complicate well-established celiac disease, but may also be the presenting clinical feature in the absence of diarrhea or weight loss. If iron deficiency anemia occurs, it should be thoroughly evaluated, even if celiac disease has been defined since other superimposed causes of iron deficiency anemia may be present. Most often, impaired duodenal mucosal uptake of iron is evident since surface absorptive area in the duodenum is reduced, in large part, because celiac disease is an immune-mediated disorder largely focused in the proximal small intestinal mucosa. Some studies have also suggested that blood loss may occur in celiac disease, sometimes from superimposed small intestinal disorders, including ulceration or neoplastic diseases, particularly lymphoma. In addition, other associated gastric or colonic disorders may be responsible for blood loss. Rarely, an immune-mediated hemolytic disorder with increased urine iron loss may occur that may respond to a gluten-free diet. Reduced expression of different regulatory proteins critical in iron uptake has also been defined in the presence and absence of anemia. Finally, other rare causes of microcytic anemia may occur in celiac disease, including a sideroblastic form of anemia reported to have responded to a gluten-free diet.

  8. SUBTYPES OF B LYMPHOCYTES IN PATIENTS WITH AUTOIMMUNE HEMOCYTOPENIA

    Institute of Scientific and Technical Information of China (English)

    Li-min Xing; Hai-rong Jia; Juan Sun; Chong-li Yang; Zong-hong Shao; Rong Fu; Hong Liu; Jun Shi; Lie Bai; Mei-feng Tu; Hua-quan Wang; Zhen-zhu Cui

    2007-01-01

    Objective To investigate the quantities of bone marrow CD5+ B lymphocytes in the patients with autoimmune hemocytopenia and the relationship between quantities of CD5+ B lymphocytes and clinical or laboratorial parameters.Methods Quantities of CD5+ B lymphocytes in the bone marrow of 14 patients with autoimmune hemolytic anemia (AIHA) or Evans syndrome, 22 immunorelated pancytopenia (IRP) patients, and 10 normal controls were assayed by flow cytometry. The correlation between their clinical or laboratorial parameters and CD5+ B lymphocytes was analyzed.Results The quantity of CD5+B lymphocytes of AIHA/Evans syndrome (34. 64% ± 19. 81% ) or IRP patients (35.81% ±16.83% ) was significantly higher than that of normal controls (12.00% ±1.97% , P<0. 05). However, there was no significant difference between AIHA/Evans syndrome and IRP patients (P > 0. 05). In all hemocytopenic patients, the quantity of bone marrow CD5+ B lymphocytes showed significantly negative correlation with serum complement C3 level (r = - 0. 416, P< 0. 05). In the patients with AIHA/Evans syndrome, the quantity of bone marrow CD5+ B lymphocytes showed significantly positive correlation with serum indirect bilirubin level (r = 1. 00, P<0. 05). In Evans syndrome patients, the quantity of CD5+ B lymphocytes in bone marrow showed significantly positive correlation with platelet-associated immunoglobulin G (r = 0. 761, P< 0. 05) and platelet-associated immunoglobulin M (r = 0. 925, P< 0. 05). The quantity of CD5+ B lymphocytes in bone marrow of all hemocytopenic patients showed significantly negative correlation with treatment response (tau-b = - 0. 289, P< 0. 05), but had no correlation with colony forming unit-erythroid ( r = - 0. 205, P > 0. 05 ) or colony forming unit-granulocyte-macrophage colonies ( r = -0.214, P>0.05).Conclusions The quantity of bone marrow CD5+ B lymphocytes in the patients with autoimmune hemocytopenia significantly increases and is correlated with disease

  9. Autoimmune epilepsy.

    Science.gov (United States)

    Greco, Antonio; Rizzo, Maria Ida; De Virgilio, Armando; Conte, Michela; Gallo, Andrea; Attanasio, Giuseppe; Ruoppolo, Giovanni; de Vincentiis, Marco

    2016-03-01

    Despite the fact that epilepsy is the third most common chronic brain disorder, relatively little is known about the processes leading to the generation of seizures. Accumulating data support an autoimmune basis in patients with antiepileptic drug-resistant seizures. Besides, recent studies show that epilepsy and autoimmune disease frequently co-occur. Autoimmune epilepsy is increasingly recognized in the spectrum of neurological disorders characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy. An autoimmune cause is suspected based on frequent or medically intractable seizures and the presence of at least one neural antibody, inflammatory changes indicated in serum or spinal fluid or on MRI, or a personal or family history of autoimmunity. It is essential that an autoimmune etiology be considered in the initial differential diagnosis of new onset epilepsy, because early immunotherapy assures an optimal outcome for the patient. PMID:26626229

  10. A case of severe preeclampsia diagnosed as post-partum hemolytic uremic syndrome

    Institute of Scientific and Technical Information of China (English)

    WANG Yong-qing; WANG Jing; JIANG Yuan-hui; YE Rong-hua; ZHAO Yang-yu

    2012-01-01

    Post-partum hemolytic uremic syndrome (PHUS) is a severe thrombotic microangiopathy clinically characterized by hemolytic anemia,renal dysfunction,and low platelets after birth with rapid progression and poor prognosis.Here,we reported a rare case of severe preeclampsia diagnosed as hemolytic uremic syndrome after birth.The patient was diagnosed with PHUS and underwent intermittent plasma exchange with supportive treatment including glucocorticoid injections and transfusion of suspended red blood cells.After these treatments,the patient experienced no apparent remission and chronic renal dysfunction occurred on her.PHUS is a severe emergency with acute onset,rapid progress,and poor prognosis.Early detection,diagnosis,and treatment can significantly improve the prognosis.

  11. [Autoimmune hepatitis].

    Science.gov (United States)

    Ostojić, Rajko

    2003-01-01

    Autoimmune hepatitis is an unresolving, hepatocellular inflammation of unknown cause that is characterized by the presence of periportal hepatitis on histologic examination, tissue autoantibodies in serum, and hypergammaglobulinemia. By international consensus, the designation autoimmune hepatitis has replaced alternative terms for the condition. Three types of autoimmune hepatitis have been proposed based on immunoserologic findings. Type 1 autoimmune hepatitis is characterized by the presence of antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) (or both) in serum. Seventy percent of patients with type 1 of autoimmune hepatitis are women. This type is the most common form and accounts for at least 80% of cases. Type 2 is characterized by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) in serum. Patients with this type of autoimmune hepatitis are predominantly children. Type 3 autoimmune hepatitis is characterized by the presence of antibodies to soluble liver antigen (anti-SLA) in serum. There are no individual features that are pathognomonic of autoimmune hepatitis, and its diagnosis requires the confident exclusion of other conditions. The large majority of patients show satisfactory response to corticosteroid (usually prednisone or prednisolone) therapy. For the past 30 years it has been customary to add azathioprine as a "steroid sparing" agent to allow lower doses of steroids to be used and remission, once achieved, can be sustained in many patients with azathioprine alone after steroid withdrawal. Patients with autoimmune hepatitis who have decompensated during or after corticosteroid therapy are candidates for liver transplantation.

  12. Hemolytic disease of the fetus and newborn caused by anti-E

    Directory of Open Access Journals (Sweden)

    Adiyyatu Sa′idu Usman

    2013-01-01

    Full Text Available Objective: Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother′s red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs. Case summary: We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting with severe anemia, thrombocytopenia, and conjugated hyperbilirubinemia, while the other had moderate anemia and unconjugated hyperbilrubinemia. Although both the neonates were treated by phototherapy and intravenous immunoglobulin, one of them received double volume exchange transfusion. Conclusion: There appeared to be an increase in the occurrence of hemolytic disease of the fetus and newborn caused by Rh antibodies other than anti-D. In this case report, both patients presented with anemia and hyperbilirubinemia but were successfully treated, with a favorable outcome.

  13. APLASTIC ANEMIA

    Directory of Open Access Journals (Sweden)

    Ni Made Dharma Laksmi

    2013-07-01

    Full Text Available Normal 0 false false false EN-US X-NONE X-NONE MicrosoftInternetExplorer4 Aplastic Anemia describes a disorder of the clinical syndrome is marked by a deficiency of red blood cells, neutrophils, monocytes and platelets in the absence of other forms of bone marrow damage. Aplastic anemia is classified as a rare disease in developed countries the incidence of 3-6 cases / 1 million inhabitants / year. The exact cause of someone suffering from aplastic anemia also can not be established with certainty, but there are several sources of potential risk factors. Prognosis or course of the disease varies widely aplastic anemia, but without treatment generally gives a poor prognosis /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:"Times New Roman"; mso-bidi-theme-font:minor-bidi;}

  14. Sickle cell anemia - resources

    Science.gov (United States)

    Resources - sickle cell anemia ... The following organizations are good resources for information on sickle cell anemia : American Sickle Cell Anemia Association -- www.ascaa.org National Heart, Blood, and Lung Institute -- www. ...

  15. Folate-deficiency anemia

    Science.gov (United States)

    ... medlineplus.gov/ency/article/000551.htm Folate-deficiency anemia To use the sharing features on this page, please enable JavaScript. Folate-deficiency anemia is a decrease in red blood cells (anemia) ...

  16. Fanconi Anemia Research Fund

    Science.gov (United States)

    ... Support Publications Fundraising News What is the Fanconi Anemia Research Fund? Fanconi anemia is an inherited disease that can lead to ... population. Lynn and Dave Frohnmayer started the Fanconi Anemia Research Fund, in 1989 to find effective treatments ...

  17. Living with Anemia

    Science.gov (United States)

    ... page from the NHLBI on Twitter. Living With Anemia Often, you can treat and control anemia. If ... by an inherited or chronic disease or trauma. Anemia and Children/Teens Infants and young children have ...

  18. Postoperative Atypical Hemolytic Uremic Syndrome Associated with Complement C3 Mutation

    OpenAIRE

    Eiji Matsukuma; Atsushi Imamura; Yusuke Iwata; Takamasa Takeuchi; Yoko Yoshida; Yoshihiro Fujimura; Xinping Fan; Toshiyuki Miyata; Takashi Kuwahara

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) can be distinguished from typical or Shiga-like toxin-induced HUS. The clinical outcome is unfavorable; up to 50% of affected patients progress to end-stage renal failure and 25% die during the acute phase. Multiple conditions have been associated with aHUS, including infections, drugs, autoimmune conditions, transplantation, pregnancy, and metabolic conditions. aHUS in the nontransplant postsurgical period, however, is rare. An 8-month-old boy underw...

  19. Autoimmune Diseases

    Science.gov (United States)

    ... Some examples of CAM are herbal products, chiropractic , acupuncture , and hypnosis . If you have an autoimmune disease, ... Toll-Free: 877-226-4267 National Institute of Diabetes and Digestive and Kidney Diseases, NIH, HHS Phone: ...

  20. Warm Autoimmune Haemolytic Anaemia and autoimmune hepatitis in an asymptomatic carrier of hepatitis B virus

    International Nuclear Information System (INIS)

    Warm antibody autoimmune haemolytic anaemia, a rare disease (0.2-1 per 100,000 populations), is due to the presence of warm agglutinins that react with protein antigens on the surface of red blood cells causing their premature destruction. Here, we present a case report of a 10 year old girl who came with features of haemolytic anaemia and history of blood transfusion since 3 years. On admission, laboratory test revealed that she had autoimmune hepatitis type 1 and was also an asymptomatic carrier of hepatitis B virus with positive HBs Ag. Steroid therapy resulted in clinical and laboratory remission. Direct antiglobulin test was negative after anaemia resolution, hepatitis B virus antigenemia persisted. To our knowledge, warm antibody autoimmune hemolytic anaemia has not previously been described in association with autoimmune hepatitis and asymptomatic carrier state of hepatitis B virus. (author)

  1. Iron deficiency decreases hemolysis in sickle cell anemia Anemia ferropriva diminui hemólise em anemia falciforme

    Directory of Open Access Journals (Sweden)

    Oswaldo Castro

    2009-02-01

    Full Text Available A woman with homozygous sickle cell disease developed severe iron deficiency due to long-standing uterine bleeding. At this point, the serum lactic dehydrogenase level was normal and the reticulocyte count was only minimally elevated. This suggested that the low red cell hemoglobin concentration that resulted from iron deficiency also decreased Hb S polymerization and lowered the hemolytic rate. Iron replacement led first to a substantially improved hemoglobin concentration with only a minimal increase in the hemolytic rate and secondarily to a modest further improvement in the hemoglobin concentration and a marked increase in the hemolytic rate. The hematologic changes observed in this patient, and those in other iron deficient sickle cell patients reported in the literature, suggest that it may be appropriate to consider the induction of an intermediate iron deficient stage as experimental treatment in adult sickle cell patients.Uma mulher com anemia falciforme homozigose para a Hb S evoluiu com anemia ferropriva grave devido a sangramento uterino prolongado. A dosagem de dehidrogenase lática era normal e a contagem de reticulócitos estava levemente aumentada. Isto sugere que concentrações baixas de hemoglobina, que resulta de anemia ferropriva, também diminuem a polimeração de Hb S e reduz a taxa de hemólise. O complemento de ferro levou, primeiramente, a uma concentração substancialmente maior de hemoglobina com apenas um aumento mínimo na taxa hemolítica e subsequentemente a um aumento leve adicional na concentração da hemoglobina e um aumento notável na taxa hemolítica. As mudanças hematológicas observadas nesta paciente e aquelas em outras pacientes com anemia falciforme e também deficientes de ferro relatadas na literatura sugerem que pode ser interessante considerar a indução de deficiência de ferro como tratamento experimental em pacientes adultos com anemia falciforme.

  2. Advanced Prostate Cancer Presenting as Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    R. Ramos

    2013-01-01

    Full Text Available Introduction. Hemolytic uremic syndrome (HUS is characterized by endothelial dysfunction, consumption thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. HUS generally has a dismal prognosis, except when associated with gastroenteritis caused by verotoxin-producing bacteria. Cancer associated HUS is uncommon, and there are only scarce reports on prostate cancer presenting with HUS. Case Presentation. A 72-year-old man presented to the emergency department with oliguria, hematuria, and hematemesis. Clinical evaluation revealed acute renal failure, hemolysis, normal blood-clotting studies, and prostate-specific antigen value of 1000 ng/mL. The patient was started on hemodialysis, ultrafiltration with plasma exchange, and androgen blockade with bicalutamide and completely recovered from HUS. The authors review the 14 published cases on this association. Conclusion. The association of HUS and prostate cancer occurs more frequently in patients with high-grade, clinically advanced prostate cancer. When readily recognized and appropriately treated, HUS does not seem to worsen prognosis in prostate cancer patients.

  3. Autoimmune disease

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    2005164 Optimal cut-point of glutamic acid decar-boxylase antibody (GAD-Ab) for differentiating two subtypes of latent autoimmune diabetes in adults (LADA). LI Xia(李霞), et al. Dept Endocrinol, 2nd Xiangya Hosp, Central South Univ, Changsha, 410011. Chin J Diabetes, 2005;13(1) :34-38. Objective: To investigate the optimal cut-point of glutamate decarboxylase antibody (GAD-Ab) for differentiating two subtypes of latent autoimmune diabetes in adults (I. ADA). Methods: The frequency

  4. Autoimmune synaptopathies.

    Science.gov (United States)

    Crisp, Sarah J; Kullmann, Dimitri M; Vincent, Angela

    2016-02-01

    Autoantibodies targeting proteins at the neuromuscular junction are known to cause several distinct myasthenic syndromes. Recently, autoantibodies targeting neurotransmitter receptors and associated proteins have also emerged as a cause of severe, but potentially treatable, diseases of the CNS. Here, we review the clinical evidence as well as in vitro and in vivo experimental evidence that autoantibodies account for myasthenic syndromes and autoimmune disorders of the CNS by disrupting the functional or structural integrity of synapses. Studying neurological and psychiatric diseases of autoimmune origin may provide new insights into the cellular and circuit mechanisms underlying a broad range of CNS disorders. PMID:26806629

  5. Anemia as a risk factor for childhood asthma

    Directory of Open Access Journals (Sweden)

    Ramakrishnan K

    2010-01-01

    Full Text Available Objective: This prospective-(cohort study was conducted to evaluate whether anemia is a risk factor for childhood asthma. Materials and Methods: Two hundred children in the age group of 2-18 years who attended the Outpatient Department with upper respiratory / lower respiratory tract infections were included in this study. One hundred children with anemia were taken as the study group and another 100, age - and sex-matched children without anemia were taken as the control.They were subjected to complete blood count (CBC C-reactive protein (CRP estimation, Mantoux test and chest X-ray. Pulmonary function tests (PFTs were performed on those above six years showing evidence of asthma. Peripheral smear, serum ferritin and serum iron-binding capacity were estimated for all anemic children. Results: Asthma was present in 74 (74% children in the study group and in 33 (33% children in the control group. Iron-deficiency anemia was present in 85 (85% anemia of chronic infection in 20 (20% and the other five (5% had hemolytic anemia. Anemia was found to be a risk factor for childhood asthma. Conclusion: Anemic children were 5.75 times more susceptible to asthmatic attacks when compared with nonanemic children.

  6. Reticulocyte parameters in hemoglobinopathies and iron deficiency anemia

    Directory of Open Access Journals (Sweden)

    Cortellazzi Laura C.

    2003-01-01

    Full Text Available Flow cytometric reticulocyte analysis allows the evaluation of reticulocyte maturity. New reticulocyte parameters have been used in the diagnosis and management of anemias, in the bone marrow transplant setting and in the monitoring of iron replacement or erythropoiet in therapy. Reticulocyte numbers and maturation levels have been studied in different hemoglobinopathies and the results have been correlated with the degree of ineffective erythropoiesis. In order to verify differences in reticulocyte parameters in various types of anemias and to test the absolute number of immature reticulocytes as a possible discriminating factor among various types of anemias, reticulocyte counts were performed on 219 samples from patients with sickle cell anemia (SS (n= 62, hemoglobin S trait (n=9, Sbeta thalassemia (n=7, hemoglobin SC disease (n=11, beta thalassemia trait (n=33 and iron deficiency anemia (n= 47, and non-anemic individuals (n= 50. Mean fluorescence index (MFI was defined as representative of the degree of reticulocyte immaturity and it was evaluated as a percentage and in absolute values. Reticulocyte counts and MFI values were significantly higher in SS, Sbeta thalassemic and SC groups when compared to controls, but not different among the three anemia groups. Patients with hemoglobin S trait, iron deficiency anemia and beta thalassemia trait showed reticulocyte parameters similar to the non-anemic group. There was no difference between the b thalassemic trait and iron deficiency anemia in relation to any parameters. MFI in absolute numbers were significantly higher in anemias that develop with the hemolytic process, although this was not evident in MFI percentage values. Our results showed that the erythoid expansion in sickle cell diseases (SS, SC and Sb thalassemia leads to an enhanced immature reticulocyte release from bone marrow and that the phenomena is more evident by the MFI counting in absolute figures than in percentages. We

  7. Autoimmun pankreatitis

    DEFF Research Database (Denmark)

    Fjordside, Eva; Novovic, Srdan; Schmidt, Palle Nordblad;

    2015-01-01

    Autoimmune pancreatitis (AIP) is a rare inflammatory disease. AIP has characteristic histology, serology and imaging findings. Two types of AIP exist, type 1, which is a part of the systemic immunoglobulin G4-related disease, and type 2, which is only localized to the pancreas. Patients with type 1...

  8. Autoimmun hypophysitis

    DEFF Research Database (Denmark)

    Krarup, Therese; Hagen, Claus

    2010-01-01

    during pregnancy or postpartum, but also occurs in males and children. AH is often associated with other autoimmune diseases, most frequently with Hashimoto's thyroiditis. The symptoms are caused by enlargement of the pituitary gland and disturbances of the hormone function. Treatment is either...

  9. [Neuropsychiatric manifestations ushering pernicious anemia].

    Science.gov (United States)

    Mrabet, S; Ellouze, F; Ellini, S; Mrad, M F

    2015-12-01

    Biermer disease or pernicious anemia is an autoimmune atrophic gastritis characterized by the lack of secretion of gastric intrinsic factor. This leads to an insufficient absorption of vitamin B12 in the ileum. Clinical manifestations are mainly hematologic. Neuropsychiatric manifestations are known but are less frequent especially early in the disease. Inaugural neuropsychiatric arrays are rare and various thus making diagnosis difficult. In this article, we report through two clinical cases different neuropsychiatric manifestations revealing pernicious anemia. Mrs. C.O., aged 56, presented after surgery for gallstones, an acute psychiatric array associated with gait disorders. She had no history of neurological or psychiatric problems. The psychiatric interview revealed delirious syndrome, depressive symptoms and anxiety. Neurological examination noted a flaccid paraplegia with peripheral neuropathic syndrome and myoclonus in the upper limbs. At the full blood count, a macrocytosis (VGM: 112.2fl) without anemia was found. The level of vitamin B12 in the blood was low. Cerebro-spinal MRI was suggestive of a neuro-Biermer and showed hyper signal in the cervical cord on T2-weighted sagittal section. In axial section, hyper signal appears at the posterior columns in the form of V. There were no brain abnormalities. A sensorimotor axonal polyneuropathy was diagnosed. The patient received vitamin B12 intramuscularly for ten days associated with neuroleptic treatment. Mrs. R.M., aged 40, was brought to the psychiatry consultation for acute behavioral disorders progressively worsening over a month. An anxiety syndrome, depressive syndrome and delirious syndrome were identified. Neurological examination showed a posterior cordonal syndrome with quadripyramidal syndrome. Full blood count showed a macrocytic anemia. Serum B12 level was collapsed. Cerebro-spinal MRI was normal. She received vitamin B12 with clinical and biological improvement. Features of pernicious anemia

  10. Deformabilidade eritrocitária na anemia ferropriva Erythrocyte deformability in iron deficiency

    Directory of Open Access Journals (Sweden)

    Giuseppina M. Patavino

    2006-12-01

    Full Text Available A deformabilidade é a característica que permite ao eritrócito normal de 7 a 8 micrômetros (µm circular por capilares de até 3 µm de diâmetro. Esse fenômeno depende da geometria celular, da viscosidade interna e de propriedades visco-elásticas da membrana eritrocitária. Dentre as técnicas de estudo da deformabilidade eritrocitária (DE, como aspiração por micropipeta, filtração e reoscopia, destaca-se a ectacitometria. Esta técnica utiliza um viscosímetro de fluxo laminar no qual as modificações de forma dos eritrócitos são monitoradas continuamente por um feixe de raio laser, processadas por microcomputador, gerando o "Índice de Deformabilidade" (ID, que mede a eliptocitogênese dos eritrócitos quando submetidos a uma força denominada "shear stressl". Alterações de DE foram descritas em diversas situações, como em anemias hemolíticas hereditárias ou auto-imunes. Na anemia ferropriva, os trabalhos são controversos. O presente estudo avalia a DE em 21 pacientes portadores de anemia ferropriva, utilizando a ectacitometria. Os resultados obtidos a partir do ID demonstram DE diminuída nesses doentes, quando comparada ao grupo controle (pDeformability allows the 7 to 8 µm red cell to cirDeformability allows the 7- to 8-µm red blood cells to circulate through capillaries of 3 µm. This phenomenon depends on cellular geometry, internal viscosity and viscoelastic properties of the membrane. Among the various techniques of erythrocyte deformability analysis, such as micropipette aspiration, filtration and reoscopy, we chose ektacytometry. This technique uses a laminar flow viscometry, where red blood cell shape changes are continuously monitored by laser, processed by a computer, generating the "Deformability Index", which shows the elliptocytogenesis of the erythrocyte under "shear stressl" force. Erythrocyte deformability has been described in a number of situations like hereditary or autoimmune hemolytic anemia. In

  11. Severe pneumococcal hemolytic uremic syndrome in an 8-month-old girl

    Directory of Open Access Journals (Sweden)

    Tahar Gargah

    2012-01-01

    Full Text Available The hemolytic uremic syndrome (HUS, characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure, represents one of the major causes of acute renal failure in infancy and childhood. The typical form occurring after an episode of diarrhea caused by Escherichia coli is the most frequent in children. Other microorganisms also may be responsible for HUS, such as Streptococcus pneumoniae, which causes more severe forms of the disease. We report an 8-month-old girl who presented with pneumonia and subsequently developed HUS. Renal biopsy showed characteristic lesion of thrombotic microangiopathy and extensive cortical necrosis. She was managed with peritoneal dialysis but did not improve and developed severe sepsis due to staphylococcal peritonitis, resulting in the death of the patient. Streptococcus pneumoniae-induced HUS is uncommon, but results in severe disease in the young. There is a high risk of these patients developing end-stage kidney disease in the long term.

  12. Sickle cell anemia.

    OpenAIRE

    ŘÍHOVÁ, Tereza

    2013-01-01

    This thesis is about the disease called sickle cell anemia, or drepanocytosis. In this thesis is described the history of the disease, pathophysiology, laboratory features, various clinical features, diferencial diagnosis, quality of life in sickle cell anemia and therapy.

  13. Cooley's Anemia Foundation

    Science.gov (United States)

    Cooley's Anemia Foundation Leading the Fight against Thalassemia About Us Mission/Purpose History About Thomas Benton Cooley Medical Research ... Gabriella was diagnosed with thalassemia, and the Cooley’s Anemia Foundation continues to play an almost-daily role ...

  14. Anemia and Pregnancy

    Science.gov (United States)

    ... most recent scientific research View all publications Home Anemia and Pregnancy Your body goes through significant changes ... becoming anemic. back to top Is Pregnancy-Related Anemia Preventable? Good nutrition is the best way to ...

  15. Anemia in the Newborn

    Science.gov (United States)

    ... Video) Meconium Aspiration Syndrome Additional Content Medical News Anemia in the Newborn By Arthur E. Kopelman, MD ... Prematurity (ROP) Necrotizing Enterocolitis (NEC) Jaundice in Newborns Anemia in the Newborn Polycythemia in the Newborn Thyroid ...

  16. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Deficiency Anemia What Is... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS PREVENTION LIVING WITH CLINICAL ... and women are the two groups at highest risk for iron-deficiency anemia. Outlook Doctors usually can ...

  17. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... This Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video— ... treatment. For more information about living with and managing iron-deficiency anemia, go to the Health Topics ...

  18. Imaging diagnosis of neonatal anemia: report of two unusual etiologies.

    Science.gov (United States)

    Grover, Shabnam Bhandari; Preethi, G Rajalakshmi; Saluja, Sumita; Bhargava, Ankit

    2013-01-01

    Anemia in neonatal period is rare, with the common causes being Rh and ABO blood group incompatibility, hemorrhagic disease of newborn, congenital hemolytic anemia, hemoglobinopathies, and TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes virus) infections. Congenital leukemia and infantile osteopetrosis (OP) are among the rare causes of neonatal anemia. A review of the literature shows approximately 200 reported cases of congenital leukemia. Articles describing the imaging features of congenital leukemia are still rarer. Infantile OP, another rare disorder with a reported incidence of 1 in 250,000 has characteristic imaging features, which are diagnostic of the disease. We report a case each, of two rare diseases: Congenital leukemia and infantile osteopetrosis. Additionally, our report highlights the radiological and imaging features of congenital leukemia and infantile OP and their crucial role in arriving at an early diagnosis. PMID:24605254

  19. Autoimmune Encephalitis

    OpenAIRE

    Leypoldt, Frank; Wandinger, Klaus-Peter; Bien, Christian G; Dalmau, Josep

    2013-01-01

    The term autoimmune encephalitis is used to describe a group of disorders characterised by symptoms of limbic and extra-limbic dysfunction occurring in association with antibodies against synaptic antigens and proteins localised on the neuronal cell surface. In recent years there has been a rapidly expanding knowledge of these syndromes resulting in a shift in clinical paradigms and new insights into pathogenic mechanisms. Since many patients respond well to immunosuppressive treatment, the r...

  20. Autoimmun pankreatitis

    DEFF Research Database (Denmark)

    Fjordside, Eva; Novovic, Srdan; Schmidt, Palle Nordblad;

    2015-01-01

    Autoimmune pancreatitis (AIP) is a rare inflammatory disease. AIP has characteristic histology, serology and imaging findings. Two types of AIP exist, type 1, which is a part of the systemic immunoglobulin G4-related disease, and type 2, which is only localized to the pancreas. Patients with type 1...... are predominantly older men, have involvement of other organs and more often experience relapse than patients with type 2. Both types respond well to steroid treatment. The most important differential diagnose is pancreatic cancer....

  1. Association of schizophrenia and autoimmune diseases: linkage of Danish national registers

    DEFF Research Database (Denmark)

    Eaton, William W.; Byrne, Majella; Ewald, Henrik;

    2006-01-01

    of schizophrenia patients than among parents of comparison subjects (adjusted incidence rate ratios ranging from 1.3 to 3.8). Thyrotoxicosis, celiac disease, acquired hemolytic anemia, interstitial cystitis, and Sjogren's syndrome had higher prevalence rates among patients with schizophrenia than among comparison...

  2. High Dose Cyclophosphamide Treatment for Autoimmune Disorders

    OpenAIRE

    Brodsky, Robert A.

    2002-01-01

    High-dose cyclophosphamide (200 mg/kg) was initially developed as a conditioning regimen for allogeneic bone marrow transplantation. Recently, high-dose cyclophosphamide without bone marrow transplantation has been employed as a method to induce durable treatment-free remissions in severe aplastic anemia and a variety of other severe autoimmune disorders. The premise underlying this approach is that high-dose cyclophosphamide is maximally immunosuppressive, but not myeloablative. Early hemato...

  3. Anemia in Elderly Koreans

    OpenAIRE

    Lee, Jong Hwa

    2011-01-01

    Recently, the geriatric population in Korea has grown to comprise approximately 10% of the total population, and anemia has become a significant problem among elderly patients. Many elderly patients have anemia due to nutritional deficiency, chronic inflammation, or comorbid diseases; however, in a significant fraction of the patients with anemia, the cause remains obscure. Anemia of any degree is recognized as a significant independent contributor to morbidity and mortality in elderly patien...

  4. Iron-Deficiency Anemia

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, ... Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by ...

  5. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... the NHLBI on Twitter. What Is Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily ... Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... body. Low iron levels usually are due to blood loss, poor diet, or an inability to absorb enough iron from food. Overview Iron-deficiency anemia is a common type of anemia . The term "anemia" usually refers to ...

  7. Autoimmune Epilepsy

    Science.gov (United States)

    Quek, Amy M. L.; Britton, Jeffrey W.; McKeon, Andrew; So, Elson; Lennon, Vanda A.; Shin, Cheolsu; Klein, Christopher J.; Watson, Robert E.; Kotsenas, Amy L.; Lagerlund, Terrence D.; Cascino, Gregory D.; Worrell, Gregory A.; Wirrell, Elaine C.; Nickels, Katherine C.; Aksamit, Allen J.; Noe, Katherine H.; Pittock, Sean J.

    2013-01-01

    Objective To describe clinical characteristics and immunotherapy responses in patients with autoimmune epilepsy. Design Observational, retrospective case series. Setting Mayo Clinic Health System. Patients Thirty-two patients with an exclusive (n=11) or predominant (n = 21) seizure presentation in whom an autoimmune etiology was suspected (on the basis of neural autoantibody [91%], inflammatory cerebrospinal fluid [31%], or magnetic resonance imaging suggesting inflammation [63%]) were studied. All had partial seizures: 81% had failed treatment with 2 or more anti-epileptic drugs and had daily seizures and 38% had seizure semiologies that were multifocal or changed with time. Head magnetic resonance imaging was normal in 15 (47%) at onset. Electroencephalogram abnormalities included interictal epileptiform discharges in 20; electrographic seizures in 15; and focal slowing in 13. Neural autoantibodies included voltage-gated potassium channel complex in 56% (leucine-rich, glioma-inactivated 1 specific, 14; contactin-associated proteinlike 2 specific, 1); glutamic acid decarboxylase 65 in 22%; collapsin response-mediator protein 5 in 6%; and Ma2, N-methyl-D-aspartate receptor, and ganglionic acetylcholine receptor in 1 patient each. Intervention Immunotherapy with intravenous methylprednisolone; intravenous immune globulin; and combinations of intravenous methylprednisolone, intravenous immune globulin, plasmapheresis, or cyclo-phosphamide. Main Outcome Measure Seizure frequency. Results After a median interval of 17 months (range, 3–72 months), 22 of 27 (81%) reported improvement postimmunotherapy; 18 were seizure free. The median time from seizure onset to initiating immunotherapy was 4 months for responders and 22 months for nonresponders (P<.05). All voltage-gated potassium channel complex antibody–positive patients reported initial or lasting benefit (P<.05). One voltage-gated potassium channel complex antibody–positive patient was seizure free after

  8. [Autoimmune epilepsy].

    Science.gov (United States)

    Seeck, M; Zacharia, A; Rossetti, A O

    2010-05-01

    There is increasing recognition of an autoimmune origin of pharmacoresistant epileptic disorders. Besides the paraneoplastic limbic encephalopathies (LE), reports of syndromes of non-paraneoplastic LE are increasingly reported in the last 5-10 years. Three antibodies are now relatively well described: Voltage-gated potassium channels (VGKC), glutamic acid decarboxylase (GAD) and N-methyl-D-aspartate receptor-(NMDA) antibodies. We review clinical syndromes, associated imaging and laboratory findings. While most reports arise from adult populations, children and adolescents are also concerned as evidenced by increasing observations. Early recognition is mandatory, since early immunomodulatory treatment appears to be related to significantly better outcome. PMID:20499581

  9. Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcal Infections (PANDAS): Experience at a Tertiary Referral Center

    OpenAIRE

    Helm, Caitlin E.; Blackwood, R. Alexander

    2015-01-01

    Background Pediatric autoimmune neuropsychiatric disorder associated with streptococcal infections (PANDAS) is an autoimmune disorder presenting with obsessive compulsive disorder and/or tics. Like Sydenham’s chorea, its presumed pathogenesis consists of autoantibodies cross-reacting with neurons in response to a group A beta-hemolytic streptococcal infection (GASI). There are currently no diagnostic laboratory findings and management ranges from antibiotic prophylaxis to intravenous immunogl...

  10. Is Tourette's syndrome an autoimmune disease?

    Science.gov (United States)

    Hoekstra, P J; Kallenberg, C G M; Korf, J; Minderaa, R B

    2002-01-01

    We provide a review of recent research findings which support the involvement of autoimmunity in childhood-onset tic disorders, in particular the presence of antineuronal autoantibodies, D8/17 B lymphocyte overexpression, a marker of chorea associated with streptococcal infection, and possible beneficial effects of immunomodulatory intervention. One of the most controversial areas in this field is the validity of the proposed PANDAS concept. Some researchers have delineated a putatively unique subgroup of patients, from the spectrum of illness encompassing Tourette's syndrome and obsessive-compulsive disorder (OCD), whose tics and obsessive-compulsive symptoms are shown to arise in response to beta-hemolytic streptococcal infections. They designated it by the term pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS). Herein we additionally present pros and cons concerning the concept of PANDAS. Finally, recommendations for future research directions are given.

  11. Update in Endocrine Autoimmunity

    OpenAIRE

    Anderson, Mark S.

    2008-01-01

    Context: The endocrine system is a common target in pathogenic autoimmune responses, and there has been recent progress in our understanding, diagnosis, and treatment of autoimmune endocrine diseases.

  12. Depleção de célula B no tratamento de citopenias auto-imunes B-Cell depletion in the treatment of autoimmune cytopenias

    Directory of Open Access Journals (Sweden)

    Luciana Landeiro

    2005-06-01

    Full Text Available A morbidade associada ao tratamento de citopenias auto-imunes tornou necessária a busca por novas terapêuticas. Baseado no fato de que o rituximab reage especificamente contra o antígeno CD 20, induzindo depleção de células B e conseqüentemente levando à diminuição na produção de auto-anticorpos, cinco pacientes com citopenias auto-imunes foram tratados com esta droga. Os pacientes eram refratários à terapia convencional e receberam 375 mg/m² de rituximab semanalmente, por um período de quatro semanas. Todos os pacientes apresentaram melhora, seja pelo aumento do número de células (níveis de hemoglobina ou contagem de plaquetas, seja pela suspensão do uso de corticoesteróides. Não foram observadas reações importantes durante infusão do medicamento, ou mesmo episódios de infecção durante acompanhamento subseqüente. Desta forma, o rituximab se mostrou eficaz e seguro para pacientes portadores de anemia hemolítica e púrpura trombocitopênica de etiologia imunológica, sugerindo que esta droga deva fazer parte do arsenal terapêutico utilizado nestas doenças auto-imunes.The morbidity associated with the treatment of autoimmune cytopenias has created a need for new approaches. Based on the fact that rituximab reacts specifically against the CD 20 antigen and induces B-cell depletion interfering with the production of auto-antibodies, five patients with autoimmune cytopenias were treated. All patients were previously refractory to conventional therapy and received 375 mg/m² of rituximab infusion weekly, for four weeks. All patients improved either by increasing the number of cells or by being able to reach steroid suspension. No major reactions occurred during infusion, and no major infections occurred during the follow up. Rituximab appears to be active and safe for patients with autoimmune hemolytic anemia and thrombocytopenia, suggesting that this agent can play an important part in the therapeutic arsenal for

  13. Autoimmune pancreatitis

    DEFF Research Database (Denmark)

    Detlefsen, Sönke; Drewes, Asbjørn M

    2009-01-01

    bile duct. Obstructive jaundice is a common symptom at presentation, and pancreatic cancer represents an important clinical differential diagnosis. In late stages of the disease, the normal pancreatic parenchyma is often replaced by large amounts of fibrosis. Histologically, there seem to be two...... AIP responds to steroid treatment, also a trial with steroids, can help to differentiate AIP from pancreatic cancer. OUTLOOK AND DISCUSSION: This review presents the pathological, radiologic and laboratory findings of AIP. Moreover, the treatment and pathogenesis are discussed.......BACKGROUND: Autoimmune pancreatitis (AIP) is a relatively newly recognized type of pancreatitis that is characterized by diffuse or focal swelling of the pancreas due to lymphoplasmacytic infiltration and fibrosis of the pancreatic parenchyma. MATERIAL AND METHODS: A PubMed literature search was...

  14. Celiac disease and autoimmune thyroid disease.

    Science.gov (United States)

    Ch'ng, Chin Lye; Jones, M Keston; Kingham, Jeremy G C

    2007-10-01

    Celiac disease (CD) or gluten sensitive enteropathy is relatively common in western populations with prevalence around 1%. With the recent availability of sensitive and specific serological testing, many patients who are either asymptomatic or have subtle symptoms can be shown to have CD. Patients with CD have modest increases in risks of malignancy and mortality compared to controls. The mortality among CD patients who comply poorly with a gluten-free diet is greater than in compliant patients. The pattern of presentation of CD has altered over the past three decades. Many cases are now detected in adulthood during investigation of problems as diverse as anemia, osteoporosis, autoimmune disorders, unexplained neurological syndromes, infertility and chronic hypertransaminasemia of uncertain cause. Among autoimmune disorders, increased prevalence of CD has been found in patients with autoimmune thyroid disease, type 1 diabetes mellitus, autoimmune liver diseases and inflammatory bowel disease. Prevalence of CD was noted to be 1% to 19% in patients with type 1 diabetes mellitus, 2% to 5% in autoimmune thyroid disorders and 3% to 7% in primary biliary cirrhosis in prospective studies. Conversely, there is also an increased prevalence of immune based disorders among patients with CD. The pathogenesis of co-existent autoimmune thyroid disease and CD is not known, but these conditions share similar HLA haplotypes and are associated with the gene encoding cytotoxic T-lymphocyte-associated antigen-4. Screening high risk patients for CD, such as those with autoimmune diseases, is a reasonable strategy given the increased prevalence. Treatment of CD with a gluten-free diet should reduce the recognized complications of this disease and provide benefits in both general health and perhaps life expectancy. It also improves glycemic control in patients with type 1 diabetes mellitus and enhances the absorption of medications for associated hypothyroidism and osteoporosis. It

  15. [Pernicious anemia: diagnosis and course in Burkina Faso].

    Science.gov (United States)

    Koulidiati, J; Sawadogo, S; Sagna, Y; Somda, K S; Tieno, H; Kafando, E; Drabo, Y J

    2015-01-01

    Pernicious anemia (also known as Biermer disease or anemia, Addison or Addisonian anemia, and Addison-Biermer anemia) is an autoimmune atrophic gastritis responsible for vitamin B12 malabsorption due to a deficiency of intrinsic factor. We report eight cases of pernicious anemia in Burkina Faso, collected over a 44-month period. The three criteria for diagnosis of pernicious anemia were: vitamin B12 deficiency, gastric disease (gastric histology) with presence of anti-intrinsic factor, and/or anti-gastric parietal cell antibodies in serum. All patients had anemia, with a mean hemoglobin level of 8.75 g/100 mL. The average mean corpuscular volume (MCV) was 122.1 fL the average mean corpuscular hemoglobin (MCH) 39.3 pg, the mean reticulocyte count 12.069 10(9)/L reticulocytes, and the mean rate of megaloblast marrow cells 17.2%. The serum vitamin B12 level ranged from 35 to 71 pmol/L. Antibodies against intrinsic factor were found in all eight patients. All ABO blood groups were present with a predominance (4 cases) of group O. Endoscopy found a normal fundic mucosa in three patients. Histology showed gastric atrophy and intestinal metaplasia for six patients (85.7%). Under B12 vitamin therapy, the course was favorable in all patients; seven patients also had 10 days of iron therapy. We recommend a gastric biopsy even in the absence of macroscopic gastric lesions on the upper gastrointestinal endoscopy. PMID:25787024

  16. Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

    Directory of Open Access Journals (Sweden)

    Parissis Haralabos

    2010-09-01

    Full Text Available Abstract Background To report our experience of a rather uncommon drug interaction, resulting in hemolytic uremic syndrome (HUS. Methods Two consecutive cases of hemolytic uremic syndrome were diagnosed in our service. In both patients the use of macrolides in patients taking Tacrolimus, resulted in high levels of Tacrolimus. Results The first patient was a 48 years old female with Bilateral emphysema. She underwent Single Sequential Lung Transplantation. She developed reperfusion injury requiring prolonged stay. Tacrolimus introduced (Day 51. The patient remained well up till 5 months later; Erythromycin commenced for chest infection. High Tacrolimus levels and a clinical diagnosis of HUS were made. She was treated with plasmapheresis successfully. The second case was a 57 years old female with Emphysema & A1 Antithrypsin deficiency. She underwent Right Single Lung Transplantation. A2 rejection with mild Obliterative Bronchiolitis diagnosed 1 year later and she switched to Tacrolimus. She was admitted to her local Hospital two and a half years later with right middle lobe consolidation. The patient commenced on amoxicillin and clarithromycin. Worsening renal indices, high Tacrolimus levels, hemolytic anemia & low Platelets were detected. HUS diagnosed & treated with plasmapheresis. Conclusions There are 21 cases of HUS following lung transplantation in the literature that may have been induced by high tacrolimus levels. Macrolides in patients taking Cyclosporin or Tacrolimus lead to high levels. Mechanism of action could be glomeruloconstrictor effect with reduced GFR increased production of Endothelin-1 and increased Platelet aggregation.

  17. Laboratory Evaluation of Anemia

    OpenAIRE

    Wallerstein, Ralph O.

    1987-01-01

    The laboratory evaluation of anemia begins with a complete blood count and reticulocyte count. The anemia is then categorized as microcytic, macrocytic or normocytic, with or without reticulocytosis. Examination of the peripheral smear and a small number of specific tests confirm the diagnosis. The serum iron level, total iron-binding capacity, serum ferritin level and hemoglobin electrophoresis generally separate the microcytic anemias. The erythrocyte size-distribution width may be particul...

  18. Investigation of the Etiology of Anemia in Thromboangiitis Obliterans.

    Science.gov (United States)

    Akbarin, Mohammad Mehdi; Ravari, Hassan; Rajabnejad, Ataollah; Valizadeh, Narges; Fazeli, Bahare

    2016-09-01

    During a review of patients admitted with thromboangiitis obliterans (TAO), there was evidence of normochromic normocytic anemia and abrupt changes in hemoglobin (Hgb) levels in patients with several hospital admissions. Therefore, the evidence of hemolytic anemia was evaluated based on 37 banked plasma samples taken from Caucasian male TAO patients during disease exacerbation between 2012 and 2014. The patients' hospital records, including clinical manifestations and complete blood count, were evaluated. The following tests were performed on all samples: indirect antiglobulin test (IAT), C-reactive protein (CRP), high-sensitivity CRP (hsCRP), lactate dehydrogenase (LDH), haptoglobin, indirect bilirubin, d-aspartate aminotransferase (AST), and d-alanine aminotransferase (ALT). The mean age of the patients was 40 ± 7 years. Two patients underwent below-knee amputation. The mean hospital-documented Hgb of the patients was 12.9 ± 2.6 g/dL. CRP and IAT were positive in 75.6 and 70.2% of the samples, respectively. The tests and corresponding results were as follows: hsCRP, 14.07 ± 2.37 µg/mL; LDH, 2,552 ± 315 u/L; haptoglobin, 2.27 ± 1.1 g/L; indirect bilirubin, 0.09 ± 0.04 mg/dL; AST, 67 ± 7 u/L; and ALT, 26 ± 3 u/L. There was a significant inverse correlation between hsCRP and hospital-documented Hgb level (p = 0.03). Anemia with the positive IAT in most of the samples, high LDH and AST, and normal ALT are suggestive of hemolytic anemia. Normal indirect bilirubin is consistent with intravascular hemolysis. The positive CRP and elevated haptoglobin levels could be due to systemic inflammation in TAO. However, it is not known if an autoantigen or an infectious antigen is responsible for TAO systemic inflammation and induction hemolytic anemia. As such, the underlying mechanism of anemia in TAO could be part of the footprint of its main etiology. PMID:27574381

  19. Evaluation of Anemia.

    Science.gov (United States)

    Kujovich, Jody L

    2016-06-01

    Anemia is a common problem in primary care. Classification based on mean cell volume narrows the differential diagnosis and directs testing. A marked macrocytosis is characteristic of vitamin B12 and folate deficiencies, certain medications, and primary bone marrow disorders. The three most common causes of microcytic anemia are iron deficiency, thalassemia trait, and anemia of inflammation. Additional laboratory testing is required for diagnosis. Determination of the rate of development of anemia and examination of a blood smear may provide diagnostic clues to guide more specialized testing. Diagnosis of iron, vitamin B12, or folate deficiency mandates determination of the underlying cause. PMID:27212091

  20. The autoimmune tautology

    OpenAIRE

    Anaya, Juan-Manuel

    2010-01-01

    Although autoimmune diseases exhibit contrasting epidemiological features, pathology, and clinical manifestations, three lines of evidence demonstrate that these diseases share similar immunogenetic mechanisms (that is, autoimmune tautology). First, clinical evidence highlights the co-occurrence of distinct autoimmune diseases within an individual (that is, polyautoimmunity) and within members of a nuclear family (that is, familial autoimmunity). Second, physiopathologic evidence indicates th...

  1. Sickle Cell Anemia (For Teens)

    Science.gov (United States)

    ... Can You Do to Stay Well? en español Anemia falciforme What Is Sickle Cell Disease? Sickle cell ... about 10 to 20 days. This usually causes anemia . Anemia is what happens when the body's number ...

  2. Aplastic Anemia and Myelodysplastic Syndromes

    Science.gov (United States)

    ... Organizations (PDF, 270 KB). Alternate Language URL Aplastic Anemia and Myelodysplastic Syndromes Page Content On this page: ... References For More Information Acknowledgments What are aplastic anemia and myelodysplastic syndromes (MDS)? Aplastic anemia and myelodysplastic ...

  3. Genetics Home Reference: Fanconi anemia

    Science.gov (United States)

    ... Understand Genetics Home Health Conditions Fanconi anemia Fanconi anemia Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Fanconi anemia is a condition that affects many parts of ...

  4. How Is Aplastic Anemia Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Aplastic Anemia Treated? Treatments for aplastic anemia include blood transfusions , blood and marrow stem cell ... a transplant. Removing a known cause of aplastic anemia, such as exposure to a toxin, also may ...

  5. How Is Pernicious Anemia Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Pernicious Anemia Treated? Doctors treat pernicious anemia by replacing the missing vitamin B12 in the body. People who have pernicious anemia may need lifelong treatment. The goals of treating ...

  6. Genetics Home Reference: atypical hemolytic-uremic syndrome

    Science.gov (United States)

    ... the genes associated with atypical hemolytic-uremic syndrome C3 CD46 CFB CFH CFHR5 CFI THBD Related Information ... Manual Consumer Version: Thrombocytopenia Merck Manual Professional Version: Complement System Orphanet: Atypical hemolytic-uremic syndrome Patient Support ...

  7. Sickle Cell Anemia

    Science.gov (United States)

    Sickle cell anemia is a disease in which your body produces abnormally shaped red blood cells. The cells are shaped like a crescent or sickle. They ... last as long as normal, round red blood cells. This leads to anemia. The sickle cells also ...

  8. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Blood Tests Blood Transfusion Restless Legs Syndrome Send a link to NHLBI to someone by E-MAIL | ... Iron-Deficiency Anemia? Español Iron-deficiency anemia is a common, easily treated condition that occurs if you ...

  9. The autoimmune tautology.

    Science.gov (United States)

    Anaya, Juan-Manuel

    2010-01-01

    Although autoimmune diseases exhibit contrasting epidemiological features, pathology, and clinical manifestations, three lines of evidence demonstrate that these diseases share similar immunogenetic mechanisms (that is, autoimmune tautology). First, clinical evidence highlights the co-occurrence of distinct autoimmune diseases within an individual (that is, polyautoimmunity) and within members of a nuclear family (that is, familial autoimmunity). Second, physiopathologic evidence indicates that the pathologic mechanisms may be similar among autoimmune diseases. Lastly, genetic evidence shows that autoimmune phenotypes might represent pleiotropic outcomes of the interaction of non-specific disease genes.

  10. Questions and Answers on Autoimmunity and Autoimmune Diseases

    Science.gov (United States)

    ... dermatomyositis . What are some of the treatments for autoimmune diseases? Of first importance in treating any autoimmune disease ... being researched. What is the family connection in autoimmune diseases? The ability to develop an autoimmune disease is ...

  11. Shock: A possible presenting manifestation of autoimmune polyendocrine syndrome type II

    Directory of Open Access Journals (Sweden)

    Subodh Banzal

    2014-01-01

    Full Text Available Autoimmune polyendocrine syndrome Type II (APS II, also known as polyglandular autoimmune syndrome Type II or Schmidt syndrome, is constellations of multiple endocrine gland insufficiencies. It is a rare, but most common of the immunoendocrinopathy syndrome. It is characterized by the obligatory occurrence of autoimmune Addison′s disease in combination with thyroid autoimmune diseases and/or Type I diabetes, hypogonadism, hypophysitis, myasthenia gravis, vitiligo, alopecia, pernicious anemia, and celiac disease. Here, we report a case of 38-year-old female patient presented with shock, further diagnosed to have APS II.

  12. The influence of pregnancy on the development of autoimmunity in chronic lymphocytic leukemia

    DEFF Research Database (Denmark)

    Jønsson, Viggo; Bock, Johannes E; Hilden, Jørgen;

    2006-01-01

    -years of follow-up. Autoimmune manifestations were analyzed in 194 women with known obstetric history and known number of long-term sexual partners, and in the 389 male CLL patients for comparison. One hundred and fifty-nine of the CLL patients exhibited autoimmune manifestations, 38% in females and 21% in men...... additional sexual partner (the odds of autoimmunity increased 11 times with each long-term sexual partner). The average numbers of pregnancies in female CLL patients with and without autoimmunity were 4.92 and 2.24, respectively (P anemia, a gastric ulcer with parietal...

  13. Autoimmune Polyglandular Syndrome Type 3 with Anorexia

    Directory of Open Access Journals (Sweden)

    Toshio Kahara

    2012-01-01

    Full Text Available A 71-year-old man with diabetes mellitus visited our hospital with complaints of anorexia and weight loss (12 kg/3 months. He had megaloblastic anemia, cobalamin level was low, and autoantibody to intrinsic factor was positive. He was treated with intramuscular cyanocobalamin, and he was able to consume meals. GAD autoantibody and ICA were positive, and he was diagnosed with slowly progressive type 1 diabetes mellitus (SPIDDM. Thyroid autoantibodies were positive. According to these findings, he was diagnosed with autoimmune polyglandular syndrome type 3 with SPIDDM, pernicious anemia, and Hashimoto's thyroiditis. Extended periods of cobalamin deficiency can cause serious complications such as ataxia and dementia, and these complications may not be reversible if replacement therapy with cobalamin is delayed. Although type 1 diabetes mellitus with coexisting pernicious anemia is very rare in Japan, physicians should consider the possibility of pernicious anemia when patients with diabetes mellitus have cryptogenic anorexia with the finding of significant macrocytosis (MCV > 100 fL.

  14. Evaluation of Macrocytic Anemias.

    Science.gov (United States)

    Green, Ralph; Dwyre, Denis M

    2015-10-01

    Macrocytic anemia, defined as a mean cell volume (MCV) ≥100 fL in adults, has a narrow differential diagnosis that requires evaluation of the peripheral blood smear as well as additional laboratory testing taken in conjunction with clinical information that includes patient history and physical examination findings. This review is an update on the approach to a patient with macrocytic anemia with attention paid to the differentiation of megaloblastic and non-megaloblastic macrocytic anemias. Critical to the determination of the diagnosis is the judicious use of laboratory testing and the evaluation of those findings in conjunction with the patient medical, surgical, and medication history. PMID:26404440

  15. Autoimmune Autonomic Ganglionopathy

    Science.gov (United States)

    ... Accessed 9/2/2015. Autoimmune Autonomic Ganglionopathy Summary. Dysautonomia International . http://www.dysautonomiainternational.org/page.php?ID= ... page Basic Information In Depth Information Basic Information Dysautonomia International offers an information page on Autoimmune autonomic ...

  16. Unexplained Anemia in the Elderly

    OpenAIRE

    Makipour, Sasan; Kanapuru, Bindu; Ershler, William B.

    2008-01-01

    Among the elderly, anemia occurs with increasing frequency with each advancing decade. Unlike when anemia occurs in younger adults, the cause of anemia in the elderly is oftentimes not readily apparent or attributable to a single cause. However, this commonly observed form of anemia in the elderly (termed unexplained anemia [UA]) can generally be dissected to its root causes, which include renal insufficiency, inflammation, testosterone deficiency, and stem cell proliferative decline. Myelody...

  17. Perspectives on autoimmunity

    Energy Technology Data Exchange (ETDEWEB)

    Cohen, I.R.

    1987-01-01

    The contents of this book are: HLA and Autoimmunity; Self-Recognition and Symmetry in the Immune System; Immunology of Insulin Dependent Diabetes Mellitus; Multiple Sclerosis; Autoimmunity and Immune Pathological Aspects of Virus Disease; Analyses of the Idiotypes and Ligand Binding Characteristics of Human Monoclonal Autoantibodies to DNA: Do We Understand Better Systemic Lupus Erythematosus. Autoimmunity and Rheumatic Fever; Autoimmune Arthritis Induced by Immunization to Mycobacterial Antigens; and The Interaction Between Genetic Factors and Micro-Organisms in Ankylosing Spondylitis: Facts and Fiction.

  18. Severe hemolytic transfusion reaction due to anti-D in a D+ patient with sickle cell disease

    OpenAIRE

    Ipe, Tina S.; Wilkes, Jennifer J.; Hartung, Helge D.; Westhoff, Connie M.; Chou, Stella T.; Friedman, David F.

    2015-01-01

    A 5-year-old male with sickle cell disease presented with pain, dark urine, and fatigue 10 days after a red blood cell (RBC) transfusion. Laboratory evaluation demonstrated severe anemia, blood type O+, and anti-D in the serum. Anti-D in a D+ patient led to RH genotyping which revealed homozygosity for RHD*DAU4 that encodes partial D antigen. Anti-D in this patient whose RBCs exclusively express partial D caused a delayed hemolytic transfusion reaction after exposure to D+ RBCs. The finding o...

  19. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... intravenous iron therapy. Rate This Content: NEXT >> Featured Video Living With and Managing Iron-Deficiency Anemia 05/18/2011 This video—presented by the National Heart, Lung, and Blood ...

  20. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... symptoms. Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in ... 18/2011 This video—presented by the National Heart, Lung, and Blood Institute, part of the National ...

  1. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Entire Site Health Topics News & Resources Intramural Research Public Health Topics Education & Awareness Resources Contact The Health ... Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in children, ...

  2. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Health Topics Education & Awareness Resources Contact The Health Information Center Health Professionals Systematic Evidence Reviews & Clinical Practice ... and see the benefits of treatment. For more information about living with and managing iron-deficiency anemia, ...

  3. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... CAUSES WHO IS AT RISK SIGNS & SYMPTOMS DIAGNOSIS TREATMENTS PREVENTION LIVING WITH CLINICAL TRIALS LINKS Related Topics ... Doctors usually can successfully treat iron-deficiency anemia. Treatment will depend on the cause and severity of ...

  4. Sickle cell anemia

    Science.gov (United States)

    ... for avascular necrosis of the hip Surgery for eye problems Treatment for overuse or abuse of narcotic pain medicines Wound care for leg ulcers Bone marrow or stem cell transplants can cure sickle cell anemia, but this treatment ...

  5. Mouse models of Fanconi anemia

    Energy Technology Data Exchange (ETDEWEB)

    Parmar, Kalindi; D' Andrea, Alan [Department of Radiation Oncology, Dana-Farber Cancer Institute, Harvard Medical School, 44 Binney Street, Boston, MA 02115 (United States); Niedernhofer, Laura J., E-mail: niedernhoferl@upmc.edu [Department of Microbiology and Molecular Genetics, University of Pittsburgh School of Medicine and Cancer Institute, 5117 Centre Avenue, Hillman Cancer Center, Research Pavilion 2.6, Pittsburgh, PA 15213-1863 (United States)

    2009-07-31

    Fanconi anemia is a rare inherited disease characterized by congenital anomalies, growth retardation, aplastic anemia and an increased risk of acute myeloid leukemia and squamous cell carcinomas. The disease is caused by mutation in genes encoding proteins required for the Fanconi anemia pathway, a response mechanism to replicative stress, including that caused by genotoxins that cause DNA interstrand crosslinks. Defects in the Fanconi anemia pathway lead to genomic instability and apoptosis of proliferating cells. To date, 13 complementation groups of Fanconi anemia were identified. Five of these genes have been deleted or mutated in the mouse, as well as a sixth key regulatory gene, to create mouse models of Fanconi anemia. This review summarizes the phenotype of each of the Fanconi anemia mouse models and highlights how genetic and interventional studies using the strains have yielded novel insight into therapeutic strategies for Fanconi anemia and into how the Fanconi anemia pathway protects against genomic instability.

  6. Megaloblastic anemia in Japan

    Directory of Open Access Journals (Sweden)

    Taguchi,Hirokuni

    1978-08-01

    Full Text Available Since 1903, 744 cases of megaloblastic anemia have been reported in Japan: 490 cases of pernicious anemia; 95 cases associated with pregnancy; 66 cases after gastrectomy; 22 cases of megaloblastic anemia of infants; 21 cases of folic acid deficiency other than pregnancy and 19 cases of vitamin B12 malabsorption after ileal resection. It is generally agreed among hematologists in Japan that pernicious anemia is relatively rare, as in other Asian countries. The diagnosis of pernicious anemia in Japan is usually made by stained marrow films, radioisotopic assay of serum vitamin B12, Schilling test and good response to vitamin B12 therapy. Serum folate level, intrinsic factor or its antibody, methylmalonic acid excretion, formiminoglutamic acid excretion and deoxyuridine suppression test are performed only at a small number of laboratories. The drugs of choice are hydroxocobalamin, deoxyadenosylcobalamin and methylcobalamin. Cyanocobalamin has nearly disappeared from commercial sources in Japan. Vitamin B12 administration is common in patients with neurological disorders. Megaloblastic anemia due to folic acid deficiency is extremely rare in Japan. Low serum folate levels are frequently observed among patients receiving anticonvulsants or in pregnant women, but in such samples megaloblastic anemia is almost never detected. The folic acid content of hospital diets indicates that satisfactory amounts of folate are taken in Japan. The intake of folic acid from rice is well over the minimum daily requirement of folate. Other factors in folic acid deficiency, such as food taboos, severe alcoholism and malabsorption syndrome are not frequently found in Japanese. The inadequate intake of folate was the critical factor in most reported cases.

  7. Acute Systolic Heart Failure Associated with Complement-Mediated Hemolytic Uremic Syndrome

    Directory of Open Access Journals (Sweden)

    John L. Vaughn

    2015-01-01

    Full Text Available Complement-mediated hemolytic uremic syndrome (otherwise known as atypical HUS is a rare disorder of uncontrolled complement activation that may be associated with heart failure. We report the case of a 49-year-old female with no history of heart disease who presented with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given her normal ADAMSTS13 activity, evidence of increased complement activation, and renal biopsy showing evidence of thrombotic microangiopathy, she was diagnosed with complement-mediated HUS. She subsequently developed acute hypoxemic respiratory failure secondary to pulmonary edema requiring intubation and mechanical ventilation. A transthoracic echocardiogram showed evidence of a Takotsubo cardiomyopathy with an estimated left ventricular ejection fraction of 20%, though ischemic cardiomyopathy could not be ruled out. Treatment was initiated with eculizumab. After several failed attempts at extubation, she eventually underwent tracheotomy. She also required hemodialysis to improve her uremia and hypervolemia. After seven weeks of hospitalization and five doses of eculizumab, her renal function and respiratory status improved, and she was discharged in stable condition on room air and independent of hemodialysis. Our case illustrates a rare association between acute systolic heart failure and complement-mediated HUS and highlights the potential of eculizumab in stabilizing even the most critically-ill patients with complement-mediated disease.

  8. Low levels of serum erythropoietin in children with endemic hemolytic uremic syndrome.

    Science.gov (United States)

    Exeni, R; Donato, H; Rendo, P; Antonuccio, M; Rapetti, M C; Grimoldi, I; Exeni, A; de Galvagni, A; Trepacka, E; Amore, A

    1998-04-01

    Serum erythropoietin (EPO) levels were measured in ten previously non-transfused children with hemolytic uremic syndrome (HUS). Complete blood cell count, serum EPO, and renal function tests were carried out upon admission and weekly thereafter. Blood samples were obtained: (1) prior to the first transfusion; (2) after the first transfusion but before recovery from renal failure; (3) during the recovery stage. All patients required transfusions (mean 1.8+/-0.8 per child). Absolute values of EPO correlated positively with the hematocrit during the three stages (r = 0.53, 0.36, and 0.12, respectively) which is opposite to expected results. The observed EPO logarithm/predicted EPO logarithm upon admission was low (0.70+/-0.08), falling further during stage 2 (0.57+/-0.03), but increasing thereafter (0.78+/-0.07) without reaching normal values. The reticulocyte production rate followed a parallel course (0.74+/-0.14, 0.54+/-0.11, and 0.60+/-0.10, respectively). On comparing the observed serum EPO levels with those expected, 9 of 11 pre-transfusion samples showed low values; in stage 2, all samples were below normal; in the recovery phase most (77.8%) were still low. Our results show an inadequate EPO synthesis in children with HUS, which could play an important pathogenic role, since it aggravates the severity of the existing hemolytic anemia; the secondary inhibitory effect of repeated transfusions exacerbates this inadequate synthesis.

  9. Induction of Neutrophil Extracellular Traps in Shiga Toxin-Associated Hemolytic Uremic Syndrome.

    Science.gov (United States)

    Ramos, Maria Victoria; Mejias, Maria Pilar; Sabbione, Florencia; Fernandez-Brando, Romina Jimena; Santiago, Adriana Patricia; Amaral, Maria Marta; Exeni, Ramon; Trevani, Analia Silvina; Palermo, Marina Sandra

    2016-01-01

    Hemolytic uremic syndrome (HUS), a vascular disease characterized by hemolytic anemia, thrombocytopenia, and acute renal failure, is caused by enterohemorrhagic Shiga toxin (Stx)-producing bacteria, which mainly affect children. Besides Stx, the inflammatory response mediated by neutrophils (PMN) is essential to HUS evolution. PMN can release neutrophil extracellular traps (NET) composed of DNA, histones, and other proteins. Since NET are involved in infectious and inflammatory diseases, the aim of this work was to investigate the contribution of NET to HUS. Plasma from HUS patients contained increased levels of circulating free-DNA and nucleosomes in comparison to plasma from healthy children. Neutrophils from HUS patients exhibited a greater capacity to undergo spontaneous NETosis. NET activated human glomerular endothelial cells, stimulating secretion of the proinflammatory cytokines IL-6 and IL-8. Stx induced PMN activation as judged by its ability to trigger reactive oxygen species production, increase CD11b and CD66b expression, and induce NETosis in PMN from healthy donors. During HUS, NET can contribute to the inflammatory response and thrombosis in the microvasculature and thus to renal failure. Intervention strategies to inhibit inflammatory mechanisms mediated by PMN, such as NETosis, could have a potential therapeutic impact towards amelioration of the severity of HUS.

  10. Development of cerebral venous sinus thrombosis in an aplastic anemia patient with antiphospholipid syndrome

    Institute of Scientific and Technical Information of China (English)

    CHEN Jian-hua; YOU Xin; QIAN Min

    2010-01-01

    @@ Aplastic anemia (AA) is an acquired disorder in which bone marrow fails to produce or release sufficient amounts of blood cell. Antiphospholipid syndrome (APS)is an autoimmune disease characterised by recurrent arterial or venous thrombosis, pregnancy morbidity and the persistence of positive antiphospholipid antibodies (aPL), including anticardiolipin antibody (ACL) and lupus anticoagulant (LA).

  11. Anemia in the Preoperative Patient

    OpenAIRE

    Patel, Manish S; Carson, Jeffrey L.

    2009-01-01

    Anemia is commonly encountered in the preoperative patient. With variable etiology, determination of the cause of the anemia can impact perioperative surgical and medical management and outcome. Red blood cell transfusions are often administered during the perioperative time period in patients with preoperative anemia, although evidence to support the optimal transfusion threshold is limited. We review the evaluation of anemia, as well as evidence regarding perioperative blood transfusions. R...

  12. Autoimmune pancreatitis: A review

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Autoimmune pancreatitis has emerged over the last 40 years from a proposed concept to a well established and recognized entity. As an efficient mimicker of pancreatic carcinoma, its early and appropriate recognition are crucial. With mounting understanding of its pathogenesis and natural history, significant advances have been made in the diagnosis of autoimmune pancreatitis. The characteristic laboratory features and imaging seen in autoimmune pancreatitis are reviewed along with some of the proposed diagnostic criteria and treatment algorithms.

  13. Anemia in People with Cancer

    Science.gov (United States)

    ... My ACS » Your Local Offices Close + - Text Size Anemia in People With Cancer What is anemia? When you don’t have enough healthy red ... the symptoms that bother people most. What causes anemia? There are many different reasons a person with ...

  14. Anemia in Chronic Kidney Disease

    Science.gov (United States)

    ... Disease Organizations​​ . (PDF, 345 KB)​​​​​ Alternate Language URL Anemia in CKD Page Content On this page: What ... Nutrition Points to Remember Clinical Trials What is anemia? Anemia is a condition in which the body ...

  15. How Is Fanconi Anemia Diagnosed?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Fanconi Anemia Diagnosed? People who have Fanconi anemia (FA) are born with the disorder. They may ... questions about: Any personal or family history of anemia Any surgeries you’ve had related to the ...

  16. How Is Fanconi Anemia Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Fanconi Anemia Treated? Doctors decide how to treat Fanconi anemia (FA) based on a person's age and how ... Long-term treatments for FA can: Cure the anemia. Damaged bone marrow cells are replaced with healthy ...

  17. How Is Aplastic Anemia Diagnosed?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Aplastic Anemia Diagnosed? Your doctor will diagnose aplastic anemia based on your medical and family histories, a ... your primary care doctor thinks you have aplastic anemia, he or she may refer you to a ...

  18. Severe Anemia in Malawian Children

    NARCIS (Netherlands)

    Calis, J.C.J.; Kamija, S.P.; Faragher, E.B.; Brabin, B.J.; Bates, I.; Cuevas, L.E.; Haan, de R.J.; Phiri, A.I.; Malange, P.; Khoka, M.; Hulshof, P.J.M.; Lieshout, L.; Beld, M.G.H.M.; Teo, Y.Y.; Rockett, K.A.; Richardson, A.; Kwiatkowski, D.P.; Molyneux, M.E.; Hensbroek, van M.B.

    2008-01-01

    Background Severe anemia is a major cause of sickness and death in African children, yet the causes of anemia in this population have been inadequately studied. Methods We conducted a case¿control study of 381 preschool children with severe anemia (hemoglobin concentration,

  19. Pernicious anemia: New insights from a gastroenterological point of view

    Institute of Scientific and Technical Information of China (English)

    Edith Lahner; Bruno Annibale

    2009-01-01

    Pernicious anemia (PA) is a macrocytic anemia that is caused by vitamin B_(12) deficiency, as a result of intrinsic factor deficiency. PA is associated with atrophic body gastritis (ABG), whose diagnosis is based on histological confirmation of gastric body atrophy. Serological markers that suggest oxyntic mucosa damage are increased fasting gastrin and decreased pepsinogen Ⅰ. Without performing Schilling's test,intrinsic factor deficiency may not be proven, and intrinsic factor and parietal cell antibodies are useful surrogate markers of PA, with 73% sensitivity and 100% specificity. PA is mainly considered a disease of the elderly, but younger patients represent about 15% of patients. PA patients may seek medical advice due to symptoms related to anemia, such as weakness and asthenia. Less commonly, the disease is suspected to be caused by dyspepsia. PA is frequently associated with autoimmune thyroid disease (40%) and other autoimmune disorders, such as diabetes mellitus (10%), as part of the autoimmune polyendocrine syndrome. PA is the end-stage of ABG. Longstanding Helicobacter pylori infection probably plays a role in many patients with PA, in whom the active infectious process has been gradually replaced by an autoimmune disease that terminates in a burned-out infection and the irreversible destruction of the gastric body mucosa. Human leucocyte antigen-DR genotypes suggest a role for genetic susceptibility in PA. PA patients should be managed by cobalamin replacement treatment and monitoring for onset of iron deficiency. Moreover, they should be advised about possible gastrointestinal long-term consequences, such as gastric cancer and carcinoids.

  20. Evaluation of clinical, biochemical and hematological parameters in macrocytic anemia

    Directory of Open Access Journals (Sweden)

    Aarthi Kannan

    2016-07-01

    Results: Primary bone marrow disorders were the most common cause of macrocytosis (46%. The other causes in decreasing order of frequency were megaloblastic anaemia (38%, hemolytic anemia (6%, drug induced (5%, alcoholism and liver disease (4% and idiopathic thrombocytopenic purpura (1%. There was a significant difference in the mean values of MCV and serum LDH between megaloblastic and non and ndash; megaloblastic macrocytosis. When serum LDH >1345.2 IU/L or MCV>121fl (criterion values of ROC curve with reticulocyte count <2% was taken as criteria, the sensitivity was 92.1% and specificity was 93.5% for diagnosing megaloblastic anemia. Conclusions: Systematic evaluation of macrocytosis will help us to distinguish megaloblastic and non and ndash; megaloblastic macrocytosis. The blood and biochemical parameters especially CBC, RC, and serum LDH along with supporting clinical features help us in diagnosing megaloblastic anemia in a setup where vitamin and metabolite levels are difficult to obtain. [Int J Res Med Sci 2016; 4(7.000: 2670-2678

  1. American Autoimmune Related Diseases Association

    Science.gov (United States)

    ... Its 25th Anniversary With #25FOR25 Campaign During National Autoimmune Disease Awareness Month AARDA officially kicks of National Autoimmune ... will benefit AARDA. Click here to read more. Autoimmune Disease Awareness Month AARDA and the NCAPG held two ...

  2. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... a lower than normal number of red blood cells. Red blood cells carry oxygen and remove carbon dioxide (a waste ... Anemia also can occur if your red blood cells don't contain enough hemoglobin (HEE-muh-glow- ...

  3. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... the body. Iron-deficiency anemia usually develops over time if your body doesn't have enough iron ... Institutes of Health—shows how Susan, a full-time worker and student, has coped with having iron- ...

  4. Sickle Cell Anemia Bibliography.

    Science.gov (United States)

    Christy, Steven C.

    Presents sources for the acquisition of medical, social, psychological, educational, and practical knowledge of sickle cell anemia. The materials listed are designed to help parents, educators, and public service workers. Materials include journal articles, films, brochures, slides, and fact sheets. The usual bibliographic information is given.…

  5. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... of red blood cells. Red blood cells carry oxygen and remove carbon dioxide (a waste product) from your body. Anemia ... Hemoglobin is an iron-rich protein that carries oxygen from the lungs to the rest of the ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... of red blood cells. Red blood cells carry oxygen and remove carbon dioxide (a waste product) from your body. Anemia also can occur if your red blood cells don't contain enough hemoglobin (HEE-muh-glow-bin). Hemoglobin is an iron-rich protein that carries oxygen from the lungs to the rest of the ...

  7. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... blood has a lower than normal number of red blood cells. Red blood cells carry oxygen and remove carbon dioxide ( ... your body. Anemia also can occur if your red blood cells don't contain enough hemoglobin (HEE- ...

  8. FEBRILE SEIZURE AND ANEMIA

    Directory of Open Access Journals (Sweden)

    A. Talebian

    2008-11-01

    Full Text Available ObjectiveConsidering the controversial results in present day literature regarding the relationship between febrile seizures and anemia and the high rate of such seizures in children, this study was conducted to evaluate the association between pediatric febrile seizures and anemia.Material and MethodsIn this case-control study, conducted in 2003, 60 children with febrile seizure(cases and 60 febrile children without seizure(controls were evaluated in the Kashan Shahid Beheshti hospital; all patients were matched for age, sex, type of feeding, and use of supplemental iron. Thirty-six (60% and 39 (65% of the patients in case and control groups respectively were male, and the remaining female. Levels of hemoglobin, hematocrit, and red blood cell indices were determined in all children and Chi-square and Fisher exact tests were used to analyze data.ResultsOf the case group, 13.3% (6 male, 2 female and of controls, 20% (9 male, 3 female of children had anemia (p= 0.327, the condition being more common in male children aged over 6 months. Febrile seizures were found to occur mostly between the ages of 6 to 24 months.ConclusionThe risk of febrile seizure occurrence in anemic children seems to be less than that in children who do not suffer from the condition.Keywords:Febrile seizure, Anemia, Children

  9. Anemia and School Participation

    Science.gov (United States)

    Bobonis, Gustavo J.; Miguel, Edward; Puri-Sharma, Charu

    2006-01-01

    Anemia is among the most widespread health problems for children in developing countries. This paper evaluates the impact of a randomized health intervention delivering iron supplementation and deworming drugs to Indian preschool children. At baseline, 69 percent were anemic and 30 percent had intestinal worm infections. Weight increased among…

  10. Twin anemia polycythemia sequence

    NARCIS (Netherlands)

    Slaghekke, Femke

    2014-01-01

    In this thesis we describe that Twin Anemia Polycythemia Sequence (TAPS) is a form of chronic feto-fetal transfusion in monochorionic (identical) twins based on a small amount of blood transfusion through very small anastomoses. For the antenatal diagnosis of TAPS, Middle Cerebral Artery – Peak Syst

  11. Autoimmune Polyglandular Syndrome Type 2: An Unusual Presentation

    Directory of Open Access Journals (Sweden)

    Hamdollah Karamifar

    2010-05-01

    Full Text Available "nAutoimmune polyglandular syndrome (APS type 2 is characterized by the presence of Addison's disease, in association with autoimmune thyroid disease and/or type 1 diabetes mellitus. APS type 2 occurs most often in middle aged females and is rare in children. Here an 11 year old boy is reported with Addison's disease who developed symptom's of diabetes mellitus, goiter, malabsorption, macrocytic anemia and keratitis. APS type 2 occurs most often in middle aged females and is quite rare in children but one should think to autoimmune poly glandular syndrome type II in patient at any age especially in patients with Addison's disease.

  12. THE AUTOIMMUNE ECOLOGY.

    Directory of Open Access Journals (Sweden)

    Juan-Manuel eAnaya

    2016-04-01

    Full Text Available Autoimmune diseases (ADs represent a heterogeneous group of disorders that affect specific target organs or multiple organ systems. These conditions share common immunopathogenic mechanisms (i.e., the autoimmune tautology, which explain the clinical similarities they have among them as well as their familial clustering (i.e., coaggregation. As part of the autoimmune tautology, the influence of environmental exposure on the risk of developing ADs is paramount (i.e., the autoimmune ecology. In fact, environment, more than genetics, shapes immune system. Autoimmune ecology is akin to exposome, that is all the exposures - internal and external - across the lifespan, interacting with hereditary factors (both genetics and epigenetics to favor or protect against autoimmunity and its outcomes. Herein we provide an overview of the autoimmune ecology, focusing on the immune response to environmental agents in general, and microbiota, cigarette smoking, alcohol and coffee consumption, socioeconomic status, gender and sex hormones, vitamin D, organic solvents and vaccines in particular. Inclusion of the autoimmune ecology in disease etiology and health will improve the way personalized medicine is currently conceived and applied.

  13. Bistability in autoimmune diseases

    DEFF Research Database (Denmark)

    Rapin, Nicolas; Mosekilde, Erik; Lund, Ole

    2011-01-01

    Autoimmune diseases damage host tissue, which, in turn, may trigger a stronger immune response. Systems characterized by such positive feedback loops can display co-existing stable steady states. In a mathematical model of autoimmune disease, one steady state may correspond to the healthy state...

  14. The Autoimmune Ecology.

    Science.gov (United States)

    Anaya, Juan-Manuel; Ramirez-Santana, Carolina; Alzate, Maria A; Molano-Gonzalez, Nicolas; Rojas-Villarraga, Adriana

    2016-01-01

    Autoimmune diseases (ADs) represent a heterogeneous group of disorders that affect specific target organs or multiple organ systems. These conditions share common immunopathogenic mechanisms (i.e., the autoimmune tautology), which explain the clinical similarities they have among them as well as their familial clustering (i.e., coaggregation). As part of the autoimmune tautology, the influence of environmental exposure on the risk of developing ADs is paramount (i.e., the autoimmune ecology). In fact, environment, more than genetics, shapes immune system. Autoimmune ecology is akin to exposome, that is all the exposures - internal and external - across the lifespan, interacting with hereditary factors (both genetics and epigenetics) to favor or protect against autoimmunity and its outcomes. Herein, we provide an overview of the autoimmune ecology, focusing on the immune response to environmental agents in general, and microbiota, cigarette smoking, alcohol and coffee consumption, socioeconomic status (SES), gender and sex hormones, vitamin D, organic solvents, and vaccines in particular. Inclusion of the autoimmune ecology in disease etiology and health will improve the way personalized medicine is currently conceived and applied.

  15. Early-Onset Neutropenia Induced by Rituximab in a Patient with Lupus Nephritis and Hemolytic Anemia

    OpenAIRE

    Mariangelí Arroyo-Ávila; Fred-Jiménez, Ruth M.; Vilá, Luis M.

    2015-01-01

    Rituximab is an anti-CD20 monoclonal antibody that has been used to treat several complications of systemic lupus erythematosus (SLE) including nephritis, cerebritis, and hematological disorders. Neutropenia is among the adverse events associated with rituximab; this usually occurs several weeks after therapy. However, early-onset neutropenia has been reported only in a few cases. Herein, we describe a 36-year-old Hispanic SLE woman who developed severe early-onset neutropenia (0.3 × 109/L) a...

  16. Acute methemoglobinemia with hemolytic anemia following bio-organic plant nutrient compound exposure: Two case reports.

    Science.gov (United States)

    Malkarnekar, Santoshi Balkrishna; Anjanappa, Raveesha; Naveen, L; Kiran, B G

    2014-02-01

    Two young women, were reffered to our hospital on two different occasions with history of breathlessness and mental confusion, following consumption of two different bio-organic plant nutrient compounds with a suicidal intent. On examination, they had cyanotic mucous membranes, and their blood samples showed the classic 'dark chocolate brown' appearance. Work up revealed cyanosis unresponsive to oxygen supplementation and absence of cardiopulmonary abnormality. Pulse oximetry revealed saturation of 75% in case 1 and 80% in case 2, on 8 liters oxygen supplementation via face masks, although their arterial blood gas analysis was normal, suggestive of "saturation gap". Methemoglobinemia was suspected based on these findings and was confirmed by Carbon monoxide-oximetry (CO-oximetry). Methylene blue was administered and the patients showed dramatic improvement. Both the patients developed evidence of hemolysis approximately 72 hours following admission which improved with blood transfusion and supportive treatment. The patients were eventually discharged without any neurological sequalae. PMID:24678158

  17. ROLE OF RBC COUNT AND RBC INDICES IN DIAGNOSING AND DIFFERENTIATING ANEMIAS CAUSED DUE TO VARIOUS CLINICAL SITUATIONS IN A TERTIARY CARE HOSPITAL IN VADODARA, GUJARAT

    Directory of Open Access Journals (Sweden)

    Sandeep

    2015-11-01

    Full Text Available BACKGROUND : Latest technologies have made it easier for the pathologist and clinician to diagnose any case of anemia. Automated cell counters are widely used since last two decades that have further facilitated and made it easy for the clinicians to reach to the root cause of anemia. RBC count and entire RBC indices along with HB estimation gives a definite idea as to what could be the cause of anemia. The whole spectrum of RBC indices i.e. MCV, MCH and MCHC along with RBC count, PCV and Hemoglobin estimation plays a vital role to analyze the cause of anemia. MATERIAL AND METHODS: A cross sectional study was carried out at Parul Institute of Medical Science and Research, Limda, Vadodara, Gujarat. Investigations of RBC count and RBC indic es of patients were carried out on fully automated cell counter – by Merck diagnostics. Study included 700 patients with Hemoglobin <10gm% which included males and females of age group 15 - 50 years. RESULTS: Total 700 anemic patients with Hemoglobin < 10gm % were enrolled in the study. Among them 569 were females which included pregnant females also and 131 were males. Patients were of age group 15 - 50 years. Data revealed that 45 % of patients had iron deficiency anemia, 14 % had megaloblastic anemia, 13% ha d hemolytic anemia, 6 % had anemia due to chronic illness whereas 10 % had dimorphic type of anemia. In iron deficiency anemia, blood picture showed relatively high to normal RBC count and PCV i.e. hematocrit, typically low MCV and MCH values and low to no rmal MCHC. In megaloblastic anemia, RBC count was very low due to premature destruction of RBC’s and they had low PCV whereas high to very high MCV, MCH and MCHC values. In hemolytic anemia it is seen that RBC count is low and so is the PCV or hematocrit w hile such patients have normal to high MCV and normal MCH and MCHC. In anemia of chronic disease all parameters are relatively normal except low to normal MCV. CONCLUSION: RBC indices vary in

  18. [Severe hemolytic jaundice and Wilson's disease].

    Science.gov (United States)

    Storck, D; Bareiss, P; Jesel, B; Warter, J

    1976-12-01

    The onset of spontaneous hemolytic jaundice in a young subject should lead to the search for Wilson's disease when clinical examination reveals cirrhosis. This hemolysis may evolve in the form of severe jaundice to a stage where the cirrhosis remains usually latent or well tolerated. The intervention of a toxic, allergic of infective factor liable to produce a hepatic lesion which frees a dose of copper sufficient to trigger off hemolysis, is discussed. The mechanism of the latter, that of the coagulation disorders observed, liver cell failure and widespread intravascular coagulation, are analysed in this paper and compared with data in the literature. The dramatic character of the case indicates that it is necessary to treat as a routine with penicillamine all homozygous forms of Wilson's disease.

  19. Autoimmunity in visual loss.

    Science.gov (United States)

    Petzold, Axel; Wong, Sui; Plant, Gordon T

    2016-01-01

    There are a number of autoimmune disorders which can affect visual function. There are a very large number of mechanisms in the visual pathway which could potentially be the targets of autoimmune attack. In practice it is the retina and the anterior visual pathway (optic nerve and chiasm) that are recognised as being affected in autoimmune disorders. Multiple Sclerosis is one of the commonest causes of visual loss in young adults because of the frequency of attacks of optic neuritis in that condition, however the basis of the inflammation in Multiple Sclerosis and the confirmation of autoimmunity is lacking. The immune process is known to be highly unusual in that it is not systemic and confined to the CNS compartment. Previously an enigmatic partner to Multiple Sclerosis, Neuromyelitis Optica is now established to be autoimmune and two antibodies - to Aquaporin4 and to Myelin Oligodendrocyte Glycoprotein - have been implicated in the pathogenesis. The term Chronic Relapsing Inflammatory Optic Neuropathy is applied to those cases of optic neuritis which require long term immunosuppression and hence are presumed to be autoimmune but where no autoimmune pathogenesis has been confirmed. Optic neuritis occurring post-infection and post vaccination and conditions such as Systemic Lupus Erythematosus and various vasculitides may cause direct autoimmune attack to visual structures or indirect damage through occlusive vasculopathy. Chronic granulomatous disorders such as Sarcoidosis affect vision commonly by a variety of mechanisms, whether and how these are placed in the autoimmune panoply is unknown. As far as the retina is concerned Cancer Associated Retinopathy and Melanoma Associated Retinopathy are well characterised clinically but a candidate autoantibody (recoverin) is only described in the former disorder. Other, usually monophasic, focal retinal inflammatory disorders (Idiopathic Big Blind Spot Syndrome, Acute Zonal Occult Outer Retinopathy and Acute Macular

  20. Autoimmunity in Immunodeficiency

    Science.gov (United States)

    Todoric, Krista; Koontz, Jessica B.; Mattox, Daniel; Tarrant, Teresa K.

    2013-01-01

    Primary immunodeficiencies (PID) comprise a diverse group of clinical disorders with varied genetic defects. Paradoxically, a substantial proportion of PID patients develop autoimmune phenomena in addition to having increased susceptibility to infections from their impaired immunity. Although much of our understanding comes from data gathered through experimental models, there are several well-characterized PID that have improved our knowledge of the pathways that drive autoimmunity. The goals of this review will be to discuss these immunodeficiencies and to review the literature with respect to the proposed mechanisms for autoimmunity within each put forth to date. PMID:23591608

  1. Autoimmunity and the Gut

    Directory of Open Access Journals (Sweden)

    Andrew W. Campbell

    2014-01-01

    Full Text Available Autoimmune diseases have increased dramatically worldwide since World War II. This is coincidental with the increased production and use of chemicals both in industrial countries and agriculture, as well as the ease of travel from region to region and continent to continent, making the transfer of a pathogen or pathogens from one part of the world to another much easier than ever before. In this review, triggers of autoimmunity are examined, principally environmental. The number of possible environmental triggers is vast and includes chemicals, bacteria, viruses, and molds. Examples of these triggers are given and include the mechanism of action and method by which they bring about autoimmunity.

  2. Ribavirin-induced anemia in hepatitis C virus patients undergoing combination therapy.

    Directory of Open Access Journals (Sweden)

    Sheeja M Krishnan

    Full Text Available The current standard of care for hepatitis C virus (HCV infection - combination therapy with pegylated interferon and ribavirin - elicits sustained responses in only ∼50% of the patients treated. No alternatives exist for patients who do not respond to combination therapy. Addition of ribavirin substantially improves response rates to interferon and lowers relapse rates following the cessation of therapy, suggesting that increasing ribavirin exposure may further improve treatment response. A key limitation, however, is the toxic side-effect of ribavirin, hemolytic anemia, which often necessitates a reduction of ribavirin dosage and compromises treatment response. Maximizing treatment response thus requires striking a balance between the antiviral and hemolytic activities of ribavirin. Current models of viral kinetics describe the enhancement of treatment response due to ribavirin. Ribavirin-induced anemia, however, remains poorly understood and precludes rational optimization of combination therapy. Here, we develop a new mathematical model of the population dynamics of erythrocytes that quantitatively describes ribavirin-induced anemia in HCV patients. Based on the assumption that ribavirin accumulation decreases erythrocyte lifespan in a dose-dependent manner, model predictions capture several independent experimental observations of the accumulation of ribavirin in erythrocytes and the resulting decline of hemoglobin in HCV patients undergoing combination therapy, estimate the reduced erythrocyte lifespan during therapy, and describe inter-patient variations in the severity of ribavirin-induced anemia. Further, model predictions estimate the threshold ribavirin exposure beyond which anemia becomes intolerable and suggest guidelines for the usage of growth hormones, such as erythropoietin, that stimulate erythrocyte production and avert the reduction of ribavirin dosage, thereby improving treatment response. Our model thus facilitates, in

  3. Autoimmune Pancreatitis: A Succinct Overview

    OpenAIRE

    Juan Putra; Xiaoying Liu

    2015-01-01

    Autoimmune pancreatitis is a rare type of chronic pancreatitis with characteristic clinical, radiologic, and histopathologic findings. Diagnosis of autoimmune pancreatitis is often challenging due to its low incidence and nonspecific clinical and radiologic findings. Patients with autoimmune pancreatitis and pancreatic cancer share similar clinical presentations, including obstructive jaundice, abdominal pain and weight loss. Due to these overlapping features, autoimmune pancreatitis patients...

  4. Thiamine– Responsive Megaloblastic Anemia Syndrome

    Directory of Open Access Journals (Sweden)

    F Motavaselian

    2009-01-01

    Full Text Available Thiamine Responsive megaloblastic anemia in DIDMOA (Wolfram syndrome has an autosomal- recessive mode of inheritance . Megaloblastic anemia and sideroblastic anemia is accompanied by diabetes insipidus (DI, diabetes mellitus (DM ,optic atrophy (OA and deafness (D. Neutropenia and thrombocytopenia are also present. We report a 7 month old girl with congenital macrocytic anemia; a rare clinical feature of Wolfram,s syndrome with increased plasma levels of blood glucose, both of which dramatically responded to administration of thiamine in large doses . The patient also had neurosensorial deafness, but no improvement was observed in the deafness. We presented the case because thiamine-responsive megaloblastic anemia is a rare clinical presentation of Wolfram syndrome and after institution of treatment with thiamine, the anemia and hyperglycemia returned to normal.

  5. Anemia, Growth Failure and Hypothyroidism

    OpenAIRE

    Chaytors, Richard Gordon; Higgins, Gerald

    1980-01-01

    A 12-year-old Caucasian female presented to her family physician with an old complaint of anemia and a new complaint of failure to grow. The anemia, first observed four years previously, had been diagnosed as iron deficiency, but had never satisfactorily responded to adequate iron therapy. Investigation of the failure to grow resulted in a diagnosis of hypothyroidism with related normochromic normocytic anemia.

  6. Parvovirus B19 infection in Tunisian patients with sickle-cell anemia and acute erythroblastopenia

    Directory of Open Access Journals (Sweden)

    Zili Mohamed

    2007-10-01

    Full Text Available Abstract Background Human parvovirus B19 is the etiologic agent of erythema infectiosum in children. It is also associated with other clinical manifestations in different target groups. Patients with chronic hemolytic anemia are at high risk of developing acute erythroblastopenia following infection by the virus. They usually become highly viremic and pose an increased risk of virus transmission. Close monitoring of such high risk groups is required for epidemiologic surveillance and disease prevention activities. Here we report a molecular epidemiological study on B19 virus infection in Tunisian patients with chronic hemolytic anemia. Methods This study was conducted on 92 young chronic hemolytic anemia patients who attended the same ward at the National Bone Marrow Transplantation Center of Tunis and 46 controls from a different hospital. Screening for IgM and IgG anti-B19 antibodies was performed using commercially available enzyme immunoassays and B19 DNA was detected by nested PCR in the overlapping VP1/VP2 region. DNA was sequenced using dideoxy-terminator cycle sequencing technology. Results Anti-parvovirus B19 IgG antibodies were detected in 26 of 46 sickle-cell anemia patients, 18 of 46 β-thalassemia and 7 of 46 controls. Anti-parvovirus B19 IgM antibodies were detected only in 4 of the sickle-cell anemia patients: two siblings and two unrelated who presented with acute erythroblastopenia at the time of blood collection for this study and had no history of past transfusion. B19 DNA was detected only in sera of these four patients and the corresponding 288 bp nested DNA amplicons were sequenced. The sequences obtained were all identical and phylogenetic analysis showed that they belonged to a new B19 virus strain of Genotype1. Conclusion A new parvovirus B19 strain of genotype1 was detected in four Tunisian patients with sickle-cell anemia. Virus transmission appeared to be nosocomial and resulted in acute erythroblastopenia in the four

  7. Autoimmune liver diseases

    Institute of Scientific and Technical Information of China (English)

    Pietro Invernizzi; Ian R Mackay

    2008-01-01

    The liver was one of the earliest recognized sites among autoimmune diseases yet autoimmune hepatitis,primary biliary cirrhosis,primary sclerosing cholangitis,and their overlap forms,are still problematic in diagnosis and causation.The contributions herein comprise 'pairs of articles' on clinical characteristics,and concepts of etiopathogenesis,for each of the above diseases,together with childhood autoimmune liver disease,overlaps,interpretations of diagnostic serology,and liver transplantation.This issue is timely,since we are witnessing an ever increasing applicability of immunology to a wide variety of chronic diseases,hepatic and non-hepatic,in both developed and developing countries.The 11 invited expert review articles capture the changing features over recent years of the autoimmune liver diseases,the underlying immunomolecular mechanisms of development,the potent albeit still unexplained genetic influences,the expanding repertoire of immunoserological diagnostic markers,and the increasingly effective therapeutic possibilities.

  8. Psychoneuroimmunology of autoimmune disorders.

    Science.gov (United States)

    Rogers, M P; Fozdar, M

    1996-01-01

    The interactions between the immune system and psychological states are both intricate and intriguing. Research at a molecular level has thrown considerable light on the previously ill-defined area of psychoneuroimmunology. In this report, we explore the psychoneuroimmunology of autoimmune disorders, particularly rheumatoid arthritis and lupus erythematosus. Animal models of these diseases have provided a particularly useful window on complex psychoneuroimmunological interactions. Observations about the effect of stress on the onset and course of autoimmune disorders has added to our understanding of psychoneuroimmunological interactions. These interactions are bi-directional, as reflected in the autoimmune-mediated neuropsychiatric manifestations of systemic lupus. Exploring the role of various neurotransmitters and neuromodulators in the stress response may have important therapeutic implications for autoimmune disorders.

  9. Anemia of Chronic Liver Diseases

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hyun Chung; Lee, Jhung Sang; Koh, Chang Soon; Lee, Mun Ho [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1971-09-15

    The pathogenetic mechanisms of anemia in patients with chronic liver disease were observed. Seventeen patients with moderate to advanced hepatic diseases were studied by various methods. Only patients without previous blood loss were included : 14 had cirrhosis, 2 had active chronic hepatitis, and one had inferior vena cava obstruction with associated liver cirrhosis. The followings were the results: 1. The anemia based on red blood cell count, Hb., and Ht. was found in 76.5-78.6% of the patients. 2. Red cell indices indicated that normo-macrocytic and normochromic anemia was present is the majority of the patients. 3. No evidence of megaloblastic anemia was found on the basis of the morphological examinations. 4. Serum iron, TIBC, % saturation and iron content in the bone marrow indicated that iron deficiency anemia was present in about half of the patients. 5. In the view of the erythrocyte dynamics, primary increase in the red cell destruction was ascribed to the cause of the anemia. 6. Decrease in the red cell survival time was not correlated with MCV, % saturation and S.L. ratio. Also, hemoglobin level was not correlated with MCV, % saturation and T{sub 50} Cr. Therefore, multiple causes may be involved in the pathogenesis of the anemia. 7. Anemia as determined by the red cell volume was found in only 60% of the patients. It may be possible that hemodilutional anemia is present.

  10. Iron Deficiency Anemia in Pregnancy.

    Science.gov (United States)

    Breymann, Christian

    2015-10-01

    Anemia is a common problem in obstetrics and perinatal care. Any hemoglobin below 10.5 g/dL can be regarded as true anemia regardless of gestational age. Reasons for anemia in pregnancy are mainly nutritional deficiencies, parasitic and bacterial diseases, and inborn red blood cell disorders such as thalassemias. The main cause of anemia in obstetrics is iron deficiency, which has a worldwide prevalence between estimated 20%-80% and consists of a primarily female population. Stages of iron deficiency are depletion of iron stores, iron-deficient erythropoiesis without anemia, and iron deficiency anemia, the most pronounced form of iron deficiency. Pregnancy anemia can be aggravated by various conditions such as uterine or placental bleedings, gastrointestinal bleedings, and peripartum blood loss. In addition to the general consequences of anemia, there are specific risks during pregnancy for the mother and the fetus such as intrauterine growth retardation, prematurity, feto-placental miss ratio, and higher risk for peripartum blood transfusion. Besides the importance of prophylaxis of iron deficiency, the main therapy options for the treatment of pregnancy anemia are oral iron and intravenous iron preparations.

  11. Avoiding Anemia: Boost Your Red Blood Cells

    Science.gov (United States)

    ... link, please review our exit disclaimer . Subscribe Avoiding Anemia Boost Your Red Blood Cells If you’re ... and sluggish, you might have a condition called anemia. Anemia is a common blood disorder that many ...

  12. Genetics Home Reference: Diamond-Blackfan anemia

    Science.gov (United States)

    ... Home Health Conditions Diamond-Blackfan anemia Diamond-Blackfan anemia Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Diamond-Blackfan anemia is a disorder of the bone marrow . The ...

  13. Special Issues for People with Aplastic Anemia

    Science.gov (United States)

    ... Menu Donate Special Issues for People with Aplastic Anemia Because you have aplastic anemia , everyday events can ... bleeding, such as contact sports. Pregnancy and Aplastic Anemia Pregnancy is possible for women who have been ...

  14. Anemia of Inflammation and Chronic Disease

    Science.gov (United States)

    ... Disease Organizations (PDF, 270 KB). Alternate Language URL Anemia of Inflammation and Chronic Disease Page Content On ... Nutrition Points to Remember Clinical Trials What is anemia? Anemia is a condition in which a person ...

  15. Iron-Deficiency Anemia (For Parents)

    Science.gov (United States)

    ... Things to Know About Zika & Pregnancy Iron-Deficiency Anemia KidsHealth > For Parents > Iron-Deficiency Anemia Print A ... common nutritional deficiency in children. About Iron-Deficiency Anemia Every red blood cell in the body contains ...

  16. Etiopathogenesis of insulin autoimmunity.

    OpenAIRE

    Åke Lenmark; Moustakas, Antonis K; Papadopoulos, George K; Norio Kanatsuna

    2012-01-01

    Autoimmunity against pancreatic islet beta cells is strongly associated with proinsulin, insulin, or both. The insulin autoreactivity is particularly pronounced in children with young age at onset of type 1 diabetes. Possible mechanisms for (pro)insulin autoimmunity may involve beta-cell destruction resulting in proinsulin peptide presentation on HLA-DR-DQ Class II molecules in pancreatic draining lymphnodes. Recent data on proinsulin peptide binding to type 1 diabetes-associated HLA-DQ2 and ...

  17. Silica, Silicosis and Autoimmunity.

    Directory of Open Access Journals (Sweden)

    Kenneth Michael Pollard

    2016-03-01

    Full Text Available Inhalation of dust containing crystalline silica is associated with a number of acute and chronic diseases including systemic autoimmune diseases. Evidence for the link with autoimmune disease comes from epidemiological studies linking occupational exposure to crystalline silica dust with the systemic autoimmune diseases SLE, SSc and RA. Although little is known regarding the mechanism by which silica exposure leads to systemic autoimmune disease, there is a voluminous literature on silica exposure and silicosis that may help identify immune processes that precede development of autoimmunity. The pathophysiology of silicosis consists of deposition of silica particles in the alveoli of the lung. Ingestion of these particles by macrophages initiates an inflammatory response which stimulates fibroblasts to proliferate and produce collagen. Silica particles are encased by collagen leading to fibrosis and the nodular lesions characteristic of the disease. The steps in the development of silicosis, including acute and chronic inflammation and fibrosis, have different molecular and cellular requirements suggesting that silica-induced inflammation and fibrosis may be mechanistically separate. Significantly, it is unclear whether silica-induced inflammation and fibrosis contribute similarly to the development of autoimmunity. Nonetheless, the findings from human and animal model studies are consistent with an autoimmune pathogenesis that begins with activation of the innate immune system leading to proinflammatory cytokine production, pulmonary inflammation leading to activation of adaptive immunity, breaking of tolerance, autoantibodies and tissue damage. The variable frequency of these immunological features following silica exposure suggests substantial genetic involvement and gene/environment interaction in silica-induced autoimmunity. However numerous questions remain unanswered.

  18. Vaccines and autoimmunity.

    Science.gov (United States)

    De Martino, M; Chiappini, E; Galli, L

    2013-01-01

    Vaccines have eradicated or controlled many infectious diseases, saving each year millions of lives and quality of life of many other millions of people. In spite of the success of vaccines over the last two centuries, parents (and also some health care workers) gloss over the devastating consequences of diseases, which are now avoided thanks to vaccines, and direct their attention to possible negative effects of immunization. Three immunological objections are raised: vaccines cause antigenic overload, natural immunity is safer and better than vaccine-induced immunity, and vaccines induce autoimmunity. The last point is examined in this review. Theoretically, vaccines could trigger autoimmunity by means of cytokine production, anti-idiotypic network, expression of human histocompatibility leukocyte antigens, modification of surface antigens and induction of novel antigens, molecular mimicry, bystander activation, epitope spreading, and polyclonal activation of B cells. There is strong evidence that none of these mechanisms is really effective in causing autoimmune diseases. Vaccines are not a source of autoimmune diseases. By contrast, absolute evidence exists that infectious agents can trigger autoimmune mechanisms and that they do cause autoimmune diseases.

  19. Covalent binding and hemolytic activity of complement proteins.

    OpenAIRE

    Law, S K; Lichtenberg, N A; Levine, R P

    1980-01-01

    We report the inactivation of the third component of complement (C3) by hydroxylamine. C3 hemolytic and covalent binding activities decline with identical kinetics, demonstrating a direct correlation between the two activities. We conclude that covalent, surface-bound C3b is hemolytically active. The inactivation of C3 is first order with respect to hydroxylamine. We also studied C3 inactivation with [14C]methylamine. The inactivation corresponds quantitatively with the labeling of C3 in the ...

  20. Pernicious anemia presenting as catatonia: correlating vitamin B12 levels and catatonic symptoms.

    Science.gov (United States)

    Bram, Damien; Bubrovszky, Maxime; Durand, Jean-Paul; Lefevre, Guillaume; Morell-Dubois, Sandrine; Vaiva, Guillaume

    2015-01-01

    Pernicious anemia has been associated with various psychiatric manifestations, such as depression, mania and psychosis. Psychiatric symptoms can sometimes occur without hematological and neurological abnormalities and can be prodromal of vitamin B12 deficiency. We report a case of autoimmune B12 deficiency presenting as catatonia without signs of anemia or macrocytosis, in which a correlation was found between the patient's B12 blood levels and catatonic symptoms over time. This catatonic episode was successfully treated with only lorazepam and adequate doses of cyanocobalamin.

  1. Causes of macrocytic anemia among 628 patients: mean corpuscular volumes of 114 and 130 fL as critical markers for categorization.

    Science.gov (United States)

    Takahashi, Natsuko; Kameoka, Junichi; Takahashi, Naoto; Tamai, Yoshiko; Murai, Kazunori; Honma, Riko; Noji, Hideyoshi; Yokoyama, Hisayuki; Tomiya, Yasuo; Kato, Yuichi; Ishizawa, Kenichi; Ito, Shigeki; Ishida, Yoji; Sawada, Kenichi; Harigae, Hideo

    2016-09-01

    There have been no studies on the distribution of causes of macrocytic anemia with respect to mean corpuscular volume (MCV) cutoff values. We retrospectively investigated the causes of macrocytic anemia (MCV ≥100 fL) among 628 patients who visited the outpatient hematology clinic in Tohoku University Hospital. To ensure data validity, we also analyzed data from 307 patients in eight other hospitals in the Tohoku district. The leading causes of macrocytic anemia (number of patients, %) were myelodysplastic syndromes (121, 19.3 %), suspected bone marrow failure syndromes (BMF; 74, 11.8 %), aplastic anemia (51, 8.1 %), plasma cell dyscrasia (45, 7.2 %), and vitamin B12 deficiency (40, 6.4 %) in Tohoku University Hospital. We made three primary findings as follows. First, the most common cause of macrocytic anemia is BMF. Second, lymphoid and solid malignancies are also common causes of macrocytosis. Third, macrocytic anemia may be classified into three groups: Group 1 (megaloblastic anemia and medications), which can exceed MCV 130 fL; Group 2 (alcoholism/liver disease, BMF, myeloid malignancy, and hemolytic anemia), which can exceed MCV 114 fL; and Group 3 (lymphoid malignancy, chronic renal failure, hypothyroidism, and solid tumors), which does not exceed MCV 114 fL. These conclusions were supported by the results from eight other hospitals. PMID:27352093

  2. The Effect of Human Recombinant Erythropoietin on Prevention of Anemia of Prematurity

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    K Hajian

    2007-06-01

    Full Text Available Objective: Premature infants often develop significant anemia that requires blood transfusion, this carries significant risks. This study was carried out to determine the effect of recombinant human erythropoietin (r-HuEPO on prevention of anemia of prematurity. Material & Methods: From April 2001 to March 2002, 24 neonates in  newborn services at Amirkola childrens hospital randomly were assigned to erythropoietin group and control (no treatment group. Inclusion criteria were birth weight of ≤1750 grams and gestational age ≤34 weeks. Exclusion criteria were problems of hemolytic anemia, congenital infections, congenital malformations, severe asphyxia, intraventricular hemorrhage (grade III and IV, need for exchange transfusion and death during the first week of life. Erythropoietin group received r-HuEPO400 unit/kg/dose subcutaneously three times a week plus 4 mg/kg/day iron orally. White blood cell, hemoglobin (Hgb, hematocrit (Hct, platelet and reticulocyte count were obtained every 2 weeks until the 42nd day of life. Anemia was defined as Hgb≤8gr/dl and Hct≤24%. Student t test and Fisher exact were used to evaluate differences between the two groups.Findings: Hemoglobin and hematocrit values were significantly higher in erythropoietin group than the control group after the 14th day of the study (P<0.04 and this difference was getting higher until the end of the trial (P<0.001. Five neonates developed anemia; all of them were from control group. One of these neonates required transfusion. None of the erythropoietin group newborns developed anemia.Conclusion: The results of this study confirm the efficacy of recombinant human erythropoietin in the prevention of anemia of prematurity.

  3. ANEMIA HEMOLÍTICA EM CÃES: RELATO DE CASO

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    Silvia Franco Andrade

    2010-06-01

    Full Text Available Imunne-mediated hemolytic anemia (IMHA is defined as a reduction in the number of red cells due to the destruction of them by the immune system due to the presence of immunoglobulins (IgM and IgG in theerythrocyte membrane. Were attempted two cases of this disease, which were diagnosed by the sum clinical history, examination results and positive development through effective clinical treatment. This report aims to briefly review the issue and contribute with more data about this disease.

  4. Deep vein thrombosis, an unreported first manifestation of polyglandular autoimmune syndrome type III

    Science.gov (United States)

    Hogan, P; Oliver, T

    2016-01-01

    Summary A 71-year-old woman with severe right lower leg pain, edema and erythema was presented to the Emergency Department and was found to have an extensive deep vein thrombosis (DVT) confirmed by ultrasound. She underwent an extensive evaluation due to her prior history of malignancy and new hypercoagulable state, but no evidence of recurrent disease was detected. Further investigation revealed pernicious anemia (PA), confirmed by the presence of a macrocytic anemia (MCV=115.8fL/red cell, Hgb=9.0g/dL), decreased serum B12 levels (56pg/mL), with resultant increased methylmalonic acid (5303nmol/L) and hyperhomocysteinemia (131μmol/L), the presumed etiology of the DVT. The patient also suffered from autoimmune thyroid disease (AITD), and both antithyroglobulin and anti-intrinsic factor antibodies were detected. She responded briskly to anticoagulation with heparin and coumadin and treatment of PA with intramuscular vitamin B12 injections. Our case suggests that a DVT secondary to hyperhomocystenemia may represent the first sign of polyglandular autoimmune syndrome III-B (PAS III-B), defined as the coexistent autoimmune conditions AITD and PA. It is important to recognize this clinical entity, as patients may not only require acute treatment with vitamin B12 supplementation and prolonged anticoagulation, as in this patient, but may also harbor other autoimmune diseases. Learning points A DVT can be the first physical manifestation of a polyglandular autoimmune syndrome. Hyperhomocysteinemia secondary to pernicious anemia should be considered as an etiology of an unprovoked DVT in a euthyroid patient with autoimmune thyroid disease. Patients with DVT secondary to hyperhomocysteinemia should undergo screening for the presence of co-existent autoimmune diseases in addition to treatment with B12 supplementation and anticoagulation to prevent recurrent thromboembolism. PMID:27482386

  5. Sickle cell anemia Review

    OpenAIRE

    Antmen, Bülent

    2009-01-01

    Sickle hemoglobin HbS so called because of the sickle shape it imparts to deoxynated red cells is responsible for a wide spectrum of disorders that vary with respect to degree of anemia frequency of crises extent of organ injury and duration of survival The sickle mutation substitutes thymine for adenine in the sixth codon of the b gene GAGÆGTG thereby encoding valine instead of glutamine in the sixth position of the ß chain This ostensibly minor change in structure is responsible for profoun...

  6. Roles of the Valine Clusters in Domain 3 of the Hemolytic Lectin CEL-III in Its Oligomerization and Hemolytic Abilities

    OpenAIRE

    Hisamatsu, Keigo; Unno, Hideaki; Goda, Shuichiro; Hatakeyama, Tomomitsu

    2009-01-01

    The hemolytic lectin CEL-III and its site-directed mutants were expressed in Escherichia coli cells. Replacement of the valine clusters in domain 3 with alanine residues led to increased self-oligomerization in solution and higher hemolytic activity. The results suggest the involvement of these valine clusters in CEL-III oligomerization and hemolytic activity.

  7. Manejo, prevención y control de la anemia perniciosa Management, prevention and control of pernicious anemia

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    R. De Paz

    2005-12-01

    Full Text Available La anemia perniciosa es la causa más frecuente de anemia megaloblástica en nuestro medio y es consecuencia de una deficiencia de vitamina B12 debido a su vez a la disminución o ausencia de factor intrínseco (FI por atrofia de la mucosa gástrica o por destrucción autoinmune de las células parietales productoras de éste. Ante la existencia de una atrofia gástrica intensa, se origina un descenso en la producción de ácido y FI y una posterior alteración en la absorción de vitamina B12. En un 50% de los casos se asocia a anticuerpos anti FI, cuya presencia en otras enfermedades auto-inmunes es excepcional. En pacientes con anemia perniciosa la determinación de anticuerpos anti FI tiene una alta especificidad (95%, sin embargo, la determinación de anticuerpos anticélulas parietales cuentan con una especificidad baja. El tratamiento de elección es la administración de B12 intramuscularmente. La pauta consiste en administrar 1 mg. de Vitamina B12 diariamente durante una semana, posteriormente semanal durante un mes y después cada 2-3 meses de por vida.Pernicious anemia is the most frequent cause of megaloblastic anemia in our area, and it is the result of a vitamin B12 deficiency due, itself, to the de-crease or absence of intrinsic factor (IF because of gastric mucosa atrophy or autoimmune destruction of IF-producing parietal cells. With the existence of a severe gastric atrophy, there is a decrease in acid and IF production and a further change in vitamin B12 absorption. Fifty percent of the cases are associated to anti-IF antibodies, which presence in other autoimmune diseases is exceptional. In patients with pernicious anemia, measurement of anti-IF antibodies has high specificity (95%; however, measurement of anti-parietal cells antibodies has low specificity. The first-choice treatment is adminis-tration of vitamin B12 intramuscularly. The regimen is the administration of 1 mg of vitamin B12 daily for one week, weekly thereafter

  8. Autoimmune thyroid disease and other non-endocrine autoimmune diseases

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    Todorović-Đilas Ljiljana

    2011-01-01

    Full Text Available Introduction, Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a single patient or in the same family. Numerous autoimmune diseases have been shown to coexist frequently with thyroid autoimmune diseases. Autoimmune thyroid disease and other organ specific non-endocrine autoimmune diseases. This part of the study reviews the prevalence of autoimmune thyroid disease coexisting with: pernicious anaemia, vitiligo, celiac disease, autoimmune liver disease, miastenia gravis, alopecia areata and sclerosis multiplex, and several recommendations for screening have been given. Autoimmune thyroid disease and other organ non-specific non-endocrine autoimmune diseases. Special attention is given to the correlation between autoimmune thyroid disease and rheumatoid arthritis, systemic lupus erythematosus, syndrome Sjögren, systemic sclerosis and mixed connective tissue disease. Conclusions. Screening for autoimmune thyroid diseases should be recommended in everyday clinical practice, in patients with primary organ-specific or organ non-specific autoimmune disease. Other­wise, in patients with primary thyroid autoimmune disease, there is no good reason of seeking for all other autoimmune diseases, although these patients have a greater risk of developing other autoimmune disease. Economic aspects of medicine require further analyzing of these data, from cost/benefit point of view to justified either mandatory screening or medical practitioner judgment.

  9. Autoimmunity and Asbestos Exposure

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    Jean C. Pfau

    2014-01-01

    Full Text Available Despite a body of evidence supporting an association between asbestos exposure and autoantibodies indicative of systemic autoimmunity, such as antinuclear antibodies (ANA, a strong epidemiological link has never been made to specific autoimmune diseases. This is in contrast with another silicate dust, crystalline silica, for which there is considerable evidence linking exposure to diseases such as systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis. Instead, the asbestos literature is heavily focused on cancer, including mesothelioma and pulmonary carcinoma. Possible contributing factors to the absence of a stronger epidemiological association between asbestos and autoimmune disease include (a a lack of statistical power due to relatively small or diffuse exposure cohorts, (b exposure misclassification, (c latency of clinical disease, (d mild or subclinical entities that remain undetected or masked by other pathologies, or (e effects that are specific to certain fiber types, so that analyses on mixed exposures do not reach statistical significance. This review summarizes epidemiological, animal model, and in vitro data related to asbestos exposures and autoimmunity. These combined data help build toward a better understanding of the fiber-associated factors contributing to immune dysfunction that may raise the risk of autoimmunity and the possible contribution to asbestos-related pulmonary disease.

  10. Vaccines and autoimmunity.

    Science.gov (United States)

    Agmon-Levin, Nancy; Paz, Ziv; Israeli, Eitan; Shoenfeld, Yehuda

    2009-11-01

    Vaccines have been used for over 200 years and are the most effective way of preventing the morbidity and mortality associated with infections. Like other drugs, vaccines can cause adverse events, but unlike conventional medicines, which are prescribed to people who are ill, vaccines are administered to healthy individuals, thus increasing the concern over adverse reactions. Most side effects attributed to vaccines are mild, acute and transient; however, rare reactions such as hypersensitivity, induction of infection, and autoimmunity do occur and can be severe and even fatal. The rarity and subacute presentation of post-vaccination autoimmune phenomena means that ascertaining causality between these events can be difficult. Moreover, the latency period between vaccination and autoimmunity ranges from days to years. In this article, on the basis of published evidence and our own experience, we discuss the various aspects of the causal and temporal interactions between vaccines and autoimmune phenomena, as well as the possible mechanisms by which different components of vaccines might induce autoimmunity.

  11. Complement and autoimmunity.

    Science.gov (United States)

    Ballanti, Eleonora; Perricone, Carlo; Greco, Elisabetta; Ballanti, Marta; Di Muzio, Gioia; Chimenti, Maria Sole; Perricone, Roberto

    2013-07-01

    The complement system is a component of the innate immune system. Its main function was initially believed to be limited to the recognition and elimination of pathogens through direct killing or stimulation of phagocytosis. However, in recent years, the immunoregulatory functions of the complement system were demonstrated and it was determined that the complement proteins play an important role in modulating adaptive immunity and in bridging innate and adaptive responses. When the delicate mechanisms that regulate this sophisticated enzymatic system are unbalanced, the complement system may cause damage, mediating tissue inflammation. Dysregulation of the complement system has been involved in the pathogenesis and clinical manifestations of several autoimmune diseases, such as systemic lupus erythematosus, vasculitides, Sjögren's syndrome, antiphospholipid syndrome, systemic sclerosis, dermatomyositis, and rheumatoid arthritis. Complement deficiencies have been associated with an increased risk to develop autoimmune disorders. Because of its functions, the complement system is an attractive therapeutic target for a wide range of diseases. Up to date, several compounds interfering with the complement cascade have been studied in experimental models for autoimmune diseases. The main therapeutic strategies are inhibition of complement activation components, inhibition of complement receptors, and inhibition of membrane attack complex. At present, none of the available agents was proven to be both safe and effective for treatment of autoimmune diseases in humans. Nonetheless, data from preclinical studies and initial clinical trials suggest that the modulation of the complement system could constitute a viable strategy for the treatment of autoimmune conditions in the decades to come.

  12. Severe hemolytic transfusion reaction due to anti-D in a D+ patient with sickle cell disease.

    Science.gov (United States)

    Ipe, Tina S; Wilkes, Jennifer J; Hartung, Helge D; Westhoff, Connie M; Chou, Stella T; Friedman, David F

    2015-03-01

    A 5-year-old male with sickle cell disease presented with pain, dark urine, and fatigue 10 days after a red blood cell (RBC) transfusion. Laboratory evaluation demonstrated severe anemia, blood type O+, and anti-D in the serum. Anti-D in a D+ patient led to RH genotyping, which revealed homozygosity for RHD*DAU4 that encodes partial D antigen. Anti-D in this patient whose RBCs exclusively express partial D caused a delayed hemolytic transfusion reaction after exposure to D+ RBCs. The finding of anti-D in a D+patient should be investigated by molecular methods to help distinguish an alloantibody from an autoantibody. PMID:25171447

  13. Autoimmunity in 2015.

    Science.gov (United States)

    Selmi, Carlo

    2016-08-01

    Compared to the clear trend observed in previous years, the number of peer-reviewed articles published during 2015 and retrieved using the "autoimmunity" key word declined by 4 %, while remaining 5 % of immunology articles. On the other hand, a more detailed analysis of the published articles in leading immunology and autoimmunity journals revealed exciting scenarios, with fascinating lines of evidence being supported by convincing data and likely followed by rapid translational or clinical developments. As examples, the study of the microbiome, the development of new serum or other tissue biomarkers, and a more solid understanding of disease pathogenesis and tolerance breakdown mechanisms have been central issues in the past year. Furthermore and similar to the oncology field, progress in the understanding of single autoimmune condition is becoming most specific with psoriatic and rheumatoid arthritis being ideal paradigms with treatment options diverging after decades of common therapies, as illustrated by IL17-targeting approaches. The ultimate result of these advances is towards personalized medicine with an ideal approach being tailored on a single patient, based on a finely tuned definition of the immunogenetics, epigenetics, microbiome, and biomarkers. Finally, experimental reports suggest that cancer-associated immune mechanisms or the role of T and B cell subpopulations should be better understood in autoimmune diseases. While we hailed the 2014 literature in the autoimmunity world as part of an annus mirabilis, we should not be mistaken in the strong stimulus of research in autoimmunity represented by the 2015 articles that will be summarized in this article. PMID:27422713

  14. Common mechanisms of autoimmune diseases (the autoimmune tautology).

    Science.gov (United States)

    Anaya, Juan-Manuel

    2012-09-01

    The fact that autoimmune diseases share subphenotypes, physiopathological mechanisms and genetic factors has been called autoimmune tautology, and indicates that they have a common origin. The autoimmune phenotypes vary depending on the target cell and the affected organ, gender, ancestry, trigger factors and age at onset. Ten shared characteristics supporting this logical theory are herein reviewed.

  15. Pseudo-thrombotic thrombocytopenic purpura: A rare presentation of pernicious anemia

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    Ashvin K Tadakamalla

    2011-01-01

    Full Text Available Context: Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of pernicious anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura. Case Report : A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B 12 was found to be low. Treatment with intra-muscular vitamin B 12 led to symptomatic and hematologic improvement. Pernicious anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis. Conclusion : Clinicians must be aware of unusual clinical presentation of vitamin B 12 deficiency with schistocytes as the management is simple and effective.

  16. Epigenomics of autoimmune diseases.

    Science.gov (United States)

    Gupta, Bhawna; Hawkins, R David

    2015-03-01

    Autoimmune diseases are complex disorders of largely unknown etiology. Genetic studies have identified a limited number of causal genes from a marginal number of individuals, and demonstrated a high degree of discordance in monozygotic twins. Studies have begun to reveal epigenetic contributions to these diseases, primarily through the study of DNA methylation, but chromatin and non-coding RNA changes are also emerging. Moving forward an integrative analysis of genomic, transcriptomic and epigenomic data, with the latter two coming from specific cell types, will provide an understanding that has been missed from genetics alone. We provide an overview of the current state of the field and vision for deriving the epigenomics of autoimmunity.

  17. Critical evaluation of peripheral smear in cases of anemia with high mean corpuscular hemoglobin concentration in children: A series of four cases

    OpenAIRE

    Sharma Sunita; Pujani Meenu; Pahuja Sangeeta; Chandra Jagdish; Rath B; Labhchand

    2010-01-01

    Mean corpuscular hemoglobin concentration (MCHC), a parameter that is reported as a part of a standard complete blood count by automated analyzer, is a measure of the concentration of hemoglobin in a given volume of packed red blood cell. Values of MCHC significantly above reference range are not physiologically possible due to limitations on solubility of hemoglobin. The high MCHC can give us a clue to certain type of hemolytic anemia and necessitate critical evaluation of peripheral smear t...

  18. Managing anemia in lymphoma and multiple myeloma

    OpenAIRE

    Gunnar Birgegård

    2008-01-01

    Gunnar BirgegårdDepartment of Haematology, University Hospital, Uppsala, SwedenAbstract: Anemia is common in cancer, and lymphoproliferative disease is no exception. Erythropoiesis-stimulating agents (ESA) have been used for renal anemia since 1986, and considerably later in cancer anemia. The first studies were published around 1993, but the use of ESA did not become common in cancer anemia until in the late 1990s. Cancer anemia is still under-treated. This review gives an overview...

  19. Study of chronic hemolytic anaemia patients in Rio de Janeiro: prevalence of anti-human parvovirus B19 IgG antibodies and the developement aplastic crises

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    SANT'ANNA Anadayr L.M.

    2002-01-01

    Full Text Available The prevalence of anti-human parvovirus B19 IgG antibodies was determined in sera from 165 chronic hemolytic anemia patients, receiving medical care at Instituto Estadual de Hematologia (IEHE, Rio de Janeiro, during the year of 1994. This sample represents around 10% of the chronic hemolytic anemia patients attending at IEHE. Most of these patients (140 have sickle cell disease. Anti-B19 IgG antibodies were detected in 32.1% of patients. No statistically significant difference (p > 0.05 was seen between IgG antibody prevalence in male (27.8% and female (35.5% patients. Anti-B19 IgG antibodies were more frequent in older (37.6% than younger (28.2% than 20 years old patients, although this difference had no statistical significance (p > 0.05. Anti-B19 IgG antibody prevalence showed that 67.9% of patients enrolled in the study were susceptible to B19 acute infection. With the aim to detect acute B19 infection, patients follow up continued until February 1996. During this period four patients presented transient aplastic crisis due to human parvovirus B19 as confirmed by the detection of specific IgM antibodies. All four patients were younger than 20 years old, and 3 were younger than 10 years old. Three of them were sickle cell disease patients. Three of the four acute B19 infection occurred during 1994 springtime.

  20. Autoimmune muscular pathologies.

    Science.gov (United States)

    Dalakas, M C

    2005-05-01

    The T cell-mediated mechanism responsible for Polymyositis and inclusion Body Myositis and the complement-mediated microangiopathy associated with Dermatomyositis are reviewed. The management of autoimmune myopathies with the presently available immunotherapeutic agents as well as new therapies and ongoing trials are discussed.

  1. Autoimmune pancreatitis and cholangitis

    Institute of Scientific and Technical Information of China (English)

    Niraj; Jani; James; Buxbaum

    2015-01-01

    Autoimmune pancreatitis(AIP) is part of a systemic fibrosclerotic process characterized by lymphoplasmacytic infiltrate with immunoglobulin G subtype-4(Ig G4) positive cells. It characteristically presents with biliary obstruction due to mass-like swelling of the pancreas. Frequently AIP is accompanied by extra-pancreaticmanifestations including retroperitoneal fibrosis, thyroid disease, and salivary gland involvement. Auto-antibodies, hypergammaglobulemia, and prompt resolution of pancreatic and extrapancreatic findings with steroids signify its autoimmune nature. Refractory cases are responsive to immunomodulators and rituximab. Involvement of the biliary tree, termed IgG 4 associated cholangiopathy, mimics primary sclerosing cholangitis and is challenging to manage. High IgG 4 levels and swelling of the pancreas with a diminutive pancreatic duct are suggestive of autoimmune pancreatitis. Given similarities in presentation but radical differences in management and outcome, differentiation from pancreatic malignancy is of paramount importance. There is controversy regarding the optimal diagnostic criterion and steroid trials to make the diagnosis. Additionally, the retroperitoneal location of the pancreas and requirement for histologic sampling, makes tissue acquisition challenging. Recently, a second type of autoimmune pancreatitis has been recognized with similar clinical presentation and steroid response though different histology, serologic, and extrapancreatic findings.

  2. Autoimmunity and Turner's syndrome.

    Science.gov (United States)

    Lleo, Ana; Moroni, Luca; Caliari, Lisa; Invernizzi, Pietro

    2012-05-01

    Turner Syndrome (TS) is a common genetic disorder, affecting female individuals, resulting from the partial or complete absence of one sex chromosome, and occurring in approximately 50 per 100,000 liveborn girls. TS is associated with reduced adult height and with gonadal dysgenesis, leading to insufficient circulating levels of female sex steroids and to infertility. Morbidity and mortality are increased in TS but average intellectual performance is within the normal range. TS is closely associated to the presence of autoantibodies and autoimmune diseases (AID), especially autoimmune thyroiditis and inflammatory bowel disease. Despite the fact that the strong association between TS and AID is well known and has been widely studied, the underlying immunopathogenic mechanism remains partially unexplained. Recent studies have displayed how TS patients do not show an excess of immunogenic risk markers. This is evocative for a higher responsibility of X-chromosome abnormalities in the development of AID, and particularly of X-genes involved in immune response. For instance, the long arm of the X chromosome hosts a MHC-locus, so the loss of that region may lead to a deficiency in immune regulation. Currently no firm guidelines for diagnosis exist. In conclusion, TS is a condition associated with a number of autoimmune manifestations. Individuals with TS need life-long medical attention. As a consequence of these findings, early diagnosis and regular screening for potential associated autoimmune conditions are essential in the medical follow-up of TS patients. PMID:22154619

  3. Jaundice

    Science.gov (United States)

    ... Autoimmune hepatitis Bile Biliary atresia Biliary stricture Bilirubin blood test Cholestasis Delta agent (Hepatitis D) Dubin-Johnson syndrome Gallstones Gilbert disease Hemolytic anemia Hepatitis A Hepatitis B Hepatitis C Malaria Newborn jaundice Pancreatic cancer Primary ...

  4. Autoimmune paediatric liver disease

    Institute of Scientific and Technical Information of China (English)

    Giorgina Mieli-Vergani; Diego Vergani

    2008-01-01

    Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC),and de novo AIH after liver transplantation.AIH is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA,type 1) or liver kidney microsomal antibody (LKM1,type 2).There is a female predominance in both.LKM1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity, presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both groups. The most common type of paediatric sclerosing cholangitis is ASC.The clinical,biochemical, immunological,and histological presentation of ASC is often indistinguishable from that of AIH type 1.In both,there are high IgG,non-organ specific autoantibodies,and interface hepatitis.Diagnosis is made by cholangiography.Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters, and decreased inflammatory activity on follow up liver biopsies. However,the cholangiopathy can progress.There may be evolution from AIH to ASC over the years,despite treatment.De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies, hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and collapse.Like classical AIH,it responds to treatment with prednisolone and azathioprine.De novo AIH post liver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection.Whether this condition is a distinct entity or a form of

  5. Group A β-hemolytic streptococcal pharyngotonsillitis outbreak

    Science.gov (United States)

    Culqui, Dante R; Manzanares-Laya, Sandra; Van Der Sluis, Sarah Lafuente; Fanlo, Albert Anton; Comas, Rosa Bartolomé; Rossi, Marcello; Caylá, Joán A

    2014-01-01

    The aim was to describe an outbreak of group A β-hemolytic streptococcal pharyngotonsillitis in health care professionals. This is a cross-sectional descriptive study of 17 clients who dined at the same table in a restaurant in Barcelona in July 2012. The frequency, timing and severity of symptoms were analyzed, as were demographic variables and others concerning the food ingested. The attack rate was 58.8%. Six of the 10 clients were positive for group A β-hemolytic streptococcal. Six of the 13 individuals who handled the food involved in the dinner had symptoms. No association was identified with the food consumed. There is epidemiological evidence of foodborne group A β-hemolytic streptococcal transmission, but respiratory transmission could not be ruled out. PMID:24897054

  6. ABO incompatibility hemolytic disease following exchange transfusion 96 newborn

    Directory of Open Access Journals (Sweden)

    Khatami S.F

    2007-09-01

    Full Text Available Background: ABO incompatibility hemolytic disease of the newborn is a common cause of clinical jaundice and causes two-thirds of the hemolytic disease in newborns. This study was undertaken to determine the frequency of ABO incompatibility hemolytic disease and its complications in newborns undergoing exchange transfusion.Methods: This prospective and descriptive study was performed in jaundiced newborn infants during a three-year period. Inclusion criteria were: maternal blood type O, newborn blood type A or B, rising indirect hyperbilirubinemia in the first two days of life, positive immunohematologic test for newborns and exchange transfusion. Exclusion criteria were: incomplete information, other accompanying diseases that induce hyperbilirubinemia. All newborn infants received phototherapy before and after exchange transfusion. We did not use intravenous immunoglobulin, hemoxygenase inhibitor drugs and blood products before exchange transfusion.Results: Double-volume exchange transfusion via umbilical cord catheter was performed in 96 patients, 19 (20% of whom suffered from ABO incompatibility. Of these 19 newborns, two-thirds (13 were preterm infants. The minimum level of serum bilirubin was 10 mg/dl and the maximum serum bilirubin level was 35 mg/dl. In six patients (32% serum bilirubin levels were >25mg/dl. The most common blood group was type A for newborns. Immunohematologic tests were positive in 84% of the mothers. ABO incompatibility hemolytic disease was the fourth and second most common reasons for blood exchange transfusion in preterm and term infants, respectively. Laboratory complications were more common than clinical complications. The etiology of 48% of the alloimmunization and 42% of the hemolytic disease in these newborns was ABO incompatibility.Conclusions: Mothers with blood group O and newborns with blood group A or B with positive immunohematologic tests in first hours of life are at high risk for hemolytic disease

  7. Comparative study of hemolytic activity of Bordetella species

    Directory of Open Access Journals (Sweden)

    C N Khobragade

    2009-11-01

    Full Text Available Background and objectives: Bordetella species colonize the respiratory tract of mammals and thereby cause the whooping cough. Most of the species produce adenylate cyclase - a toxin ( hemolysin responsible for increasing intracellular cyclic AMP (cAMP levels in mammalian neutrophils and macrophages and as a consequence their phagocytic function get impaired . This study was carried out to isolate species of Bordetella and to study the hemolytic activity of each species on RBCs of sheep, human and poultry at varied culture conditions by altering the temperature, pH and cell age."nMaterials and Methods: Three pathogenic Bordetella species were isolated from fifty suspected whooping cough patients on Bordet-Gengou agar and identified by their biochemical profiles. The hemolytic activity of B. pertussis, B. parapertussis and B. bronchiseptica was investigated in terms of cell bound and cell free hemolysin on human, poultry and sheep RBCs at variable pH, temperature and cell age in Stainer Scholt broth. The hemolysin activity was also determined qualitatively on blood agar containing different blood samples."nResults: All the species revealed optimum hemolytic activity in pH range 7.5-8.0 (in slight alkaline condition, temperature 37°C and cell age up to 20-24 hrs. The cell bound hemolytic activity was found to be maximum than cell free activity and varied with blood samples of different species. B. pertussis showed maximum hemolytic activity on human red blood cells followed by poultry and sheep RBCs. B. parapertussis and B. bronchiseptica showed maximum hemolytic activity on sheep and poultry RBCs respectively."nConclusion: The findings of our study revealed that different determinants are involved in host interactions and virulence of Bordetella species.

  8. Lack of specific association between gastric autoimmunity hallmarks and clinical presentations of atrophic body gastritis

    Institute of Scientific and Technical Information of China (English)

    Bruno Annibale; Edith Lahner; Riccardo Negrini; Flavia Baccini; Cesare Bordi; Bruno Monarca; Gianfranco Delle Fave

    2005-01-01

    AIM: To investigate the possible relationships between gastric autoimmune phenomena and clinical presentations of this disorder, in consecutive atrophic body gastritis patients.METHODS: A total of 140 atrophic body gastritis patients,diagnosed as consecutive outpatients presenting with macrocytic or iron deficiency anemia, or longstanding dyspepsia underwent gastroscopy with antral and body biopsies, assay of intrinsic factor, parietal cells and Helicobacter pylori(H pylori) antibodies. Gastritis was assessed according to Sydney System.RESULTS: Parietal cell antibodies were equally distributed in all clinical presentations, whereas the positivity of intrinsic factor antibodies (49/140, 35%) was significantly higher in pernicious anemia patients (49.2%) than in iron deficiency (21.1%) and dyspeptic patients (27.8%). No specific pattern of autoantibodies was related to the clinical presentations of atrophic body gastritis. A positive correlation was obtained between the body atrophy score and the intrinsic factor antibody levels (r = 0.2216,P = 0.0085). Associated autoimmune diseases were present in 25/140 (17.9%) patients, but the prevalence of autoimmune diseases was comparable, irrespective of the clinical presentations.CONCLUSION: The so-called hallmarks of gastric autoimmunity, particularly in intrinsic factor antibody cannot be usefully employed in defining an autoimmune pattern in the clinical presentations of ABG.

  9. Case report: Severe hemolytic disease of the fetus and newborn due to anti-C+G.

    Science.gov (United States)

    Jernman, Riina; Stefanovic, Vedran; Korhonen, Anu; Haimila, Katri; Sareneva, Inna; Sulin, Kati; Kuosmanen, Malla; Sainio, Susanna

    2015-01-01

    Anti-G is commonly present with anti-D and/or anti-C and can confuse serological investigations. in general, anti-G is not considered a likely cause of severe hemolytic disease of the fetus and newborn (HDFN), but it is important to differentiate it from anti-D in women who should be administered anti-D immunoglobulin prophylaxis. We report one woman with three pregnancies severely affected by anti-C+G requiring intrauterine treatment and a review of the literature. In our case, the identification of the correct antibody was delayed because the differentiation of anti-C+G and anti-D+C was not considered important during pregnancy since the father was D-. In addition, anti-C+G and anti-G titer levels were not found to be reliable as is generally considered in Rh immunization. Severe HDFN occurred at a maternal anti-C+G antibody titer of S and anti-G titer of 1 in comparison with the critical titer level of 16 or more in our laboratory. close collaboration between the immunohematology laboratory and the obstetric unit is essential. In previously affected families, early assessment for fetal anemia is required even when titers are low. PMID:26829179

  10. Hemolytic disease of the fetus and newborn in the molecular era.

    Science.gov (United States)

    Fasano, Ross M

    2016-02-01

    Maternal-fetal red cell antigen incompatibility can lead to alloimmunization, maternal immunoglobulin transplacental transfer, and hemolytic disease of the fetus and newborn (HDFN). The use of routine antenatal anti-D prophylaxis (RAADP) has sharply decreased the incidence of and mortality from HDFN due to RhD allosensitization. The ability to identify pregnancies/fetuses at risk of HDFN has significantly improved due to paternal molecular RHD zygosity testing, and non-invasive fetal molecular diagnostics for detecting putative antigen(s) (notably RhD) in fetuses utilizing cff-DNA in maternal plasma. Fetal RHD genotyping using cff-DNA has become increasingly accurate for fetal RHD detection, prompting some countries to implement targeted RAADP through mass screening programs of RhD-negative pregnant women. Along with middle cerebral artery Doppler ultrasonography for predicting fetal anemia, non-invasive fetal molecular diagnostics have greatly decreased the need for invasive diagnostic procedures in pregnancies at risk for severe HDFN. This review highlights these molecular advancements in HDFN-related prenatal diagnostics. PMID:26589360

  11. Severe form of hemolytic-uremic syndrome with multiple organ failure in a child: a case report [v1; ref status: indexed, http://f1000r.es/24q

    Directory of Open Access Journals (Sweden)

    Dino Mijatovic

    2014-03-01

    Full Text Available Introduction: Hemolytic-uremic syndrome (HUS is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS, toxic megacolon with ileus, pancreatitis, central nervous system (CNS disorders and multiple organ failure (MOF. Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. In the next week of, what initially appeared as typical HUS, she developed MOF, including ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis. Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.

  12. Sexuality and sickle cell anemia

    OpenAIRE

    Viviane de Almeida Côbo; Cibele Alves Chapadeiro; João Batista Ribeiro; Helio Moraes-Souza; Paulo Roberto Juliano Martins

    2013-01-01

    BACKGROUND: Sickle cell disease, the most common hereditary blood disease in the world, is the result of an atypical hemoglobin called S (Hb S) which, when homozygous (Hb SS) is the cause of sickle cell anemia. Changes of puberty, correlated with a delayed growth spurt, begin late in both male and female sickle cell anemia individuals with repercussions on sexuality and reproduction. The objectives of this exploratory and descriptive study were to characterize the development of sexuality in ...

  13. [Iron-refractory iron deficiency anemia].

    Science.gov (United States)

    Kawabata, Hiroshi

    2016-02-01

    The major causes of iron deficiency anemia (IDA) include iron loss due to bleeding, increased iron requirements, and decreased iron absorption by the intestine. The most common cause of IDA in Japanese women is iron loss during menstruation. Autoimmune atrophic gastritis and Helicobacter pylori infection can also cause IDA by reducing intestinal iron absorption. In addition to these common etiologies, germline mutations of TMPRSS6 can cause iron-refractory IDA (IRIDA). TMPRSS6 encodes matriptase-2, a membrane-bound serine protease primarily expressed in the liver. Functional loss of matriptase-2 due to homozygous mutations results in an increase in the expression of hepcidin, which is the key regulator of systemic iron homeostasis. The serum hepcidin increase in turn leads to a decrease in iron supply from the intestine and macrophages to erythropoietic cells. IRIDA is microcytic and hypochromic, but decreased serum ferritin is not observed as in IDA. IRIDA is refractory to oral iron supplementation, but does respond to intravenous iron supplementation to some extent. Because genetic testing is required for the diagnoses of IRIDA, a considerable number of cases may go undiagnosed and may thus be overlooked. PMID:26935626

  14. [Iron-refractory iron deficiency anemia].

    Science.gov (United States)

    Kawabata, Hiroshi

    2016-02-01

    The major causes of iron deficiency anemia (IDA) include iron loss due to bleeding, increased iron requirements, and decreased iron absorption by the intestine. The most common cause of IDA in Japanese women is iron loss during menstruation. Autoimmune atrophic gastritis and Helicobacter pylori infection can also cause IDA by reducing intestinal iron absorption. In addition to these common etiologies, germline mutations of TMPRSS6 can cause iron-refractory IDA (IRIDA). TMPRSS6 encodes matriptase-2, a membrane-bound serine protease primarily expressed in the liver. Functional loss of matriptase-2 due to homozygous mutations results in an increase in the expression of hepcidin, which is the key regulator of systemic iron homeostasis. The serum hepcidin increase in turn leads to a decrease in iron supply from the intestine and macrophages to erythropoietic cells. IRIDA is microcytic and hypochromic, but decreased serum ferritin is not observed as in IDA. IRIDA is refractory to oral iron supplementation, but does respond to intravenous iron supplementation to some extent. Because genetic testing is required for the diagnoses of IRIDA, a considerable number of cases may go undiagnosed and may thus be overlooked.

  15. Mieloma Múltiplo e anemia Multiple Myeloma and anemia

    Directory of Open Access Journals (Sweden)

    Rodolfo D. Cançado

    2007-03-01

    Full Text Available Anemia é uma complicação comum em pacientes com mieloma múltiplo (MM e ocorre em mais de 2/3 dos pacientes. Anemia de doença crônica, deficiência de eritropoetina (EPO devido à insuficiência renal e efeito mielossupressivo da quimioterapia são os principais mecanismos patofisiológicos que contribuem para o desenvolvimento de anemia no MM. Nos pacientes que obtêm remissão completa com tratamento quimioterápico, anemia usualmente se normaliza. Nos pacientes que não respondem ou apresentam recaída do mieloma, anemia freqüentemente persiste. As opções de tratamento dos pacientes anêmicos com MM incluem transfusões de hemácias e EPO recombinante humana. Essa proteína é biologicamente equivalente à EPO endógena e sua administração promove aumento dos valores de hemoglobina por tempo mais prolongado sem os riscos das transfusões de sangue. Vários estudos têm relatado melhora significante da eritropoese, redução da necessidade transfusional e melhora da qualidade de vida com o uso da EPO como tratamento a longo prazo da anemia associada ao mieloma. Nesse artigo, propomos o tratamento da anemia do MM baseado nas recomendações propostas pela Sociedade Americana de Hematologia (ASH em conjunto com a Sociedade Americana de Oncologia Clínica (ASCO, pela Organização Européia para Pesquisa e Tratamento do Câncer (EORTC, pelo IMF (Internacional Myeloma Foundation e pelo NCCN (National Comprehensive Cancer Network.Anemia is a common complication in patients with multiple myeloma (MM occurring in more than two thirds of all patients. Anemia of chronic diseases, erythropoietin (EPO deficiency due to renal impairment and the myelosuppressive effect of chemotherapy are the most important pathophysiological mechanisms contributing to the development of anemia in MM. In patients who achieve complete remission after chemotherapy, anemia usually normalizes. Non-responders and relapsing myeloma patients often continue to suffer

  16. [Severe hemolytic disease of the newborn as a result of late and undiagnosed alloimmunization--case report].

    Science.gov (United States)

    Drozdowska-Szymczak, Agnieszka; Czaplińska, Natalia; Borek-Dziecioł, Beata; Kociszewska-Najman, Bozena; Bartkowiak, Robert; Wielgoś, Mirosław

    2014-03-01

    We report a case of a hemolytic disease in a newborn from the first pregnancy due to anti-D antibodies. The maternal blood group was A Rhesus negative. She had an antibody screening test twice during the pregnancy (in the second trimester) and it was negative. The pregnancy was uneventful, without any invasive procedures and bleeding. The infant was born at 39 weeks of gestation in good overall condition. After the delivery the blood group of the neonate was indicated - A Rhesus positive, BOC positive. Anti-D antibodies were detected in maternal blood. Neonatal blood tests revealed severe anemia (hemoglobin level: 6.0g/dl, hematocrit: 22.2%, erythrocytes: 2.01T/L). During the first day of neonatal life, the newborn received two transfusions of red blood cells. Bilirubin level and rate of rise were not recommendation enough for exchange transfusion. The newborn was treated with continuous phototherapy since the delivery The perinatal period was complicated with intrauterine infection and respiratory failure. Hematopoietic vitamins and iron supplementation was initiated in the second week of neonatal life due to persistent anemia. The child remained under medical care of a hematologic clinic and received human recombinant erythropoietin treatment. PMID:24783436

  17. Update on autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    Andreas Teufel; Peter R Galle; Stephan Kanzler

    2009-01-01

    Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology that occurs in children and adults of all ages. Characteristics are its autoimmune features, hyperglobulinemia (IgG), and the presence of circulating autoantibodies, as well as a response to immunosuppressant drugs. Current treatment consists of prednisone and azathioprine and in most patients this disease has become very treatable. Over the past 2 years, a couple of new insights into the genetic aspects, clinical course and treatment of AIH have been reported, which will be the focus of this review. In particular, we concentrate on genome-wide microsatellite analysis, a novel mouse model of AIH, the evaluation of a large AIH cohort for overlap syndromes,suggested novel criteria for the diagnosis of AIH, and the latest studies on treatment of AIH with budenoside and mycophenolate mofetil.

  18. [Diagnostics of autoimmune diseases].

    Science.gov (United States)

    Beleznay, Zsuzsanna; Regenass, Stephan

    2008-09-01

    Autoantibodies play a key role in diagnostic laboratories as markers of autoimmune diseases. In addition to their role as markers they mediate diverse effects in vivo. Autoantibodies with protective effect have been described. Natural protective IgM autoantibodies against tumour-antigens of malignant cells or their precursors may contribute to increased survival rates of carcinoma patients. In a mouse model of systemic lupus erythematosus it has been shown that anti-dsDNA IgM autoantibodies protect from glomerular damage. In contrast, a direct pathogenic role of autoantibodies has been well established e.g. in myasthenia gravis or in Goodpasture syndrome. Similarly autoantibodies against SSA Ro52 are detrimental in neonatal lupus erythematosus with congenital heart block. Moreover, putatively protective autoantibodies may become pathogenic during the course of the disease such as the onconeuronal autoantibodies whose pathogenicity depends on their compartmentalisation. In patients with paraneoplastic syndromes tumour cells express proteins that are also naturally present in the brain. Anti-tumour autoantibodies which temporarily suppress tumour growth can provoke an autoimmune attack on neurons once having crossed the blood-brain barrier and cause specific neurological symptoms. Only a restricted number of autoantibodies are useful follow-up markers for the effectiveness of treatment in autoimmune diseases. Certain autoantibodies hold prognostic value and appear years or even decades before the diagnosis of disease such as the antimitochondrial antibodies in primary biliary cirrhosis or anti-citrullinated protein (CCP)-antibodies in rheumatoid arthritis. It is crucial to know whether the autoantibodies in question recognise linear or conformational epitopes in order to choose the appropriate detection methods. Indirect immunofluorescence microscopy remains a very useful tool for confirmation of results of commercially available immunoassays and for detection of

  19. Cationic amphiphilic non-hemolytic polyacrylates with superior antibacterial activity.

    Science.gov (United States)

    Punia, Ashish; He, Edward; Lee, Kevin; Banerjee, Probal; Yang, Nan-Loh

    2014-07-01

    Acrylic copolymers with appropriate compositions of counits having cationic charge with 2-carbon and 6-carbon spacer arms can show superior antibacterial activities with concomitant very low hemolytic effect. These amphiphilic copolymers represent one of the most promising synthetic polymer antibacterial systems reported. PMID:24854366

  20. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome.

    NARCIS (Netherlands)

    Legendre, C.M.; Licht, C.; Muus, P.; Greenbaum, L.A.; Babu, S.; Bedrosian, C.; Bingham, C.; Cohen, D.J.; Delmas, Y.; Douglas, K.; Eitner, F.; Feldkamp, T.; Fouque, D.; Furman, R.R.; Gaber, O.; Herthelius, M.; Hourmant, M.; Karpman, D.; Lebranchu, Y.; Mariat, C.; Menne, J.; Moulin, B.; Nurnberger, J.; Ogawa, M.; Remuzzi, G.; Richard, T.; Sberro-Soussan, R.; Severino, B.; Sheerin, N.S.; Trivelli, A.; Zimmerhackl, L.B.; Goodship, T.; Loirat, C.

    2013-01-01

    BACKGROUND: Atypical hemolytic-uremic syndrome is a genetic, life-threatening, chronic disease of complement-mediated thrombotic microangiopathy. Plasma exchange or infusion may transiently maintain normal levels of hematologic measures but does not treat the underlying systemic disease. METHODS: We

  1. Autoimmune Progesterone Anaphylaxis

    Directory of Open Access Journals (Sweden)

    Mohammad Hassan Bemanian

    2007-06-01

    Full Text Available Progesterone induced dermatitis is a rare disorder. It typically occurs in females due to anautoimmune phenomenon to endogenous progesterone production, but can also be caused byexogenous intake of a synthetic progestin. Here in, we present a case of autoimmune progesterone anaphylaxis (AIPA observed in an adolescent female.The patient is an 18-year-old Caucasian female with no significant past medical history and noprior exogenous hormone use, who presented to her primary care physician complaining of cyclic skin eruptions with dyspnea, cough and respiratory distress. She noted that her symptoms occurred monthly, just prior to her menses. An intradermal skin test using 0.1 cml of progesterone was performed. The patient developed a 15mm wheal after 15 minutes, confirming the diagnosis of AIPA.The patient was started on a continuous regimen of an oral conjugated estrogen (0.625mg. The skin eruptions and respiratory symptoms have not returned since the initiation of this therapy.Autoimmune progesterone dermatitis manifests via the occurrence of cyclic skin eruptions.Women with the disorder commonly present with dermatologic lesions in the luteal phase of themenstrual cycle, if there are any other organ involvement in addition to skin (e.g. lung, GI thereaction should be called as autoimmune progesterone anaphylaxis. Diagnosis of AIPA is confirmed by performing a skin allergen test using progesterone.

  2. Etiopathogenesis of Insulin Autoimmunity

    Directory of Open Access Journals (Sweden)

    Norio Kanatsuna

    2012-01-01

    Full Text Available Autoimmunity against pancreatic islet beta cells is strongly associated with proinsulin, insulin, or both. The insulin autoreactivity is particularly pronounced in children with young age at onset of type 1 diabetes. Possible mechanisms for (proinsulin autoimmunity may involve beta-cell destruction resulting in proinsulin peptide presentation on HLA-DR-DQ Class II molecules in pancreatic draining lymphnodes. Recent data on proinsulin peptide binding to type 1 diabetes-associated HLA-DQ2 and -DQ8 is reviewed and illustrated by molecular modeling. The importance of the cellular immune reaction involving cytotoxic CD8-positive T cells to kill beta cells through Class I MHC is discussed along with speculations of the possible role of B lymphocytes in presenting the proinsulin autoantigen over and over again through insulin-carrying insulin autoantibodies. In contrast to autoantibodies against other islet autoantigens such as GAD65, IA-2, and ZnT8 transporters, it has not been possible yet to standardize the insulin autoantibody test. As islet autoantibodies predict type 1 diabetes, it is imperative to clarify the mechanisms of insulin autoimmunity.

  3. Genetics Home Reference: iron-refractory iron deficiency anemia

    Science.gov (United States)

    ... refractory iron deficiency anemia iron-refractory iron deficiency anemia Enable Javascript to view the expand/collapse boxes. ... All Close All Description Iron-refractory iron deficiency anemia is one of many types of anemia , which ...

  4. What Are the Signs and Symptoms of Anemia?

    Science.gov (United States)

    ... Twitter. What Are the Signs and Symptoms of Anemia? The most common symptom of anemia is fatigue ( ... mild symptoms or none at all. Complications of Anemia Some people who have anemia may have arrhythmias ( ...

  5. Autoimmune thyroid disease and other non-endocrine autoimmune diseases

    OpenAIRE

    Todorović-Đilas Ljiljana; Ičin Tijana; Novaković-Paro Jovanka; Bajkin Ivana

    2011-01-01

    Introduction, Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a...

  6. Epilepsy in systemic autoimmune disorders.

    Science.gov (United States)

    Valencia, Ignacio

    2014-09-01

    Autoimmunity and inflammation have been implicated as causative factors of seizures and epilepsy. Autoimmune disorders can affect the central nervous system as an isolated syndrome or be part of a systemic disease. Examples of systemic autoimmune disorders include systemic lupus erythematosus, antiphospholipid syndrome, rheumatic arthritis, and Sjögren syndrome. Overall, there is a 5-fold increased risk of seizures and epilepsy in children with systemic autoimmune disorders. Various etiologic factors have been implicated in causing the seizures in these patients, including direct inflammation, effect on blood vessels (vasculitis), and production of autoantibodies. Potential treatments for this autoimmune injury include steroids, immunoglobulins, and other immune-modulatory therapies. A better understanding of the mechanisms of epileptogenesis in patients with systemic autoimmune diseases could lead to targeted treatments and better outcomes. PMID:25510945

  7. Interleukin-2 critically regulates bone marrow erythropoiesis and prevents anemia development.

    Science.gov (United States)

    Chopra, Martin; Langenhorst, Daniela; Beilhack, Andreas; Serfling, Edgar; Patra, Amiya K

    2015-12-01

    Mice deficient in IL-2 signaling develop severe anemia indicating a defect in erythropoiesis. However, why deficiency in IL-2, an essential growth factor for lymphocytes, or in IL-2 signaling components should result in defective erythropoiesis is unclear. Here, we have analyzed the mechanism of IL-2 signaling deficiency induced anemia in mice and show that IL-2 plays an indispensable role in bone marrow (BM) erythropoiesis via maintenance of regulatory T (Treg) cells. In absence of IL-2 signaling, IFN-γ produced by the activated T cells suppressed klf1 expression, resulting in an early block in erythrocyte differentiation. Anemia, in IL-2 or IL-2 signaling deficient mice always developed prior to the manifestation of other autoimmune complications such as colitis, suggesting that anemia in these mice might be a contributing factor in inducing other pathological complications in later stages. Our study shows, how essential cytokines of lymphoid cells could exert critical influence on the development of erythrocytes and thus expanding our understanding of the complex regulation of hematopoiesis in the BM. Besides, our findings might facilitate the use of IL-2 and anti-IFN-γ as a clinical remedy against anemia that arise in cancer patients following radiotherapy or chemotherapy, a context which simulates the situation of IL-2 deficiency. PMID:26404745

  8. Autoimmune Thyroid Diseases in Children

    OpenAIRE

    Francesca Crea; Carla Bizzarri; Marco Cappa

    2011-01-01

    The two major autoimmune thyroid diseases (ATDs) include Graves' disease (GD) and autoimmune thyroiditis (AT); both of which are characterized by infiltration of the thyroid by T and B cells reactive to thyroid antigens, by the production of thyroid autoantibodies and by abnormal thyroid function (hyperthyroidism in GD and hypothyroidism in AT). While the exact etiology of thyroid autoimmunity is not known, it is believed to develop when a combination of genetic susceptibility and environment...

  9. Genetics Home Reference: congenital dyserythropoietic anemia

    Science.gov (United States)

    ... Understand Genetics Home Health Conditions CDA congenital dyserythropoietic anemia Enable Javascript to view the expand/collapse boxes. ... PDF Open All Close All Description Congenital dyserythropoietic anemia ( CDA ) is an inherited blood disorder that affects ...

  10. Anemia Boosts Stroke Death Risk, Study Finds

    Science.gov (United States)

    ... page: https://medlineplus.gov/news/fullstory_160476.html Anemia Boosts Stroke Death Risk, Study Finds Blood condition ... 2016 (HealthDay News) -- Older stroke victims suffering from anemia -- a lack of red blood cells -- may have ...

  11. FastStats: Anemia or Iron Deficiency

    Science.gov (United States)

    ... this? Submit What's this? Submit Button NCHS Home Anemia or Iron Deficiency Recommend on Facebook Tweet Share ... visits Number of visits to emergency departments with anemia as the primary hospital discharge diagnosis: 237,000 ...

  12. Autoantibodies in autoimmune liver diseases.

    Science.gov (United States)

    Sener, Asli Gamze

    2015-11-01

    Autoimmune hepatitis is a chronic hepatitis of unknown etiology characterized by clinical, histological, and immunological features, generally including circulating autoantibodies and a high total serum and/or gamma globulin. Liver-related autoantibodies are very significant for the correct diagnosis and classification of autoimmune liver diseases (AILD), namely autoimmune hepatitis types 1 and 2 (AIH-1 and 2), primary biliary cirrhosis (PBC), and the sclerosing cholangitis types in adults and children. This article intends to review recent studies that investigate autoantibodies in autoimmune liver diseases from a microbiological perspective.

  13. Iron deficiency anemia in children.

    Science.gov (United States)

    Subramaniam, Girish; Girish, Meenakshi

    2015-06-01

    Iron deficiency is not just anemia; it can be responsible for a long list of other manifestations. This topic is of great importance, especially in infancy and early childhood, for a variety of reasons. Firstly, iron need is maximum in this period. Secondly, diet in infancy is usually deficient in iron. Thirdly and most importantly, iron deficiency at this age can result in neurodevelopmental and cognitive deficits, which may not be reversible. Hypochromia and microcytosis in a complete blood count (CBC) makes iron deficiency anemia (IDA) most likely diagnosis. Absence of response to iron should make us look for other differential diagnosis like β thalassemia trait and anemia of chronic disease. Celiac disease is the most important cause of true IDA not responding to oral iron therapy. While oral ferrous sulphate is the cheapest and most effective therapy for IDA, simple nonpharmacological and pharmacological measures can go a long way in prevention of iron deficiency. PMID:25636824

  14. HEMOGLOBIN PRODUCTION FACTORS IN THE HUMAN LIVER : ANEMIAS, HYPOPROTEINEMIA, CIRRHOSIS, PIGMENT ABNORMALITIES, AND PREGANCY.

    Science.gov (United States)

    Whipple, G H; Robscheit-Robbins, F S

    1942-09-01

    Human liver tissue has been assayed to determine the amount of hemoglobin production factors in normal and abnormal states. Standardized dogs made anemic by blood removal have been used in this biological assay. Normal animal liver as control is rated as 100 per cent. Normal human liver tissue as compared with the normal animal control contains more of these hemoglobin production factors-a biological assay ratio of 120 to 160 per cent. Infections, acute and chronic, do not appear to modify these values, the concentration of hemoglobin-producing factors falling within the normal range. Pernicious anemia and aplastic anemia both show large liver stores of hemoglobin-producing factors-a biological assay ratio of 200 to 240 per cent. Therapy in pernicious anemia reduces these liver stores as new red cells are formed. Secondary anemia presents a low normal or subnormal liver store of hemoglobin-producing factors-an assay of 60 to 130 per cent. Hemochromatosis, erythroblastic anemia, and hemolytic icterus in spite of large iron deposits in the liver usually show a biological assay which is normal or close to normal. Polycythemia shows low reserve stores of hemoglobin-producing factors. Leukemias present a wide range of values discussed above. Hypoproteinemia almost always is associated with low reserve stores of hemoglobin-producing factors in the liver-biological assays of 60 to 80 per cent. Hypoproteinemia means a depletion of body protein reserve stores including the labile protein liver reserves-a strong indication that the prehemoglobin material (or globin) is related to these liver stores. Pregnancy, eclampsia, and lactation all may present subnormal liver stores of hemoglobin-producing factors. Exhaustion of protein stores lowers the barrier to infection and renders the liver very susceptible to many toxic substances. It should not be difficult to correct hypoproteinemia under these conditions and thus relieve the patient of a real hazard.

  15. Neuropathology of autoimmune encephalitides.

    Science.gov (United States)

    Bauer, Jan; Bien, Christian G

    2016-01-01

    In recent years a large number of antibody-associated or antibody-defined encephalitides have been discovered. These conditions are often referred to as autoimmune encephalitides. The clinical features include prominent epileptic seizures, cognitive and psychiatric disturbance. These encephalitides can be divided in those with antibodies against intracellular antigens and those with antibodies against surface antigens. The discovery of new antibodies against targets on the surface of neurons is especially interesting since patients with such antibodies can be successfully treated immunologically. This chapter focuses on the pathology and the pathogenetic mechanisms involved in these encephalitides and discusses some of the questions that are raised in this exciting new field. It is important to realise, however, that because of the use of antibodies to diagnose the patients, and their improvement with treatment, there are relatively few biopsy or postmortem reports, limiting the neuropathological data and conclusions that can be drawn. For this reason we especially focus on the most frequent autoimmune encephalitides, those with antibodies to the NMDA receptor and with antibodies to the known protein components of the VGKC complex. Analysis of these encephalitides show completely different pathogenic mechanisms. In VGKC complex encephalitis, antibodies seem to bind to their target and activate complement, leading to destruction and loss of neurons. On the other hand, in NMDAR encephalitis, complement activation and neuronal degeneration seems to be largely absent. Instead, binding of antibodies leads to a decrease of NMDA receptors resulting in a hypofunction. This hypofunction offers an explanation for some of the clinical features such as psychosis and episodic memory impairment, but not for the frequent seizures. Thus, additional analysis of the few human brain specimens present and the use of specific animal models are needed to further understand the effects

  16. Neuropathology of autoimmune encephalitides.

    Science.gov (United States)

    Bauer, Jan; Bien, Christian G

    2016-01-01

    In recent years a large number of antibody-associated or antibody-defined encephalitides have been discovered. These conditions are often referred to as autoimmune encephalitides. The clinical features include prominent epileptic seizures, cognitive and psychiatric disturbance. These encephalitides can be divided in those with antibodies against intracellular antigens and those with antibodies against surface antigens. The discovery of new antibodies against targets on the surface of neurons is especially interesting since patients with such antibodies can be successfully treated immunologically. This chapter focuses on the pathology and the pathogenetic mechanisms involved in these encephalitides and discusses some of the questions that are raised in this exciting new field. It is important to realise, however, that because of the use of antibodies to diagnose the patients, and their improvement with treatment, there are relatively few biopsy or postmortem reports, limiting the neuropathological data and conclusions that can be drawn. For this reason we especially focus on the most frequent autoimmune encephalitides, those with antibodies to the NMDA receptor and with antibodies to the known protein components of the VGKC complex. Analysis of these encephalitides show completely different pathogenic mechanisms. In VGKC complex encephalitis, antibodies seem to bind to their target and activate complement, leading to destruction and loss of neurons. On the other hand, in NMDAR encephalitis, complement activation and neuronal degeneration seems to be largely absent. Instead, binding of antibodies leads to a decrease of NMDA receptors resulting in a hypofunction. This hypofunction offers an explanation for some of the clinical features such as psychosis and episodic memory impairment, but not for the frequent seizures. Thus, additional analysis of the few human brain specimens present and the use of specific animal models are needed to further understand the effects

  17. Deep vein thrombosis, an unreported first manifestation of polyglandular autoimmune syndrome type III

    Directory of Open Access Journals (Sweden)

    M Horsey

    2016-07-01

    Full Text Available A 71-year-old woman with severe right lower leg pain, edema and erythema was presented to the Emergency Department and was found to have an extensive deep vein thrombosis (DVT confirmed by ultrasound. She underwent an extensive evaluation due to her prior history of malignancy and new hypercoagulable state, but no evidence of recurrent disease was detected. Further investigation revealed pernicious anemia (PA, confirmed by the presence of a macrocytic anemia (MCV=115.8fL/red cell, Hgb=9.0g/dL, decreased serum B12 levels (56pg/mL, with resultant increased methylmalonic acid (5303nmol/L and hyperhomocysteinemia (131μmol/L, the presumed etiology of the DVT. The patient also suffered from autoimmune thyroid disease (AITD, and both antithyroglobulin and anti-intrinsic factor antibodies were detected. She responded briskly to anticoagulation with heparin and coumadin and treatment of PA with intramuscular vitamin B12 injections. Our case suggests that a DVT secondary to hyperhomocystenemia may represent the first sign of polyglandular autoimmune syndrome III-B (PAS III-B, defined as the coexistent autoimmune conditions AITD and PA. It is important to recognize this clinical entity, as patients may not only require acute treatment with vitamin B12 supplementation and prolonged anticoagulation, as in this patient, but may also harbor other autoimmune diseases.

  18. Síndrome hemolítico-urêmica esporádica pós-parto Sporadic postpartum hemolytic uremic syndrome

    Directory of Open Access Journals (Sweden)

    Elza M. Moreira

    2008-08-01

    Full Text Available Anemia hemolítica microangiopática associado à trombocitopenia participa de um grupo de doenças que freqüentemente apresentam suas características clínicas muito semelhantes, sendo difícil distingui-las. A síndrome hemolítico-urêmica é dividida em duas apresentações: a forma não esporádica, que acomete comumente crianças após infecção bacteriana causando diarréia sanguinolenta, possui bom prognóstico; e a forma esporádica, que acomete adultos, sendo bem descritos casos em mulheres pósparto, é a forma sistêmica de trombocitopenia microangiopática de pior prognóstico com alta morbidade e mortalidade, cuja falência renal é o distúrbio predominante. Relatamos um caso de síndrome hemolítico-urêmica pós-parto em paciente previamente sadia, que apresentou quadro de insuficiência renal, anemia hemolítica e trombocitopenia. Instituída a terapêutica de suporte adequada e precocemente, a paciente evoluiu satisfatoriamente com normalização dos níveis pressóricos e recuperação da função renal.Microangiopathic hemolytic associated with thrombocytopenia is part of a disease group that frequently show likeness and that's why become difficult to separate them. There are two types of hemolytic uremic syndrome (HUS; the non sporadic type and the epidemic or "typical" type that is common on childreen that is associated with diarrhea and infection caused by verotoxinaproducing E. coli with a good prognostic; and the sporadic postpartum period. It is the systemic type of mocroangiophatic thrombocytopenia of poor prognostic with high morbidity and mortality which renal failure is the main disturb. We reported a case of HUS occuring in postpartum previously healthy, that showed abrupt renal failure, hemolytic anemia and thrombocytopenia. After proper therapy the patient developed a normal blood pressure and recovery renal function.

  19. Infections and autoimmune diseases.

    Science.gov (United States)

    Bach, Jean-François

    2005-01-01

    The high percentage of disease-discordant pairs of monozygotic twins demonstrates the central role of environmental factors in the etiology of autoimmune diseases. Efforts were first focussed on the search for triggering factors. The study of animal models has clearly shown that infections may trigger autoimmune diseases, as in the case of Coxsackie B4 virus in type I diabetes and the encephalomyocarditis virus in autoimmune myositis, two models in which viruses are thought to act by increasing immunogenicity of autoantigens secondary to local inflammation. The induction of a Guillain-Barré syndrome in rabbits after immunization with a peptide derived from Campylobacter jejuni is explained by mimicry between C. jejuni antigens and peripheral nerve axonal antigens. Other models involve chemical modification of autoantigens, as in the case of iodine-induced autoimmune thyroiditis. These mechanisms have so far only limited clinical counterparts (rheumatic fever, Guillain-Barré syndrome and drug-induced lupus or myasthenia gravis) but one may assume that unknown viruses may be at the origin of a number of chronic autoimmune diseases, such as type I diabetes and multiple sclerosis) as illustrated by the convergent data incriminating IFN-alpha in the pathophysiology of type I diabetes and systemic lupus erythematosus. Perhaps the difficulties met in identifying the etiologic viruses are due to the long lag time between the initial causal infection and onset of clinical disease. More surprisingly, infections may also protect from autoimmune diseases. Western countries are being confronted with a disturbing increase in the incidence of most immune disorders, including autoimmune and allergic diseases, inflammatory bowel diseases, and some lymphocyte malignancies. Converging epidemiological evidence indicates that this increase is linked to improvement of the socio-economic level of these countries, posing the question of the causal relationship and more precisely the

  20. Erythroblast transferrin receptors and transferrin kinetics in iron deficiency and various anemias

    Energy Technology Data Exchange (ETDEWEB)

    Muta, K.; Nishimura, J.; Ideguchi, H.; Umemura, T.; Ibayashi, H.

    1987-06-01

    To clarify the role of transferrin receptors in cases of altered iron metabolism in clinical pathological conditions, we studied: number of binding sites; affinity; and recycling kinetics of transferrin receptors on human erythroblasts. Since transferrin receptors are mainly present on erythroblasts, the number of surface transferrin receptors was determined by assay of binding of /sup 125/I-transferrin and the percentage of erythroblasts in bone marrow mononuclear cells. The number of binding sites on erythroblasts from patients with an iron deficiency anemia was significantly greater than in normal subjects. Among those with an aplastic anemia, hemolytic anemia, myelodysplastic syndrome, and polycythemia vera compared to normal subjects, there were no considerable differences in the numbers of binding sites. The dissociation constants (Kd) were measured using Scatchard analysis. The apparent Kd was unchanged (about 10 nmol/L) in patients and normal subjects. The kinetics of endocytosis and exocytosis of /sup 125/I-transferrin, examined by acid treatment, revealed no variations in recycling kinetics among the patients and normal subjects. These data suggest that iron uptake is regulated by modulation of the number of surface transferrin receptors, thereby reflecting the iron demand of the erythroblast.

  1. Mast Cell and Autoimmune Diseases

    OpenAIRE

    Yunzhi Xu; Guangjie Chen

    2015-01-01

    Mast cells are important in innate immune system. They have been appreciated as potent contributors to allergic reaction. However, increasing evidence implicates the important role of mast cells in autoimmune disease like rheumatoid arthritis and multiple sclerosis. Here we review the current stage of knowledge about mast cells in autoimmune diseases.

  2. Aetiopathogenesis of autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    Diego Vergani; Giorgina Mieli-Vergani

    2008-01-01

    The histological hallmark of autoimmune hepatitis (AIH) is a dense portal mononuclear cell infiltrate that invades the surrounding parenchyma and comprises T and B lymphocytes,macrophages,and plasma cells.An unknown but powerful stimulus must be promoting the formation of this massive inflammatory cellular reaction that is likely to initiate and perpetuate liver damage.An autoimmune attack can follow different pathways to inflict damage on hepatocytes.Liver damage is likely to be orchestrated by CD4+T lymphocytes recognizing an autoantigenic liver peptide.To trigger an autoimmune response,the peptide must be embraced by an HLA class Ⅱ molecule and presented to naive CD4+T helper (Th0) cells by professional antigen presenting cells,with the co-stimulation of ligand-ligand fostering interaction between the two cells.Th0 cells become activated,differentiate into functional phenotypes according to the cytokines prevailing in the microenvironment and the nature of the antigen,and initiate a cascade of immune reactions determined by the cytokines produced by the activated T cells.Th1 cells,arising in the presence of the macrophage-derived interleukin (IL)-12,secrete mainly IL-2 and interferon-gamma (IFN-γ),which activate macrophages,enhance expression of HLA class Ⅰ (increasing liver cell vulnerability to a CD8+T cell cytotoxic attack),and induce expression of HLA class Ⅱ molecules on hepatocytes.Th2 cells,which differentiate from Th0 if the microenvironment is rich in IL-4,produce mainly IL-4,IL-10,and IL-13 which favour autoantibody production by B lymphocytes.Physiologically,Th1 and Th2 antagonize each other.Th17 cells,a recently described population,arise in the presence of transforming growth factor beta (TGF-β) and IL-6 and appear to have an important effector role in inflammation and autoimmunity.The process of autoantigen recognition is strictly controlled by regulatory mechanisms,such as those exerted by CD4+CD25+regulatory T cells,which derive from Th0

  3. Anemia and survival in human immunodeficiency virus

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Mocroft, Amanda

    2003-01-01

    The prospective, multicenter cohort study EuroSIDA has previously reported on predictors and outcomes of anemia in patients infected with human immunodeficiency virus. In a Cox proportional-hazards model with serial measures of CD4+ cell count, plasma viral load, and degrees of anemia fitted...... as time-dependent variables, the relative hazard of death increased markedly for patients with anemia versus no anemia. A clinical scoring system was developed and validated for patients receiving highly active antiretroviral therapy using the most recent laboratory measures. Mild and severe anemia were...... independently (Panemia. The mechanisms underlying why hemoglobin is such a strong prognostic...

  4. In vitro assessment of recombinant, mutant immunoglobulin G anti-D devoid of hemolytic activity for treatment of ongoing hemolytic disease of the fetus and newborn

    DEFF Research Database (Denmark)

    Nielsen, Leif K; Green, Trine H; Sandlie, Inger;

    2008-01-01

    A specific treatment for ongoing hemolytic disease of the fetus and newborn (HDFN) due to anti-D would be very attractive. One approach could be administration to the mother of nonhemolytic anti-D, which by crossing the placenta can block the binding of hemolytic maternal anti-D....

  5. Cooley's Anemia: A Psychosocial Directory.

    Science.gov (United States)

    National Center for Education in Maternal and Child Health, Washington, DC.

    The directory is intended to aid patients and their families who are coping with the genetic disorder of Cooley's anemia. A brief review of the disease covers background, genetics, symptoms, effect on the patient, treatment, and current research. The next section looks at psychosocial needs at various times (time of diagnosis, infancy and toddler…

  6. Anemia in the general population

    DEFF Research Database (Denmark)

    Martinsson, Andreas; Andersson, Charlotte; Andell, Pontus;

    2014-01-01

    Low hemoglobin concentration is associated with increased mortality, but there is disagreement with regard to the clinical definition of anemia. We aimed to evaluate the prevalence, clinical correlates and association with total and cause-specific long-term mortality across the hemoglobin distrib...

  7. An anemia of Alzheimer's disease.

    Science.gov (United States)

    Faux, N G; Rembach, A; Wiley, J; Ellis, K A; Ames, D; Fowler, C J; Martins, R N; Pertile, K K; Rumble, R L; Trounson, B; Masters, C L; Bush, A I

    2014-11-01

    Lower hemoglobin is associated with cognitive impairment and Alzheimer's disease (AD). Since brain iron homeostasis is perturbed in AD, we investigated whether this is peripherally reflected in the hematological and related blood chemistry values from the Australian Imaging Biomarker and Lifestyle (AIBL) study (a community-based, cross-sectional cohort comprising 768 healthy controls (HC), 133 participants with mild cognitive impairment (MCI) and 211 participants with AD). We found that individuals with AD had significantly lower hemoglobin, mean cell hemoglobin concentrations, packed cell volume and higher erythrocyte sedimentation rates (adjusted for age, gender, APOE-ɛ4 and site). In AD, plasma iron, transferrin, transferrin saturation and red cell folate levels exhibited a significant distortion of their customary relationship to hemoglobin levels. There was a strong association between anemia and AD (adjusted odds ratio (OR)=2.43, confidence interval (CI) (1.31, 4.54)). Moreover, AD emerged as a strong risk factor for anemia on step-down regression, even when controlling for all other available explanations for anemia (adjusted OR=3.41, 95% CI (1.68, 6.92)). These data indicated that AD is complicated by anemia, which may itself contribute to cognitive decline. PMID:24419041

  8. A Web Server and Mobile App for Computing Hemolytic Potency of Peptides

    Science.gov (United States)

    Chaudhary, Kumardeep; Kumar, Ritesh; Singh, Sandeep; Tuknait, Abhishek; Gautam, Ankur; Mathur, Deepika; Anand, Priya; Varshney, Grish C.; Raghava, Gajendra P. S.

    2016-03-01

    Numerous therapeutic peptides do not enter the clinical trials just because of their high hemolytic activity. Recently, we developed a database, Hemolytik, for maintaining experimentally validated hemolytic and non-hemolytic peptides. The present study describes a web server and mobile app developed for predicting, and screening of peptides having hemolytic potency. Firstly, we generated a dataset HemoPI-1 that contains 552 hemolytic peptides extracted from Hemolytik database and 552 random non-hemolytic peptides (from Swiss-Prot). The sequence analysis of these peptides revealed that certain residues (e.g., L, K, F, W) and motifs (e.g., “FKK”, “LKL”, “KKLL”, “KWK”, “VLK”, “CYCR”, “CRR”, “RFC”, “RRR”, “LKKL”) are more abundant in hemolytic peptides. Therefore, we developed models for discriminating hemolytic and non-hemolytic peptides using various machine learning techniques and achieved more than 95% accuracy. We also developed models for discriminating peptides having high and low hemolytic potential on different datasets called HemoPI-2 and HemoPI-3. In order to serve the scientific community, we developed a web server, mobile app and JAVA-based standalone software (http://crdd.osdd.net/raghava/hemopi/).

  9. HEMOLYTIC ASPECTS OF STRAINS AND CLONES OF Trichomonas vaginalis

    OpenAIRE

    GERALDO A DE CARLI; PHILIPPE BRASSEUR; TIANA TASCA; da Silva, Ana C; DANIELA S CASTILHOS; ALINE WENDORFF

    1998-01-01

    The in vitro hemolytic activity of live strains and clones of trophozoites of Trichomonas vaginalis was investigated. The isolates and clones were tested against human erythrocytes of groups A, B, AB, and O, and against erythrocytes of six adult animals of different species (rabbit, rat, chicken, horse, bovine, and sheep). Results showed that each of the isolates and clones tested lysed all human blood groups, as well as rabbit, rat, chicken, horse, bovine and sheep erythrocytes. No hemolysin...

  10. Hemolytic disease of the newborn due to anti-U

    Directory of Open Access Journals (Sweden)

    Novaretti Marcia Cristina Zago

    2003-01-01

    Full Text Available Anti-U is a rare red blood cell alloantibody that has been found exclusively in blacks. It can cause hemolytic disease of the newborn and hemolytic transfusion reactions. We describe the case of a female newborn presenting a strongly positive direct antiglobulin test due to an IgG antibody in cord blood. Anti-U was recovered from cord blood using acid eluate technique. Her mother presented positive screening of antibodies with anti-U identified at delivery. It was of IgG1 and IgG3 subclasses and showed a titer of 32. Monocyte monolayer assay showed moderate interaction of Fc receptors with maternal serum with a positive result (3.1%. The newborn was treated only with 48 hours of phototherapy for mild hemolytic disease. She recovered well and was discharged on the 4th day of life. We conclude that whenever an antibody against a high frequency erythrocyte antigen is identified in brown and black pregnant women, anti-U must be investigated.

  11. Hemolytic disease of the newborn due to anti-U.

    Science.gov (United States)

    Novaretti, Marcia Cristina Zago; Jens, Eduardo; Pagliarini, Thiago; Bonif cio, Silvia Le o; Dorlhiac-Llacer, Pedro Enrique; Chamone Dd, Dalton de Alencar Fischer

    2003-01-01

    Anti-U is a rare red blood cell alloantibody that has been found exclusively in blacks. It can cause hemolytic disease of the newborn and hemolytic transfusion reactions. We describe the case of a female newborn presenting a strongly positive direct antiglobulin test due to an IgG antibody in cord blood. Anti-U was recovered from cord blood using acid eluate technique. Her mother presented positive screening of antibodies with anti-U identified at delivery. It was of IgG1 and IgG3 subclasses and showed a titer of 32. Monocyte monolayer assay showed moderate interaction of Fc receptors with maternal serum with a positive result (3.1%). The newborn was treated only with 48 hours of phototherapy for mild hemolytic disease. She recovered well and was discharged on the 4th day of life. We conclude that whenever an antibody against a high frequency erythrocyte antigen is identified in brown and black pregnant women, anti-U must be investigated. PMID:14762491

  12. Serratamolide is a hemolytic factor produced by Serratia marcescens.

    Directory of Open Access Journals (Sweden)

    Robert M Q Shanks

    Full Text Available Serratia marcescens is a common contaminant of contact lens cases and lenses. Hemolytic factors of S. marcescens contribute to the virulence of this opportunistic bacterial pathogen. We took advantage of an observed hyper-hemolytic phenotype of crp mutants to investigate mechanisms of hemolysis. A genetic screen revealed that swrW is necessary for the hyper-hemolysis phenotype of crp mutants. The swrW gene is required for biosynthesis of the biosurfactant serratamolide, previously shown to be a broad-spectrum antibiotic and to contribute to swarming motility. Multicopy expression of swrW or mutation of the hexS transcription factor gene, a known inhibitor of swrW expression, led to an increase in hemolysis. Surfactant zones and expression from an swrW-transcriptional reporter were elevated in a crp mutant compared to the wild type. Purified serratamolide was hemolytic to sheep and murine red blood cells and cytotoxic to human airway and corneal limbal epithelial cells in vitro. The swrW gene was found in the majority of contact lens isolates tested. Genetic and biochemical analysis implicate the biosurfactant serratamolide as a hemolysin. This novel hemolysin may contribute to irritation and infections associated with contact lens use.

  13. Lack of the lectin-like domain of thrombomodulin worsens Shiga toxin-associated hemolytic uremic syndrome in mice.

    Science.gov (United States)

    Zoja, Carlamaria; Locatelli, Monica; Pagani, Chiara; Corna, Daniela; Zanchi, Cristina; Isermann, Berend; Remuzzi, Giuseppe; Conway, Edward M; Noris, Marina

    2012-10-01

    Shiga toxin (Stx)-producing Escherichia coli is a primary cause of diarrhea-associated hemolytic uremic syndrome (HUS), a disorder of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The pathophysiology of renal microvascular thrombosis in Stx-HUS is still ill-defined. Based on evidence that abnormalities in thrombomodulin (TM), an anticoagulant endothelial glycoprotein that modulates complement and inflammation, predispose to atypical HUS, we assessed whether impaired TM function may adversely affect evolution of Stx-HUS. Disease was induced by coinjection of Stx2/LPS in wild-type mice (TM(wt/wt)) and mice that lack the lectin-like domain of TM (TM(LeD/LeD)), which is critical for its anti-inflammatory and cytoprotective properties. After Stx2/LPS, TM(LeD/LeD) mice exhibited more severe thrombocytopenia and renal dysfunction than TM(wt/wt) mice. Lack of lectin-like domain of TM resulted in a stronger inflammatory reaction after Stx2/LPS with more neutrophils and monocytes/macrophages infiltrating the kidney, associated with PECAM-1 and chemokine upregulation. After Stx2/LPS, intraglomerular fibrin(ogen) deposits were detected earlier in TM(LeD/LeD) than in TM(wt/wt) mice. More abundant fibrin(ogen) deposits were also found in brain and lungs. Under basal conditions, TM(LeD/LeD) mice exhibited excess glomerular C3 deposits, indicating impaired complement regulation in the kidney that could lead to local accumulation of proinflammatory products. TM(LeD/LeD) mice with HUS had a higher mortality rate than TM(wt/wt) mice. If applicable to humans, these findings raise the possibility that genetic or acquired TM defects might have an impact on the severity of microangiopathic lesions after exposure to Stx-producing E. coli infections and raise the potential for using soluble TM in the treatment of Stx-HUS. PMID:22942429

  14. Atypical Hemolytic Uremic Syndrome post Kidney Transplantation: Two Case Reports and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Sami eAlasfar

    2014-12-01

    Full Text Available Atypical hemolytic uremic syndrome (aHUS is a rare disorder characterized by over-activation and dysregulation of the alternative complement pathway. Its estimated prevalence is 1-2 per million. The disease is characterized by thrombotic microangiopathy, which causes anemia, thrombocytopenia, and acute renal failure. aHUS has more severe course compared to typical (Infection-induced HUS and is frequently characterized by relapses that leads to end stage renal disease (ESRD. For a long time, kidney transplantation for these patients was contraindicated because of high rate of recurrence and subsequent renal graft loss. The post-kidney transplantation recurrence rate largely depends on the pathogenetic mechanisms involved. However, over the past several years, advancements in the understanding and therapeutics of aHUS have allowed successful kidney transplantation in these patients. Eculizumab, which is a complement C5 antibody that inhibits complement factor 5a (C5a and subsequent formation of the membrane attack complex, has been used in prevention and treatment of post-transplant aHUS recurrence. In this paper, we present two new cases of aHUS patients who underwent successful kidney transplantation in our center with the use of prophylactic and maintenance eculizumab therapy that have not been published before. The purpose of reporting these two cases is to emphasize the importance of using eculizumab as a prophylactic therapy to prevent aHUS recurrence post transplant in high-risk patients. We will also review the current understanding of the genetics of aHUS, the pathogenesis of its recurrence after kidney transplantation, and strategies for prevention and treatment of post-transplant aHUS recurrence.

  15. Endocrine autoimmunity in Turner syndrome

    Science.gov (United States)

    2013-01-01

    Background Turner syndrome is caused by numeric and structural abnormalities of the X chromosome. An increased frequency of autoimmunity as well as an elevated incidence of autoantibodies was observed in Turner patients. The aim of this study was to conduct a retrospective analysis of the incidence of autoimmunity in 66 Italian patients affected by Turner syndrome. Methods Sixty-six unselected and consecutive Italian Turner patients were recruited. The association between age, karyotype and the presence of clinical/pre-clinical autoimmune disorders and of autoantibodies was examined. Results Out of the 66 Turner patients, 26 had thyroid autoimmune disorders (39.4%), 14 patients had Hashimoto’s thyroiditis with clinical or subclinical hypothyroidism (21.2%) and 12 patients had circulating anti-thyroid antibodies, echographic pattern of diffuse hypoechogenicity and normal thyroid hormone levels (18.2%). None were affected by Graves’ disease. We analyzed the overall incidence of thyroid autoimmunity within the 3 different age groups 0–9.9, 10–19.9 and 20–29.9 years. No statistically significant difference was observed in the incidence of thyroid autoimmunity within the age-groups (χ2-test p > 0.05). Out of the 66 patients, 31 patients had the 45,X karyotype; within this first group 14 out of 31 patients were affected by autoimmune thyroid disease. A second group of 29 patients included 19 patients with mosaicism, 5 patients with deletions and 5 patients with ring chromosome; out of these 29 patients 7 were affected by autoimmune thyroid disease. A third group included 6 patients with X isochromosome; 5 out of 6 were affected by autoimmune thyroid disease. A statistically significant difference in the frequency of thyroid autoimmunity within the different karyotype groups was observed (χ2-test p = 0.0173). When comparing the X isochromosome group with the pooled group of other karyotypes, of note, the frequency of thyroid autoimmunity was

  16. SOCS, inflammation and autoimmunity

    Directory of Open Access Journals (Sweden)

    Akihiko eYoshimura

    2012-03-01

    Full Text Available Cytokines play essential roles in innate and adaptive immunity. However, excess cytokines or dysregulation of cytokine signaling can cause a variety of diseases, including allergies, autoimmune diseases, inflammation, and cancer. Most cytokines utilize the so-called Janus kinase-signal transducers and activators of transcription (JAK-STAT pathway. This pathway is negatively regulated by various mechanisms including suppressors of cytokine signaling (SOCS proteins. SOCS proteins bind to JAK or cytokine receptors, thereby suppressing further signaling events. Especially, SOCS1 and SOCS3 are strong inhibitors of JAK, because these two contain kinase inhibitory region (KIR at the N-terminus. Studies using conditional knockout mice have shown that SOCS proteins are key physiological as well as pathological regulators of immune homeostasis. Recent studies have also demonstrated that SOCS1 and SOCS3 are important regulators of helper T cell differentiation and functions.

  17. Adult autoimmune enteropathy

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Recent reports have suggested that autoimmune enteropathy involving the small bowel may occur in adults as well as in children. Apparently, the endoscopic and histological changes are similar to celiac disease before treatment, but these are not altered by any form of dietary restriction, including a gluten-free diet. As in celiac disease, histologic changes in gastric and colonic biopsies have also been recorded. Anti enterocyte antibodies detected with immunofluorescent methods have been reported by a few laboratories, but these antibodies appear not to be specific and may simply represent epiphenomena. A widely available, reproducible and quantitative anti-enterocyte antibody assay is needed that could be applied in small bowel disorders that have the histological appearance of celiac disease, but fail to respond to a gluten-free diet.

  18. Psychoneuroimmunology - psyche and autoimmunity.

    Science.gov (United States)

    Ziemssen, Tjalf

    2012-01-01

    Psychoneuroimmunology is a relatively young field of research that investigates interactions between central nervous and immune system. The brain modulates the immune system by the endocrine and autonomic nervous system. Vice versa, the immune system modulates brain activity including sleep and body temperature. Based on a close functional and anatomical link, the immune and nervous systems act in a highly reciprocal manner. From fever to stress, the influence of one system on the other has evolved in an intricate manner to help sense danger and to mount an appropriate adaptive response. Over recent decades, reasonable evidence has emerged that these brain-to-immune interactions are highly modulated by psychological factors which influence immunity and autoimmune disease. For several diseases, the relevance of psychoneuroimmunological findings has already been demonstrated.

  19. Pathophysiology of autoimmune polyneuropathies.

    Science.gov (United States)

    Dalakas, Marinos C

    2013-06-01

    The most common autoimmune neuropathies include the acute inflammatory polyneuropathy [the Guillain-Barré Syndrome(s)]; chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN) and IgM anti-MAG-antibody mediated paraproteinemic neuropathy. These neuropathies occur when immunologic tolerance to peripheral nerve components (myelin, Schwann cell, axon, and motor or ganglionic neurons) is lost. Based on the immunopathologic similarities with experimental allergic neuritis induced after immunization with nerve proteins, disease transfer experiments with the patients' serum or with intraneural injections, and immunocytochemical studies on the patients' nerves, it appears that both cellular and humoral factors, either independently or in concert with each other, play a role in the cause of these neuropathies. Although in some of them there is direct evidence for autoimmune reactivity mediated by specific antibodies or autoreactive T lymphocytes, in others the underlying immune-mediated mechanisms have not been fully elucidated, in spite of good response to immunotherapies. The review highlights the factors associated with breaking the T-cell tolerance, the T-cell activation and costimulatory molecules, the immunoregulatory T-cells and relevant cytokines and the antibodies against peripheral nerve glycolipids or glycoproteins that seem to be of pathogenic relevance. Antigens in the nodal, paranodal and juxtaparanodal regions are discussed as potentially critical targets in explaining conduction failure and rapid recovery. Based on the immunopathologic network believed to play a fundamental role in the pathogenesis of these neuropathies, future therapeutic directions are highlighted using new biological agents against T-cells, cytokines, B-cells, transmigration and transduction molecules.

  20. 感染致贫血的研究进展%Advances in the studies of infection-induced anemia

    Institute of Scientific and Technical Information of China (English)

    洪之武

    2012-01-01

    Anemia is frequently associated with infection in various degrees and in various forms, and the most commonly seen is the anemia of inflammation induced by the over-expression of hepcidin, followed by hemolytic anemia, red cell aplasia, and blood loss. The degree of anemia may not be necessarily in proportion to the severity of infection, nor is it specific to the type of infection. A clear understanding of the diverse causes and recognition of frequent conspiracy of various factors may contribute enormously to the cor-rect diagnosis and appropriate management of the disease.%贫血(anemia)常以不同程度、不同形式与感染(infection)伴随存在,其中由海帕西啶(hepcidin)的过度表达所导致的炎性贫血(anemia of inflammation,AOI)最为常见,同时还包括溶血性贫血、红细胞增殖分化障碍和失血等,且不同原因所致贫血有其特有病理生理机制.贫血程度与感染严重程度之间也未必呈现必然的比例关系,此类贫血也非针对于特定类型的感染.临床医师对导致贫血的不同原因要有清晰的了解,同时认识这些原因极有可能联合存在,对于形成正确的诊断和恰当的处理思路相当重要,并极有帮助.

  1. Intravenous Immunoglobulin G Treatment in ABO Hemolytic Disease of the Newborn, is it Myth or Real?

    OpenAIRE

    Beken, Serdar; Hirfanoglu, Ibrahim; Turkyilmaz, Canan; Altuntas, Nilgun; Unal, Sezin; Turan, Ozden; Onal, Esra; Ergenekon, Ebru; Koc, Esin; Atalay, Yildiz

    2012-01-01

    Intravenous Immunoglobulin G (IVIG) therapy has been used as a component of the treatment of hemolytic disease of the newborn. There is still no consensus on its use in ABO hemolytic disease of the newborn routinely. The aim of this study is to determine whether administration of IVIG to newborns with ABO incompatibility is necessary. One hundred and seventeen patients with ABO hemolytic disease and positive Coombs test were enrolled into the study. The subjects were healthy except jaundice. ...

  2. Hemolytic disease of the fetus and newborn: Current trends and perspectives

    OpenAIRE

    Basu Sabita; Kaur Ravneet; Kaur Gagandeep

    2011-01-01

    The spectrum of hemolytic disease of the newborn has changed over the last few decades. With the implementation of Rhesus D immunoprophylaxis, hemolytic disease due to ABO incompatibility and other alloantibodies has now emerged as major causes of this condition. Though in developing countries, anti D is still a common antibody in pregnant women, many Asian countries have identified alloantibodies other than anti D as a cause of moderate-severe hemolytic disease. The most concerned fact is th...

  3. Characteristics of hemolytic activity induced by skin secretions of the frog Kaloula pulchra hainana

    OpenAIRE

    Wei, Shuangshuang; Chi, Tingting; Meng, Aiyun; Chen, Congwei; An, Tianchen; Wang, Manchuriga; Zhang, Yingxia

    2013-01-01

    Background The hemolytic activity of skin secretions obtained by stimulating the frog Kaloula pulchra hainana with diethyl ether was tested using human, cattle, rabbit, and chicken erythrocytes. The skin secretions had a significant concentration-dependent hemolytic effect on erythrocytes. The hemolytic activity of the skin secretions was studied in the presence of osmotic protectants (polyethylene glycols and carbohydrates), cations (Mg2+, Ca2+, Ba2+, Cu2+, and K+), or antioxidants (ascorbic...

  4. [Autoimmune pancreatitis as an element of autoimmune polyglandular syndrome].

    Science.gov (United States)

    Dyrla, Przemysław; Nowak, Tomasz; Gil, Jerzy; Adamiec, Cezary; Bobula, Mariusz; Saracyn, Marek

    2016-05-01

    Autoimmune pancreatitis constantly belongs to diseases which often causes significant diagnostic problem and often runs out with surgical intervention as considered to be a pancreatic cancer. Important although usually underestimated problems are polyglandular syndromes, which may consist of autoimmune pancreatitis (AIP) problem as well. This case report is an example of autoimmune polyglandular syndrome (APS), which was connected with the surgical treatment with biliary bypass anastomosis because of the unresectable lesion in the head of pancreas. The definite remission of the pancreatic lesion finally came after a steroid therapy. Differentiation between neoplastic and inflammatory pancreatic tumors very often remains a serious clinical problem. On grounds of imaging and cytopathology exams it is often difficult to decide about the nature of a lesion. The negative result of cytopathological biopsy examination does not finally settle straightforward diagnosis. Diagnostic problems affect also autoimmune pancreatitis. It is worth to undertake attempts to differentiate pancreatic lesions especially in cases of concomitance with other autoimmune polyglandular syndromes. That is because it is connected with completely different treatment and outcome. We should remember about diagnostic criteria of autoimmune pancreatitis. Appropriate diagnosis for patients with AIP gives them a chance to avoid serious surgical resection and possible complications.

  5. Fanconi anemia - learning from children

    Directory of Open Access Journals (Sweden)

    Johanna Svahn

    2011-06-01

    Full Text Available Fanconi Anemia (FA is a rare autosomic recessive and X-linked disease with chromosomal instability after exposure to crosslinking agents as the hallmark. Clinical features of FA are somatic malformations, progressive bone marrow failure and cancer proneness, however there is wide clinical heterogeneity. The symptom most frequently and early associated with morbidity and mortality is progressive pancytopenia in the first decade of life although acute myelogenous leukemia (AML or myelodysplastic syndrome (MDS can appear before aplastic anemia. Squamous cell carcinoma (SCC of the head-neck, intestinal or genital tract has a very high incidence in FA and can appear at young age. This paper will focus on treatment of bone marrow failure in FA.

  6. Pyrexia in a Patient with Megaloblastic Anemia: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Kevin Manuel

    2013-06-01

    Full Text Available Deficiency of vitamin B12 and/or folic acid as a cause of pyrexia, though known, is rarely reported in literature. We aimed to report a case in a 51 year old woman, who presented with fever and pancytopenia and was diagnosed to have megaloblastic anemia secondary to vitamin B12 and folate deficiency. The pyrexia subsided following the intramuscular injection of vitamin B12 and oral folic acid administration. All the other infective, inflammatory/autoimmune, endocrine causes of pyrexia were excluded by appropriate investigations. Therefore, we suggest that all physicians be aware of megaloblastic anemia as a treatable cause of pyrexia in order to avoid unnecessary costly investigations and antibiotic usage.

  7. Pathophysiology of anemia and erythrocytosis.

    Science.gov (United States)

    Hodges, Vivien M; Rainey, Susan; Lappin, Terence R; Maxwell, A Peter

    2007-11-01

    An increasing understanding of the process of erythropoiesis raises some interesting questions about the pathophysiology, diagnosis and treatment of anemia and erythrocytosis. The mechanisms underlying the development of many of the erythrocytoses, previously characterised as idiopathic, have been elucidated leading to an increased understanding of oxygen homeostasis. Characterisation of anemia and erythrocytosis in relation to serum erythropoietin levels can be a useful addition to clinical diagnostic criteria and provide a rationale for treatment with erythropoiesis stimulating agents (ESAs). Recombinant human erythropoietin as well as other ESAs are now widely used to treat anemias associated with a range of conditions, including chronic kidney disease, chronic inflammatory disorders and cancer. There is also heightened awareness of the potential abuse of ESAs to boost athletic performance in competitive sport. The discovery of erythropoietin receptors outside of the erythropoietic compartment may herald future applications for ESAs in the management of neurological and cardiac diseases. The current controversy concerning optimal hemoglobin levels in chronic kidney disease patients treated with ESAs and the potential negative clinical outcomes of ESA treatment in cancer reinforces the need for cautious evaluation of the pleiotropic effects of ESAs in non-erythroid tissues. PMID:17656101

  8. Autoimmune Inner Ear Disease (AIED)

    Science.gov (United States)

    ... to order. Mention “VEDA” to receive a 15% discount. Paid Advertisement Disclaimer Information on this website is ... treatment of autoimmune inner ear disease. Although drug companies are not directly studying treatments for inner ear ...

  9. Autoimmune Hepatitis and PSC Connection.

    Science.gov (United States)

    Vergani, Diego; Mieli-Vergani, Giorgina

    2008-02-01

    This article describes the connection between autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC). The two conditions have chronicity, liver inflammation, and a positive autoimmune serology in common; they differ in terms of gender distribution and bile duct damage. There is evidence suggesting that AIH and PSC are immune-mediated diseases. PSC and AIH could lie within the spectrum of the same disease process. Future studies should determine how frequently AIH evolves to PSC.

  10. Endocrine autoimmunity in Turner syndrome

    OpenAIRE

    Grossi, Armando; Crinò, Antonino; Luciano, Rosa; Lombardo, Antonietta; Cappa, Marco; Fierabracci, Alessandra

    2013-01-01

    Background Turner syndrome is caused by numeric and structural abnormalities of the X chromosome. An increased frequency of autoimmunity as well as an elevated incidence of autoantibodies was observed in Turner patients. The aim of this study was to conduct a retrospective analysis of the incidence of autoimmunity in 66 Italian patients affected by Turner syndrome. Methods Sixty-six unselected and consecutive Italian Turner patients were recruited. The association between age, karyotype and t...

  11. Diamond-Blackfan anemia and nutritional deficiency-induced anemia in children.

    Science.gov (United States)

    Gelbart, David

    2014-04-01

    Diamond-Blackfan anemia is a rare, inherited disease that characteristically presents as a chronic, normochromic macrocytosis due to red cell lineage bone marrow failure. Although studies are elaborating on the genetic basis for its associated comorbidities, little has been published comparing this anemia to other chronic anemias that have similar laboratory results in children. This article offers a global perspective of the disease and compares it with anemia due to vitamin B12 and folate deficiency in children.

  12. PERSEPSI TENTANG ANEMIA GIZI PADA REMAJA PUTRI PENDERITA ANEMIA DI SMAN 10 MAKASSAR

    OpenAIRE

    Hatma, Zumrah; Indriasari, Rahayu; Jafar, Nurhaedar

    2014-01-01

    Anemia gizi merupakan kelainan gizi yang paling sering ditemui di negara berkembang dan bersifat epidemik. Anemia gizi umumnya terjadi pada perempuan dalam usia reproduktif dan anak-anak. Keadaan ini membawa efek keseluruhan terbesar dalam hal gangguan kesehatan. Tujuan penelitian ini adalah untuk mengetahui persepsi tentang anemia gizi pada remaja putri penderita anemia. Teknik pengumpulan data melalui metode wawancara mendalam, serta focus group discussion (FGD). Selain itu juga dilakukan m...

  13. Psoriasis and autoimmune skin diseases

    Directory of Open Access Journals (Sweden)

    Poljački Mirjana N.

    2002-01-01

    Full Text Available Introduction Presuming that psoriasis is an autoimmune skin disease, the aim of this study was to establish its association with other autoimmune skin diseases. The material was obtained at the Dermatovenereological Clinic Clinical Center Novi Sad. Material and methods This 10-year retrospective study (1990-1999 included 1743 psoriasis patients. The control group consisted of 7492 nonpsoriatic dermatological patients. Results Association of psoriasis with other dermatological diseases of autoimmune nature has been established in 13 (0.74 % patients. The most frequent association was with lichen ruber planus in five patients, with alopecia areata and vitiligo in three patients, and in one with bullous pemphigoid and herpetiform dermatitis. Using Fisher's test no significant association was established. Discussion and conclusion According to literature data association of psoriasis with other autoimmune diseases is well known, but rare, which is in accordance with our results. The question arises whether this association is the matter of poor coexistence or the matter of genetic mutations. However, once established, these associations can further highlight the autoimmune nature of psoriasis. The research of autoimmunity would lead us to epithelial cells in thymus, and their badly learnt cognitive function about what is own, and what is not.

  14. Etiopathogenesis of hemolytic reactions in total artificial heart recipients.

    Science.gov (United States)

    Vasků, J; Urbánek, P

    1997-12-01

    Hemolysis in total artificial heart (TAH) recipients was analyzed. From a total of 66 long-term experiments lasting from 30-314 days performed in the Brno Research Center, in 53 animals, the total red blood cell (RBC) count, hematocrit, total hemoglobin, and free plasma hemoglobin were investigated. We could essentially divide the whole group of calves in 2 subgroups. The first subgroup was calves with hemolytic reactions, and the second subgroup was calves without any hemolytic reaction at all. In the first subgroup, hemolysis occurred in 47% of the overall number of animals during extracorporeal circulation (ECC), in 15% during ECC and later periodically during the experiment, in 8% during ECC and then continuously during the experiment, and finally in 10% not during ECC but repeatedly during the experiment. In 20% of the animals from the overall number, hemolysis did not occur at all (second subgroup). These results testify to the great individual differences within 1 breed (Bohemian with a substantial component of Holstein). These differences are further modified by exogenous and endogenous factors. First, the inborn resistance of the RBC membrane and also thrombi formation and the mineralization of the driving diaphragm are very important. The extreme situation of decreased RBC membrane resistance was proved using a calf from another breed, the slow growing Scottish Highland breed, which did not survive 22 days of pumping due to intractable lethal hemolysis. These factors are also indicated by the hemolytic action of some drugs (e.g., Dopegyt) used during the experiment for another reason. Also important are the mechanical forces of pumping, surface moieties of the biomaterial, mineralization of the driving diaphragms, thrombi formation, infection, etc. Essentially, the hemolytic reaction in the TAH recipient has a multifactorial character. Hemolysis is undoubtedly an important factor, which can have a profound impact on the length of survival. The

  15. Recurrent Hemolytic and Uremic Syndrome Induced by Escherichia Coli

    Science.gov (United States)

    Commereuc, Morgane; Weill, Francois-Xavier; Loukiadis, Estelle; Gouali, Malika; Gleizal, Audrey; Kormann, Raphaël; Ridel, Christophe; Frémeaux-Bacchi, Véronique; Rondeau, Eric; Hertig, Alexandre

    2016-01-01

    Abstract A widespread belief is that typical hemolytic and uremic syndrome (HUS) does not recur. We report the case of a patient infected twice with raw milk taken from his own cow and containing a Shiga toxin–producing Escherichia coli O174:H21 that induced recurrent HUS causing severe renal and cerebral disorders. A genomic comparison of the human and bovine Shiga toxin–producing Escherichia coli O174:H21 isolates revealed that they were identical. Typical HUS may recur. Since milk from this animal was occasionally distributed locally, thereby posing a serious threat for the whole village, this particular cow was destroyed. PMID:26735524

  16. Thrombotic microangiopathy: focus on atypical hemolytic uremic syndrome.

    Science.gov (United States)

    Sperati, C John; Moliterno, Alison R

    2015-06-01

    Thrombotic microangiopathies (TMA) such as atypical hemolytic uremic syndrome (aHUS) have evolved from rare, fulminant childhood afflictions to uncommon diseases with acute and chronic phases involving both children and adults. Breakthroughs in complement and coagulation regulation have allowed redefinition of specific entities despite substantial phenotypic mimicry. Reconciliation of phenotypes and delivery of life saving therapies require a multidisciplinary team of experts. The purpose of this review is to describe advances in the molecular pathophysiology of aHUS and to share the 2014 experience of the multidisciplinary Johns Hopkins TMA Registry in applying diagnostic assays, reporting disease associations, and genetic testing. PMID:26043391

  17. HEPARANASE AND AUTOIMMUNE DIABETES

    Directory of Open Access Journals (Sweden)

    Charmaine Joy Simeonovic

    2013-12-01

    Full Text Available Heparanase (Hpse is the only known mammalian endo-β-D-glucuronidase that degrades the glycosaminoglycan heparan sulfate (HS, found attached to the core proteins of heparan sulfate proteoglycans (HSPGs. Hpse plays a homeostatic role in regulating the turnover of cell-associated HS and also degrades extracellular HS in basement membranes (BMs and the extracellular matrix (ECM, where HSPGs function as a barrier to cell migration. Secreted Hpse is harnessed by leukocytes to facilitate their migration from the blood to sites of inflammation. In the non-obese diabetic (NOD model of autoimmune Type 1 diabetes (T1D, Hpse is also used by insulitis leukocytes to solubilize the islet BM to enable intra-islet entry of leukocytes and to degrade intracellular HS, an essential component for the survival of insulin-producing islet beta cells. Treatment of prediabetic adult NOD mice with the Hpse inhibitor PI-88 significantly reduced the incidence of T1D by ~50% and preserved islet HS. Hpse therefore acts as a novel immune effector mechanism in T1D. Our studies have identified T1D as a Hpse-dependent disease and Hpse inhibitors as novel therapeutics for preventing T1D progression and possibly the development of T1D vascular complications.

  18. Genetics Home Reference: X-linked sideroblastic anemia

    Science.gov (United States)

    ... Conditions X-linked sideroblastic anemia X-linked sideroblastic anemia Enable Javascript to view the expand/collapse boxes. ... Open All Close All Description X-linked sideroblastic anemia is an inherited disorder that prevents developing red ...

  19. Do You Know about Sickle Cell Anemia? (For Kids)

    Science.gov (United States)

    ... Lunch Recipes Do You Know About Sickle Cell Anemia? KidsHealth > For Kids > Do You Know About Sickle ... stay in the hospital. What Causes Sickle Cell Anemia? Sickle cell anemia is an inherited (say: in- ...

  20. Treatment of anemia with darbepoetin alfa in systolic heart failure

    DEFF Research Database (Denmark)

    Swedberg, Karl; Young, James B; Anand, Inder S;

    2013-01-01

    Patients with systolic heart failure and anemia have worse symptoms, functional capacity, and outcomes than those without anemia. We evaluated the effects of darbepoetin alfa on clinical outcomes in patients with systolic heart failure and anemia....

  1. Genetics Home Reference: X-linked sideroblastic anemia and ataxia

    Science.gov (United States)

    ... anemia and ataxia X-linked sideroblastic anemia and ataxia Enable Javascript to view the expand/collapse boxes. ... Close All Description X-linked sideroblastic anemia and ataxia is a rare condition characterized by a blood ...

  2. A rare presentation of hypopituitarism in hepatic overlap syndrome of autoimmune hepatitis and autoimmune cholangitis

    OpenAIRE

    Gupta V; Singh H.; Talapatra P; Ray S

    2016-01-01

    Autoimmune cholangitis is the antimitochondrial antibody-negative autoimmune hepatopathy with clinical and histological features similar to that of primary biliary cirrhosis. Autoimmune cholangitis has a predominant cholestatic phase. However, transaminasemia might be dominant in certain patients, indicating associated autoimmune hepatitis. Such an autoimmune hepatopathy has been termed as hepatic overlap syndrome. Due to the autoimmune nature of the disease, associated diseases of other orga...

  3. Proliferation and Apoptosis of Bone Marrow CD4~+ T Cells in Patients with Aplastic Anemia and Impacts of the Secreted Cytokines on Hematopoietic Stem Cells from Umbilical Cord Blood

    Institute of Scientific and Technical Information of China (English)

    郑邈; 孙汉英; 周剑峰; 徐慧珍; 黄丽芳; 刘文励

    2010-01-01

    Recent studies indicate that immune-associated aplastic anemia(AA)resembles such autoimmune diseases as insulin-dependent diabetes and chronic autoimmune thyroiditis that belong to organ-specific autoimmune diseases.Many independent investigation groups have successfully isolated the pathopoiesis-associated T cell clone causing hematopoiesis failure with a CD4 phenotype from peripheral blood and bone marrow(BM)in AA patients.In the current study,BM CD4+ T cells were isolated from AA patients and healthy con...

  4. The multiple autoimmune syndromes. A clue for the autoimmune tautology.

    Science.gov (United States)

    Anaya, Juan-Manuel; Castiblanco, John; Rojas-Villarraga, Adriana; Pineda-Tamayo, Ricardo; Levy, Roger A; Gómez-Puerta, José; Dias, Carlos; Mantilla, Ruben D; Gallo, Juan Esteban; Cervera, Ricard; Shoenfeld, Yehuda; Arcos-Burgos, Mauricio

    2012-12-01

    The multiple autoimmune syndromes (MAS) consist on the presence of three or more well-defined autoimmune diseases (ADs) in a single patient. The aim of this study was to analyze the clinical and genetic characteristics of a large series of patients with MAS. A cluster analysis and familial aggregation analysis of ADs was performed in 84 patients. A genome-wide microsatellite screen was performed in MAS families, and associated loci were investigated through the pedigree disequilibrium test. Systemic lupus erythematosus (SLE), autoimmune thyroid disease (AITD), and Sjögren's syndrome together were the most frequent ADs encountered. Three main clusters were established. Aggregation for type 1 diabetes, AITD, SLE, and all ADs as a trait was found. Eight loci associated with MAS were observed harboring autoimmunity genes. The MAS represent the best example of polyautoimmunity as well as the effect of a single genotype on diverse phenotypes. Its study provides important clues to elucidate the common mechanisms of ADs (i.e., autoimmune tautology).

  5. Listeria monocytogenes and hemolytic Listeria innocua in poultry.

    Science.gov (United States)

    Milillo, S R; Stout, J C; Hanning, I B; Clement, A; Fortes, E D; den Bakker, H C; Wiedmann, M; Ricke, S C

    2012-09-01

    Listeria monocytogenes is a ubiquitous, saprophytic, Gram-positive bacterium and occasional food-borne pathogen, often associated with ready-to-eat meat products. Because of the increased consumer interest in organic, all natural, and free range poultry products, it is important to understand L. monocytogenes in the context of such systems. Pasture-reared poultry were surveyed over the course of two 8-wk rearing periods. Cecal, soil, and grass samples were collected for Listeria isolation and characterization. Seven of 399 cecal samples (or 1.75%) were Listeria-positive. All positive cecal samples were obtained from broilers sampled at 2 wk of age. Grass and soil samples were collected from the pasture both before and after introduction of the poultry. Environmental samples collected after introduction of poultry were significantly more likely to contain Listeria (P Listeria, sigB allelic typing, and hlyA PCR tests found that both L. monocytogenes and L. innocua, including hemolytic L. innocua, were recovered from the cecal and environmental (grass/soil) samples. The sigB allelic typing also revealed that (1) positive samples could be composed of 2 or more allelic types; (2) allelic types found in cecal samples could also be found in the environment; and (3) allelic types could persist through the 2 rearing periods. Our data indicate that both pasture-reared poultry and their environment can be contaminated with L. monocytogenes and hemolytic L. innocua.

  6. Cobalt-doped nanohydroxyapatite: synthesis, characterization, antimicrobial and hemolytic studies

    Energy Technology Data Exchange (ETDEWEB)

    Tank, Kashmira P., E-mail: kashmira_physics@yahoo.co.in [Saurashtra University, Crystal Growth Laboratory, Physics Department (India); Chudasama, Kiran S.; Thaker, Vrinda S. [Saurashtra University, Bioscience Department (India); Joshi, Mihir J., E-mail: mshilp24@rediffmail.com [Saurashtra University, Crystal Growth Laboratory, Physics Department (India)

    2013-05-15

    Hydroxyapatite (Ca{sub 10}(PO{sub 4}){sub 6}(OH){sub 2}; HAP) is a major mineral component of the calcified tissues, and it has various applications in medicine and dentistry. In the present investigation, cobalt-doped hydroxyapatite (Co-HAP) nanoparticles were synthesized by surfactant-mediated approach and characterized by different techniques. The EDAX was carried out to estimate the amount of doping in Co-HAP. The transmission electron microscopy result suggested the transformation of morphology from needle shaped to spherical type on increasing the doping concentration. The powder XRD study indicated the formation of a new phase of brushite for higher concentration of cobalt. The average particle size and strain were calculated using Williamson-Hall analysis. The average particle size was found to be 30-60 nm. The FTIR study confirmed the presence of various functional groups in the samples. The antimicrobial activity was evaluated against four organisms Pseudomonas aeruginosa and Shigella flexneri as Gram negative as well as Micrococcus luteus and Staphylococcus aureus as Gram positive. The hemolytic test result suggested that all samples were non-hemolytic. The photoluminescence study was carried out to identify its possible applicability as a fluorescent probe.

  7. Hemolytic mechanism of dioscin proposed by molecular dynamics simulations.

    Science.gov (United States)

    Lin, Fu; Wang, Renxiao

    2010-01-01

    Saponins are a class of compounds containing a triterpenoid or steroid core with some attached carbohydrate modules. Many saponins cause hemolysis. However, the hemolytic mechanism of saponins at the molecular level is not yet fully understood. In an attempt to explore this issue, we have studied dioscin-a saponin with high hemolytic activity-through extensive molecular dynamics (MD) simulations. Firstly, all-atom MD simulations of 8 ns duration were conducted to study the stability of the dioscin-cholesterol complex and the cholesterol-cholesterol complex in water and in decane, respectively. MM-GB/SA computations indicate that the dioscin-cholesterol complex is energetically more favorable than the cholesterol-cholesterol complex in a non-polar environment. Next, several coarse-grained MD simulations of 400 ns duration were conducted to directly observe the distribution of multiple dioscin molecules on a DPPC-POPC-PSM-CHOL lipid bilayer. Our results indicate that dioscin can penetrate into the lipid bilayer, accumulate in the lipid raft micro-domain, and then bind cholesterol. This leads to the destabilization of lipid raft and consequent membrane curvature, which may eventually result in the hemolysis of red cells. This possible mechanism of hemolysis can well explain some experimental observations on hemolysis. PMID:19513766

  8. Case reports: delayed hemolytic transfusion reaction in sickle cell disease.

    Science.gov (United States)

    Syed, S K; Sears, D A; Werch, J B; Udden, M M; Milam, J D

    1996-10-01

    This article reports the details of delayed hemolytic transfusion reactions in four patients with sickle cell disease. These cases demonstrate the characteristics of the reactions, the significant risks involved, and the principles useful in diagnosis and treatment. Patients with sickle cell disease are at particular risk for delayed hemolytic transfusion reactions because they may be transfused at intervals over many years; they frequently form alloantibodies because of antigenic differences from the donor population; and they may receive emergency care in different hospitals where transfusion records are not available. In addition, exchange transfusions, which are often used for patients with sickle cell disease and which were given in three of these cases, raise the risks through increased exposure to foreign erythrocyte antigens and through an increased volume of erythrocytes susceptible to hemolysis. It was concluded that the hazards of these transfusion reactions justify preventive measures, such as extended erythrocyte phenotyping of patients with sickle cell disease and extended phenotypic matching of transfused cells. PMID:8853066

  9. Recent approaches for reducing hemolytic activity of chemotherapeutic agents.

    Science.gov (United States)

    Jeswani, Gunjan; Alexander, Amit; Saraf, Shailendra; Saraf, Swarnlata; Qureshi, Azra; Ajazuddin

    2015-08-10

    Drug induced hemolysis is a frequent complication associated with chemotherapy. It results from interaction of drug with erythrocyte membrane and leads to cell lysis. In recent past, various approaches were made to reduce drug-induced hemolysis, which includes drug polymer conjugation, drug delivery via colloidal carriers and hydrogels, co-administration of botanical agents and modification in molecular chemistry of drug molecules. The basic concept behind these strategies is to protect the red blood cells from membrane damaging effects of drugs. There are several examples of drug polymer conjugate that either are approved by Food and Drug Administration or are under clinical trial for delivering drugs with reduced toxicities. Likewise, colloidal carriers are also used successfully nowadays for the delivery of various chemotherapeutic agents like gemcitabine and amphotericin B with remarkable decrease in their hemolytic activity. Similarly, co-administration of botanical agents with drugs works as secondary system proving protection and strength to erythrocyte membranes. In addition to the above statement, interaction hindrance between RBC and drug molecule by molecular modification plays an important role in reducing hemolysis. This review predominantly describes the above recent approaches explored to achieve the reduced hemolytic activity of drugs especially chemotherapeutic agents. PMID:26047758

  10. Hemolytic uremic syndrome in Argentina: An attack scenario

    Directory of Open Access Journals (Sweden)

    Alcides Troncoso

    2013-08-01

    Full Text Available The recent Escherichia coli epidemic in Germany gave a lesson at an international level. There is no time to solve food security problems when an epidemic is on the way. The epidemic in Germany exposed the fissures in the control systems of the Federal Risk Evaluation Institute of this country, as well as showing the incompetency of health authorities, who had great difficulty in resolving the situation. To summarize, the possibility of prevention was confused with the utopian idea of non-occurrence. It was not less important the public’s recognition and the “awakening” of health ministers in the European Union as regards the proven fact that pathogenic and even lethal microorganisms may be present in the food we eat. Argentina has the highest incidence of hemolytic uremic syndrome in the world, and the next epidemic is likely not to occur in Germany, but in any other country, such as Argentina. In order to avoid complicity, we do not wish to remain silent about the situation in Argentina. Therefore, this is the writer’s motive for writing this article, which describes the scientific advances and the ethical pitfalls related to a disease transmitted by food, particularly hemolytic uremic syndrome, in Argentina.

  11. Hemolytic uremic syndrome in Argentina:An attack scenario

    Institute of Scientific and Technical Information of China (English)

    Alcides Troncoso

    2013-01-01

    The recent Escherichia coli epidemic in Germany gave a lesson at an international level. There is no time to solve food security problems when an epidemic is on the way. The epidemic in Germany exposed the fissures in the control systems of the Federal Risk Evaluation Institute of this country, as well as showing the incompetency of health authorities, who had great difficulty in resolving the situation. To summarize, the possibility of prevention was confused with the utopian idea of non-occurrence. It was not less important the public’s recognition and the “awakening” of health ministers in the European Union as regards the proven fact that pathogenic and even lethal microorganisms may be present in the food we eat. Argentina has the highest incidence of hemolytic uremic syndrome in the world, and the next epidemic is likely not to occur in Germany, but in any other country, such as Argentina. In order to avoid complicity, we do not wish to remain silent about the situation in Argentina. Therefore, this is the writer’s motive for writing this article, which describes the scientific advances and the ethical pitfalls related to a disease transmitted by food, particularly hemolytic uremic syndrome, in Argentina.

  12. AUTOIMMUNE EPIDERMAL BLISTERING DISEASES

    Directory of Open Access Journals (Sweden)

    Ana Maria Abreu Velez

    2013-11-01

    Full Text Available Autoimmune bullous skin diseases (ABDs are uncommon, potentially fatal diseases of skin and mucous membranes which are associated with deposits of autoantibodies and complement against distinct molecules of the epidermis and dermal/epidermal basement membrane zone (BMZ. These autoantibodies lead to a loss in skin molecular integrity, which manifests clinically as formation of blisters or erosions. In pemphigus vulgaris, loss of adhesion occurs within the epidermis. The pioneering work of Ernst H. Beutner, Ph.D. and Robert E. Jordon, M.D. confirmed the autoimmune nature of these diseases. Walter F. Lever, M.D. contributed significantly to our understanding of the histopathologic features of these diseases. Walter Lever, M.D. and Ken Hashimoto, M.D. contributed electron microscopic studies of these diseases, especially in pemphigus vulgaris and bullous pemphigoid. In bullous pemphigoid (BP, linear IgA bullous dermatosis, epidermolysis bullosa acquisita (EBA and dermatitis herpetiformis (DH, loss of adhesion takes place within or underneath the BMZ. Classic EBA demonstrates extensive skin fragility; DH is commonly associated with gluten-sensitive enteropathy, and manifests clinically with pruritic papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The clinical spectrum of bullous pemphigoid includes tense blisters, urticarial plaques, and prurigo-like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy, and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a „cluster of jewels”-like pattern in childhood (chronic bullous disease of childhood and is more clinically heterogeneous in adulthood. Many of the autoantigens in these disorders are known and have been well characterized. ABDs may be influenced by both genetic and exogenous factors. The diagnoses of

  13. Estrogens and autoimmune diseases.

    Science.gov (United States)

    Cutolo, Maurizio; Capellino, Silvia; Sulli, Alberto; Serioli, Bruno; Secchi, Maria Elena; Villaggio, Barbara; Straub, Rainer H

    2006-11-01

    Sex hormones are implicated in the immune response, with estrogens as enhancers at least of the humoral immunity and androgens and progesterone (and glucocorticoids) as natural immune-suppressors . Several physiological, pathological, and therapeutic conditions may change the serum estrogen milieu and/or peripheral conversion rate, including the menstrual cycle, pregnancy, postpartum period, menopause, being elderly, chronic stress, altered circadian rhythms, inflammatory cytokines, and use of corticosteroids, oral contraceptives, and steroid hormonal replacements, inducing altered androgen/estrogen ratios and related effects. In particular, cortisol and melatonin circadian rhythms are altered, at least in rheumatoid arthritis (RA), and partially involve sex hormone circadian synthesis and levels as well. Abnormal regulation of aromatase activity (i.e., increased activity) by inflammatory cytokine production (i.e., TNF-alpha, IL-1, and IL-6) may partially explain the abnormalities of peripheral estrogen synthesis in RA (i.e., increased availability of 17-beta estradiol and possible metabolites in synovial fluids) and in systemic lupus erythematosus, as well as the altered serum sex-hormone levels and ratio (i.e., decreased androgens and DHEAS). In the synovial fluids of RA patients, the increased estrogen concentration is observed in both sexes and is more specifically characterized by the hydroxylated forms, in particular 16alpha-hydroxyestrone, which is a mitogenic and cell proliferative endogenous hormone. Local effects of sex hormones in autoimmune rheumatic diseases seems to consist mainly in modulation of cell proliferation and cytokine production (i.e., TNF-alpha, Il-1, IL-12). In this respect, it is interesting that male patients with RA seem to profit more from anti-TNFalpha strategies than do female patients. PMID:17261796

  14. Celiac Disease and Autoimmune-Associated Conditions

    Directory of Open Access Journals (Sweden)

    Eugenia Lauret

    2013-01-01

    Full Text Available Celiac disease (CD is frequently accompanied by a variety of extradigestive manifestations, thus making it a systemic disease rather than a disease limited to the gastrointestinal tract. This is primarily explained by the fact that CD belongs to the group of autoimmune diseases. The only one with a known etiology is related to a permanent intolerance to gluten. Remarkable breakthroughs have been achieved in the last decades, due to a greater interest in the diagnosis of atypical and asymptomatic patients, which are more frequent in adults. The known presence of several associated diseases provides guidance in the search of oligosymptomatic cases as well as studies performed in relatives of patients with CD. The causes for the onset and manifestation of associated diseases are diverse; some share a similar genetic base, like type 1 diabetes mellitus (T1D; others share pathogenic mechanisms, and yet, others are of unknown nature. General practitioners and other specialists must remember that CD may debut with extraintestinal manifestations, and associated illnesses may appear both at the time of diagnosis and throughout the evolution of the disease. The implementation of a gluten-free diet (GFD improves the overall clinical course and influences the evolution of the associated diseases. In some cases, such as iron deficiency anemia, the GFD contributes to its disappearance. In other disorders, like T1D, this allows a better control of the disease. In several other complications and/or associated diseases, an adequate adherence to a GFD may slow down their evolution, especially if implemented during an early stage.

  15. Differential expression of the cytotoxic and hemolytic activities of the ApxIIA toxin from Actinobacillus pleuropneumoniae.

    OpenAIRE

    Tu, A H; Hausler, C; Young, R.; Struck, D K

    1994-01-01

    The ApxIIA protein secreted from Actinobacillus pleuropneumoniae is both hemolytic and cytotoxic. However, when the cloned apxII operon is expressed in Escherichia coli, two forms of the ApxIIA protein can be recovered. Toxin which remains intracellular has hemolytic and cytotoxic activities, while toxin that is secreted is cytotoxic with little or no hemolytic activity. This indicates that the cytotoxicity of ApxIIA is independent of its hemolytic activity.

  16. Autoimmune hepatitis in association with lymphocytic colitis.

    LENUS (Irish Health Repository)

    Cronin, Edmond M

    2012-02-03

    Autoimmune hepatitis is a rare, chronic inflammatory disorder which has been associated with a number of other auto-immune conditions. However, there are no reports in the medical literature of an association with microscopic (lymphocytic) colitis. We report the case of a 53-year-old woman with several autoimmune conditions, including lymphocytic colitis, who presented with an acute hepatitis. On the basis of the clinical features, serology, and histopathology, we diagnosed autoimmune hepatitis. To our knowledge, this is the first report of autoimmune hepatitis in association with lymphocytic colitis, and lends support to the theory of an autoimmune etiology for lymphocytic colitis.

  17. Colonic lymphangiomatosis associated with anemia

    Institute of Scientific and Technical Information of China (English)

    Woo Chul Chung; Hye-Kang Kim; Jin Young Yoo; Jeong Rok Lee; Kang-Moon Lee; Chang Nyol Paik; U-Im Jang; Jin Mo Yang

    2008-01-01

    lymphangioma is an uncommon malformation of lymphatic system.Multiple colonic lymphangioma named as lymphangiomatosis is considered an extremely rare disease.Although lymphangioma is a benign tumor and most colonic lymphangiomas do not cause symptoms and do not require treatment,resection of lymphangioma is necessary in the presence of symptoms such as abdominal pain,bleeding,intussusceptions.We report a case of colonic lymphangiomatosis in a man who presented with abdominal discomfort and anemia,which was diagnosed and treated with endoscopic snare polyperctomy.

  18. Therapeutic apheresis in autoimmune diseases

    Directory of Open Access Journals (Sweden)

    Bambauer R

    2013-11-01

    Full Text Available Rolf Bambauer,1 Reinhard Latza,2 Carolin Bambauer,3 Daniel Burgard,4 Ralf Schiel5 1Institute for Blood Purification, Homburg, 2Laboratorium of Medicine, St Ingbert, 3Main Hospital Darmstadt, Darmstadt, 4Herz Zentrum, Cardiology, Völklingen, 5Inselklinik Heringsdorf GmbH, Seeheilbad Heringsdorf, Germany Abstract: Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a bad prognosis without treatment. Therapeutic apheresis in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 35 years. Here we provide an overview of the most important pathogenic aspects indicating that therapeutic apheresis can be a supportive therapy in some systemic autoimmune diseases, such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, and inflammatory eye disease. With the introduction of novel and effective biologic agents, therapeutic apheresis is indicated only in severe cases, such as in rapid progression despite immunosuppressive therapy and/or biologic agents, and in patients with renal involvement, acute generalized vasculitis, thrombocytopenia, leucopenia, pulmonary, cardiac, or cerebral involvement. In mild forms of autoimmune disease, treatment with immunosuppressive therapies and/or biologic agents seems to be sufficient. The prognosis of autoimmune diseases with varying organ manifestations has improved considerably in recent years, due in part to very aggressive therapy schemes. Keywords: therapeutic apheresis, autoimmune diseases, systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, inflammatory eye disease

  19. Anemia of Inflammation and Chronic Disease

    Science.gov (United States)

    ... 699–710. 4 Anemia of Inflammation and Chronic Disease Eating, Diet, and Nutrition People with anemia caused by ... Phone: 202–776–0544 Fax: 202–776–0545 Internet: www. hematology. org Iron Disorders Institute P.O. Box 675 Taylors, SC 29687 ...

  20. The Student with Sickle Cell Anemia.

    Science.gov (United States)

    Tetrault, Sylvia M.

    1981-01-01

    Sickle cell anemia is the most common and severe of inherited chronic blood disorders. In the United States, sickle cell anemia is most common among the Black population. Among the most commonly occurring symptoms are: an enlarged spleen, episodes of severe pain, easily contracted infections, skin ulcers, and frequent urination. (JN)

  1. Anemia and survival in human immunodeficiency virus

    DEFF Research Database (Denmark)

    Lundgren, Jens Dilling; Mocroft, Amanda

    2003-01-01

    The prospective, multicenter cohort study EuroSIDA has previously reported on predictors and outcomes of anemia in patients infected with human immunodeficiency virus. In a Cox proportional-hazards model with serial measures of CD4+ cell count, plasma viral load, and degrees of anemia fitted...

  2. 9 CFR 311.34 - Anemia.

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Anemia. 311.34 Section 311.34 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE, DEPARTMENT OF AGRICULTURE AGENCY ORGANIZATION... CERTIFICATION DISPOSAL OF DISEASED OR OTHERWISE ADULTERATED CARCASSES AND PARTS § 311.34 Anemia. Carcasses...

  3. Silent Infarcts with Sickle Cell Anemia

    OpenAIRE

    J Gordon Millichap

    2002-01-01

    The effect of transfusion therapy on the risk for new silent infarct or stroke in children with sickle cell anemia and abnormal transcranial Doppler (TCD) ultrasonography was determined at the University of Miami, FL, and other centers in the STOP trial (Stroke Prevention in Sickle Cell Anemia).

  4. Unexplained Aspects of Anemia of Inflammation

    Directory of Open Access Journals (Sweden)

    Elizabeth A. Price

    2010-01-01

    Full Text Available Anemia of inflammation (AI, also known as anemia of chronic inflammation or anemia of chronic disease was described over 50 years ago as anemia in association with clinically overt inflammatory disease, and the findings of low plasma iron, decreased bone marrow sideroblasts and increased reticuloendothelial iron. Pathogenic features underlying AI include a mild shortening of red cell survival, impaired erythropoietin production, blunted responsiveness of the marrow to erythropoietin, and impaired iron metabolism mediated by inflammatory cytokines and the iron regulatory peptide, hepcidin. Despite marked recent advances in understanding AI, gaps remain, including understanding of the pathogenesis of AI associated with “noninflammatory” or mildly inflammatory diseases, the challenge of excluding iron deficiency anemia in the context of concomitant inflammation, and understanding more precisely the contributory role of hepcidin in the development of AI in human inflammatory diseases.

  5. Autoimmune pancreatitis. An update

    International Nuclear Information System (INIS)

    Autoimmune pancreatitis (AIP) is a rare disease, the pathophysiological understanding of which has been greatly improved over the last years. The most common form, type 1 AIP belongs to the IgG4-related diseases and must be distinguished from type 2 AIP, which is a much rarer entity associated with chronic inflammatory bowel disease. Clinically, there is an overlap with pancreatic cancer. Imaging and further criteria, such as serological and histological parameters are utilized for a differentiation between both entities in order to select the appropriate therapy and to avoid the small but ultimately unnecessary number of pancreatectomies. The diagnostics of AIP are complex, whereby the consensus criteria of the International Association of Pancreatology have become accepted as the parameters for discrimination. These encompass five cardinal criteria and one therapeutic criterion. By applying these criteria AIP can be diagnosed with a sensitivity of 84.9 %, a specificity of 100 % and an accuracy of 93.8 %. The diagnosis of AIP is accomplished by applying several parameters of which two relate to imaging. As for the routine diagnostics of the pancreas these are ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI). Important for the differential diagnosis is the exclusion of signs of local and remote tumor spread for which CT and MRI are established. The essential diagnostic parameter of histology necessitates sufficient sample material, which cannot usually be acquired by a fine needle biopsy. CT or MRI are the reference standard methods for identification of the optimal puncture site and imaging-assisted (TruCut) biopsy. In patients presenting with unspecific upper abdominal pain, painless jaundice combined with the suspicion of a pancreatic malignancy in imaging but a mismatch of secondary signs of malignancy, AIP should also be considered as a differential diagnosis. As the diagnosis of AIP only partially relies on imaging radiologists also

  6. Type 1 autoimmune pancreatitis

    Directory of Open Access Journals (Sweden)

    Zen Yoh

    2011-12-01

    Full Text Available Abstract Before the concept of autoimmune pancreatitis (AIP was established, this form of pancreatitis had been recognized as lymphoplasmacytic sclerosing pancreatitis or non-alcoholic duct destructive chronic pancreatitis based on unique histological features. With the discovery in 2001 that serum IgG4 concentrations are specifically elevated in AIP patients, this emerging entity has been more widely accepted. Classical cases of AIP are now called type 1 as another distinct subtype (type 2 AIP has been identified. Type 1 AIP, which accounts for 2% of chronic pancreatitis cases, predominantly affects adult males. Patients usually present with obstructive jaundice due to enlargement of the pancreatic head or thickening of the lower bile duct wall. Pancreatic cancer is the leading differential diagnosis for which serological, imaging, and histological examinations need to be considered. Serologically, an elevated level of IgG4 is the most sensitive and specific finding. Imaging features include irregular narrowing of the pancreatic duct, diffuse or focal enlargement of the pancreas, a peri-pancreatic capsule-like rim, and enhancement at the late phase of contrast-enhanced images. Biopsy or surgical specimens show diffuse lymphoplasmacytic infiltration containing many IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. A dramatic response to steroid therapy is another characteristic, and serological or radiological effects are normally identified within the first 2 or 3 weeks. Type 1 AIP is estimated as a pancreatic manifestation of systemic IgG4-related disease based on the fact that synchronous or metachronous lesions can develop in multiple organs (e.g. bile duct, salivary/lacrimal glands, retroperitoneum, artery, lung, and kidney and those lesions are histologically identical irrespective of the organ of origin. Several potential autoantigens have been identified so far. A Th2-dominant immune reaction and the activation of

  7. [Infectious agents and autoimmune diseases].

    Science.gov (United States)

    Riebeling-Navarro, C; Madrid-Marina, V; Camarena-Medellín, B E; Peralta-Zaragoza, O; Barrera, R

    1992-01-01

    In this paper the molecular aspects of the relationships between infectious agents and autoimmune diseases, the mechanisms of immune response to infectious agents, and the more recent hypotheses regarding the cause of autoimmune diseases are discussed. The antigens are processed and selected by their immunogenicity, and presented by HLA molecules to the T cell receptor. These events initiate the immune response with the activation and proliferation of T-lymphocytes. Although there are several hypotheses regarding the cause of autoimmune diseases and too many findings against and in favor of them, there is still no conclusive data. All these hypothesis and findings are discussed in the context of the more recent advances. PMID:1615352

  8. PD-1, gender, and autoimmunity

    Science.gov (United States)

    Dinesh, Ravi K.; Hahn, Bevra H.; Singh, Ram Pyare

    2010-01-01

    Programmed death 1 (PD-1) and its ligands (PD-L1 and PD-L2) are responsible for inhibitory T cell signaling that helps mediate the mechanisms of tolerance and immune homeostasis. The PD-1:PD-L signaling pathway has been shown to play an important role in a variety of diseases, including autoimmune conditions, chronic infection, and cancer. Recently, investigators have explored the role of sex hormones in modulating the pathway in autoimmune conditions. Exploring the effects of sex hormones on the PD-1:PD-L pathway could shed light on the gender biased nature of many autoimmune conditions as well as aide in the development of therapeutics targeting the immune system. PMID:20433954

  9. Autoimmunity in chronic lymphocytic leukaemia.

    Science.gov (United States)

    Lischner, M; Prokocimer, M; Zolberg, A; Shaklai, M

    1988-08-01

    Seventy-nine patients with chronic lymphocytic leukaemia were evaluated for the presence of autoimmune diseases and autoantibodies. One patient has polymyositis and two additional patients presented with features suggestive of pernicious anaemia and chronic active hepatitis. The Coombs' direct test was positive in 7% and immune thrombocytopenia was present in 8.1% of patients. Five (7%) patients had M-protein in the serum. No increased frequency of other autoantibodies was noted in our study group. We conclude that the propensity to develop antibodies is restricted only to the haematopoietic system and that there is no increased frequency of non-haematological autoimmune diseases in chronic lymphatic leukaemia. PMID:3249703

  10. Coeliac disease with autoimmune haemolytic anaemia.

    OpenAIRE

    Miller, D. G.

    1984-01-01

    Two patients are described who have developed autoimmune haemolytic anaemia in association with their coeliac disease. Autoimmune haemolytic anaemia may represent an extension of immunological disorders linked with coeliac disease, centred on the histocompatibility antigen B8.

  11. Anemia of Chronic Disease and Iron Deficiency Anemia in Inflammatory Bowel Diseases: Pathophysiology, Diagnosis, and Treatment.

    Science.gov (United States)

    Murawska, Natalia; Fabisiak, Adam; Fichna, Jakub

    2016-05-01

    Anemia coexists with inflammatory bowel disease (IBD) in up to two-thirds of patients, significantly impairing quality of life. The most common types of anemia in patients with IBD are iron deficiency anemia and anemia of chronic disease, which often overlap. In most cases, available laboratory tests allow successful diagnosis of iron deficiency, where difficulties appear, recently established indices such as soluble transferrin-ferritin ratio or percentage of hypochromic red cells are used. In this review, we discuss the management of the most common types of anemia in respect of the latest available data. Thus, we provide the mechanisms underlying pathophysiology of these entities; furthermore, we discuss the role of hepcidin in developing anemia in IBD. Next, we present the treatment options for each type of anemia and highlight the importance of individual choice of action. We also focus on newly developed intravenous iron preparations and novel, promising drug candidates targeting hepcidin. Concurrently, we talk about difficulties in differentiating between the true and functional iron deficiency, and discuss tools facilitating the process. Finally, we emphasize the importance of proper diagnosis and treatment of anemia in IBD. We conclude that management of anemia in patients with IBD is tricky, and appropriate screening of patients regarding anemia is substantial. PMID:26818422

  12. New Codanin-1 Gene Mutations in a Italian Patient with Congenital Dyserythropoietic Anemia Type I and Heterozygous Beta-Thalassemia.

    Science.gov (United States)

    D'Alcamo, Elena; Agrigento, V; Pitrolo, L; Sclafani, S; Barone, R; Calvaruso, G; Buffa, V; Maggio, A

    2016-06-01

    Congenital dyserythropoietic anemia type I is an autosomal recessive disorder associated with macrocytic anemia, ineffective erythropoiesis, iron overloading and characterized by abnormal chromatin ultrastructure in erythroblasts such as internuclear chromatin bridges, spongy heterochromatin and invagination of the nuclear membrane. A 58-year-old Causasian man with chronic hemolytic anemia, heterozygous for β (+) -globin IVS1, nt110 G>A mutation (causing abnormal alpha:beta globin chain ratio) showed clinical, laboratory and hematological features suggesting diagnosis of CDA1. Sequence analysis of CDA-related genes revealed compound heterozygosity for two novel mutations in the CDAN1 gene: a frameshift mutation 3367 del 4 (TTAG) in exon 25 and a missense mutation c.1811 G>T in exon 11 causing an aminoacid change from glycine to valine at codon 565 (G565V). One of the propositus' brothers showed the same gene mutations. As the CDA1 can mimic thalassemia, a frequent misdiagnosis is possible especially in countries where the prevalence of thalassemia is high. A strong clinical suspicion in patients who do not reveal a clear genetic basis for presumed thalassemia may help clinch the correct diagnosis. PMID:27408412

  13. Molecular Basis for Group B β -hemolytic Streptococcal Disease

    Science.gov (United States)

    Hellerqvist, Carl G.; Sundell, Hakan; Gettins, Peter

    1987-01-01

    Group B β -hemolytic Streptococcus (GBS) is a major pathogen affecting newborns. We have investigated the molecular mechanism underlying the respiratory distress induced in sheep after intravenous injection of a toxin produced by this organism. The pathophysiological response is characterized by pulmonary hypertension, followed by granulocytopenia and increased pulmonary vascular permeability to protein. 31P NMR studies of GBS toxin and model components before and after reductive alkaline hydrolysis demonstrated that phosphodiester residues are an integral part of the GBS toxin. Reductive alkaline treatment cleaves phosphate esters from secondary and primary alcohols and renders GBS toxin nontoxic in the sheep model and inactive as a mediator of elastase release in vitro from isolated human granulocytes. We propose that the interaction of cellular receptors with mannosyl phosphodiester groups plays an essential role in the pathophysiological response to GBS toxin.

  14. Hemolytic uremic syndrome: pathogenesis and update of interventions.

    Science.gov (United States)

    Palermo, Marina S; Exeni, Ramón A; Fernández, Gabriela C

    2009-08-01

    The typical form of hemolytic uremic syndrome (HUS) is the major complication of Shiga toxin-producing Escherichia coli infections. HUS is a critical health problem in Argentina since it is the main cause of acute renal failure in children and the second cause of chronic renal failure, accounting for 20% of renal transplants in children and adolescents in Argentina. Despite extensive research in the field, the mainstay of treatment for patients with HUS is supportive therapy, and there are no specific therapies preventing or ameliorating the disease course. In this review, we present the current knowledge about pathogenic mechanisms and discuss traditional and innovative therapeutic approaches, with special focus in Argentinean contribution. The hope that a better understanding of transmission dynamics and pathogenesis of this disease will produce better therapies to prevent the acute mortality and the long-term morbidity of HUS is the driving force for intensified research.

  15. Anemia

    Science.gov (United States)

    ... not have enough hemoglobin. The body needs certain vitamins, minerals, and nutrients to make enough red blood cells. ... of iron, vitamin B12, folic acid, and other vitamins and minerals Red blood count and hemoglobin level Reticulocyte count ...

  16. Anemia

    Science.gov (United States)

    ... B-12 (meat and dairy), and folic acid (citrus juices, dark green leafy vegetables, legumes, and fortified ... ASH Meeting on Lymphoma Biology ASH Workshop on Genome Editing Publications Blood The Hematologist ASH Clinical News ...

  17. Anemia

    Science.gov (United States)

    ... leafy green vegetables, fruits, and dried beans and peas. Folic acid is found in fortified breads, pastas, ... Changes in skin color, making it look gray, yellow or bronze (not caused by sun) Treatment depends ...

  18. Autoimmune Skin Diseases in the Dog

    OpenAIRE

    Parker, W M

    1981-01-01

    Diagnoses of autoimmune skin diseases require very careful observation of the skin lesions, and selection of an intact vesicle for histopathological examination. If available, immunofluorescent studies can be very useful in confirming the diagnosis of autoimmune skin disease. Seven autoimmune skin diseases are briefly reviewed. Therapy must be aggressive and owner warned of the guarded prognosis.

  19. 224例住院婴幼儿贫血病因分析%Analysis of the Cause of Anemia in 224 Cases of Infants and Young Children

    Institute of Scientific and Technical Information of China (English)

    杨俊杰

    2015-01-01

    Objective To study the hospitalized infants and young children crowd in children with anemia, provide theoretical basis for the prevention and treatment of anemia in infants. Methods On 2010.8.1~2010.8.30 hospitalized in our department infant anemia investigation, to find the cause and fol ow-up. Results A total of 224 cases of infants and young children to complete fol ow-up. The most common etiology anemia is iron deficiency disease (69.4%), but 89.3% of them are mild, hemolytic anemia take second place, with favism is given priority to, and send out the Mediter anean anemia; Because of the repeated infection or third of anemia with other diseases. Conclusion Iron deficiency anemia is stil the infant anemia main reason, it is necessary to further strengthen the anemia prevention and control work.%目的探讨住院患儿中婴幼儿人群贫血原因,为防治婴幼儿贫血提供理论依据。方法对2010年8月1日~2013年8月30日在我科住院婴幼儿进行贫血调查,查找病因并进行随访。结果共224例婴幼儿完成随访。贫血病因以缺铁性病最常见(69.4%),但其中89.3%均为轻度表现,溶血性贫血次之,以蚕豆病为主,并有散发地中海贫血;因反复感染或伴随其它疾病的贫血占第3位。结论缺铁性贫血仍是婴幼儿贫血主要原因,有必要进一步加强对贫血防治工作。

  20. 224例住院婴幼儿贫血病因分析%Analysis of the Cause of Anemia in 224 Cases of Infants and Young Children

    Institute of Scientific and Technical Information of China (English)

    杨俊杰

    2015-01-01

    目的探讨住院患儿中婴幼儿人群贫血原因,为防治婴幼儿贫血提供理论依据。方法对2010年8月1日~2013年8月30日在我科住院婴幼儿进行贫血调查,查找病因并进行随访。结果共224例婴幼儿完成随访。贫血病因以缺铁性病最常见(69.4%),但其中89.3%均为轻度表现,溶血性贫血次之,以蚕豆病为主,并有散发地中海贫血;因反复感染或伴随其它疾病的贫血占第3位。结论缺铁性贫血仍是婴幼儿贫血主要原因,有必要进一步加强对贫血防治工作。%Objective To study the hospitalized infants and young children crowd in children with anemia, provide theoretical basis for the prevention and treatment of anemia in infants. Methods On 2010.8.1~2010.8.30 hospitalized in our department infant anemia investigation, to find the cause and fol ow-up. Results A total of 224 cases of infants and young children to complete fol ow-up. The most common etiology anemia is iron deficiency disease (69.4%), but 89.3% of them are mild, hemolytic anemia take second place, with favism is given priority to, and send out the Mediter anean anemia; Because of the repeated infection or third of anemia with other diseases. Conclusion Iron deficiency anemia is stil the infant anemia main reason, it is necessary to further strengthen the anemia prevention and control work.

  1. The cardio-renal anemia syndrome

    Directory of Open Access Journals (Sweden)

    Dimković Siniša

    2007-01-01

    Full Text Available Introduction. The problem of anemia in congestive heart failure and chronic kidney disease was thought to be insignificant for a long period of time. Recent investigations pointed out that the problem of anemia should be defined in the context of the cardio-renal anemia syndrome. A positive feedback mechanism indicates that cardio-renal anemia syndrome is due to an interaction between congestive heart failure, chronic renal failure and anemia. The aim of the study was to present the possible pathophysiological mechanisms of this syndrome, epidemiological characteristics and therapeutic results of the former investigations. Results. The results of the retrospective and prospective controlled trails have shown that management of anemia with subcutaneous administration of recombinant human erythropoietin together with intravenous iron infusion for at least 3-6 months lead to: relief of symptoms (improved NYHA functional class; increased left ventricular ejection fraction; reduced cardiovascular morbidity and mortality; reduced number of rehospitalizations; reduced requirements for usual therapeutic agents (especially diuretics; and improved renal function. Conclusion. In patients with heart and kidney disease anemia should be routinely identified and appropriately treated. Subcutaneous recombinant erythropoietin and intravenous iron may significantly improve overall survival and quality of life of these patients. .

  2. Anemia in the Neonate: The Differential Diagnosis and Treatment.

    Science.gov (United States)

    Nassin, Michele L; Lapping-Carr, Gabrielle; de Jong, Jill L O

    2015-07-01

    Anemia is a common problem in the neonatal period. Presenting symptoms may suggest numerous possible diagnoses ranging from anemia seen as a normal part of development to anemia due to critical pathology. An illustrative case is presented to highlight the appropriate evaluation of the neonate with significant anemia. Several important features of the evaluation of neonatal anemia are highlighted. The constellation of signs and symptoms that occur in conjunction with the anemia are critical for the evaluation. The evaluation should be performed in a step-wise process that starts by eliminating common causes of anemia. Manual review of the peripheral blood smear with a hematologist can be helpful. PMID:26171704

  3. Pancreatic Tuberculosis or Autoimmune Pancreatitis

    OpenAIRE

    Ayesha Salahuddin; Muhammad Wasif Saif

    2014-01-01

    Introduction. Isolated pancreatic and peripancreatic tuberculosis is a challenging diagnosis due to its rarity and variable presentation. Pancreatic tuberculosis can mimic pancreatic carcinoma. Similarly, autoimmune pancreatitis can appear as a focal lesion resembling pancreatic malignancy. Endoscopic ultrasound-guided fine needle aspiration provides an effective tool for differentiating between benign and malignant pancreatic lesions. The immune processes involved in immunoglobulin G4 relate...

  4. Autoimmune Epilepsy Guidelines for Diagnosis

    OpenAIRE

    J Gordon Millichap

    2013-01-01

    Investigators at the Children’s Hospital at Westmead, University of Sydney, Australia, and John Radcliffe Hospital, Oxford, UK, describe 13 children (11 female; mean age 6 years, range 1-13 years) seen over a period of 3.5 years with suspected autoimmune epilepsy.

  5. Autoimmune Epilepsy Guidelines for Diagnosis

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2013-08-01

    Full Text Available Investigators at the Children’s Hospital at Westmead, University of Sydney, Australia, and John Radcliffe Hospital, Oxford, UK, describe 13 children (11 female; mean age 6 years, range 1-13 years seen over a period of 3.5 years with suspected autoimmune epilepsy.

  6. Epilepsy as an Autoimmune Disease

    OpenAIRE

    J Gordon Millichap; John J Millichap

    2014-01-01

    Investigators at University of New South Wales, Sydney, Australia, and Boston Children's Hospital, Harvard Medical School, conducted a retrospective population-level study of the relationship between epilepsy and 12 common autoimmune diseases: type 1 diabetes mellitus, psoriasis, rheumatoid arthritis, Graves disease, Hashimoto thyroiditis, Crohn disease, ulcerative colitis, systemic lupus erythematosus, antiphospholipid syndrome, Sjogren syndrome, myasthenia gravis, and celiac ...

  7. American Autoimmune Related Diseases Association

    Science.gov (United States)

    ... to navigate the health-care system in the age of the Affordable Care Act. News in the world of AARDA’s Grassroots ... was the “Status of Autoimmune Disease in the Age of the Affordable Care Act. Watch the briefing video View the power ...

  8. Therapeutic apheresis in autoimmune diseases

    Science.gov (United States)

    Bambauer, Rolf; Latza, Reinhard; Bambauer, Carolin; Burgard, Daniel; Schiel, Ralf

    2013-01-01

    Systemic autoimmune diseases based on an immune pathogenesis produce autoantibodies and circulating immune complexes, which cause inflammation in the tissues of various organs. In most cases, these diseases have a bad prognosis without treatment. Therapeutic apheresis in combination with immunosuppressive therapies has led to a steady increase in survival rates over the last 35 years. Here we provide an overview of the most important pathogenic aspects indicating that therapeutic apheresis can be a supportive therapy in some systemic autoimmune diseases, such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, and inflammatory eye disease. With the introduction of novel and effective biologic agents, therapeutic apheresis is indicated only in severe cases, such as in rapid progression despite immunosuppressive therapy and/or biologic agents, and in patients with renal involvement, acute generalized vasculitis, thrombocytopenia, leucopenia, pulmonary, cardiac, or cerebral involvement. In mild forms of autoimmune disease, treatment with immunosuppressive therapies and/or biologic agents seems to be sufficient. The prognosis of autoimmune diseases with varying organ manifestations has improved considerably in recent years, due in part to very aggressive therapy schemes.

  9. An autosomal locus causing autoimmune disease: Autoimmune polyglandular disease type I assigned to chromosome 21

    OpenAIRE

    Aaltonen, Johanna; Björses, Petra; Sandkuijl, Lodewijk; Perheentupa, Jaakko; Peltonen, Leena Johanna

    1994-01-01

    textabstractAutoimmune polyglandular disease type I (APECED) is an autosomal recessive autoimmune disease characterized by a variable combination of the failure of the endocrine glands. The pathogenesis of this unique autoimmune disease is unknown; unlike many other autoimmune diseases, APECED does not show association to specific HLA haplotypes. Unravelling the APECED locus will identify a novel gene outside the HLA loci influencing the outcome of autoimmune diseases. We have assigned the di...

  10. Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

    Directory of Open Access Journals (Sweden)

    Clarisse Lopes de Castro Lobo

    Full Text Available This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had significantly higher lactate dehydrogenase (LDH levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr > 1.0 mg/dL was lower than the group with Cr ≤ 1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly correlated with hemoglobin suggesting that the TRJV values were not correlated with the hemolytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.

  11. Correlates of anemia in pregnant women

    Directory of Open Access Journals (Sweden)

    Ranjana Singh

    2015-09-01

    Full Text Available Background: Anemia during pregnancy is a global public health challenge facing the world today. Prevalence of anemia in pregnancy in all the age groups is higher in India as compared to other developing countries. Objective: This study is aimed at determining the magnitude and to explore the socio-demographic and other correlates of anemia among pregnant women. Methodology: This descriptive study with cross-sectional design was conducted in a tertiary care hospital. Pregnant who were attending antenatal clinic for a period of one year were comprised the study material. Correlation between variables was analyzed using the chi-square and odd ratio. Results: Three hundred and thirty eight pregnant women were registered for the present study, whose age ranged from 16 to 45 years with a mean age of 26.08 years. Majority (81.95% participants were found to be anemic. It was observed that anemia was more prevalent in pregnant women age groups i.e. 25-29 years and 30+years i.e. 86.67% and 86.21% respectively. Anemia was 82.92% in women were belonging to Hindu and others religion and 82.24% in women having vegetarian diet. Maximum prevalence (83.93% of anemia was observed in women who were booked for antenatal care in the 3rd trimester of pregnancy. The prevalence of anemia is higher (>85% in women having parity two or more, but this association was not statistically significant. Very few (6.21% were found to be severely anemic as compared to women who were moderately anemic (43.19%. Multiple logistic regression analysis of these factors showed that possibility of anemia is less in women who belong to rural area and it is highly significant. Analysis further showed significant association between anemia and type of diet and other factors like women having parity 1 and 4. Conclusion: The prevalence of anemia amongst the pregnant participants was very high. The socio-demographic and obstetrics factors were found to be associated with anemia. To prevent

  12. Syngeneic transplantation in aplastic anemia

    DEFF Research Database (Denmark)

    Gerull, Sabine; Stern, Martin; Apperley, Jane;

    2013-01-01

    . About half of transplants with data available (39 of 86) were followed by posttransplant immunosuppression. Graft source was bone marrow in the majority of cases (n=77). Transplant practice changed over time with more transplants with conditioning and anti-thymocyte globulin as well as peripheral blood...... stem cells performed in later years. Ten year overall survival was 93% with 5 transplant-related deaths. Graft failure occurred in 32% of transplants. Risk of graft failure was significantly increased in transplants without conditioning, and with bone marrow as graft source. Lack of posttransplant...... a retrospective analysis of all syngeneic transplants for aplastic anemia reported to the European Group for Blood and Marrow Transplantation. Between 1976 and 2009, 88 patients received 113 transplants. Most transplants (n=85) were preceded by a conditioning regimen, 22 of these including anti-thymocyte globulin...

  13. Characterization of a New DGKE Intronic Mutation in Genetically Unsolved Cases of Familial Atypical Hemolytic Uremic Syndrome

    Science.gov (United States)

    Mele, Caterina; Lemaire, Mathieu; Iatropoulos, Paraskevas; Piras, Rossella; Bresin, Elena; Bettoni, Serena; Bick, David; Helbling, Daniel; Veith, Regan; Valoti, Elisabetta; Donadelli, Roberta; Murer, Luisa; Neunhäuserer, Maria; Breno, Matteo; Frémeaux-Bacchi, Véronique; Lifton, Richard; Noris, Marina

    2015-01-01

    Background and objectives Genetic and acquired abnormalities causing dysregulation of the complement alternative pathway contribute to atypical hemolytic uremic syndrome (aHUS), a rare disorder characterized by thrombocytopenia, nonimmune microangiopathic hemolytic anemia, and acute kidney failure. However, in a substantial proportion of patients the disease-associated alterations are still unknown. Design, setting, participants, & measurements Whole-exome and whole-genome sequencing were performed in two unrelated families with infantile recessive aHUS. Sequencing of cDNA from affected individuals was used to test for the presence of aberrant mRNA species. Expression of mutant diacylglycerol kinase epsilon (DGKE) protein was evaluated with western blotting. Results Whole-exome sequencing analysis with conventional variant filtering parameters did not reveal any obvious candidate mutation in the first family. The report of aHUS-associated mutations in DGKE, encoding DGKE, led to re-examination of the noncoding DGKE variants obtained from next-generation sequencing, allowing identification of a novel intronic DGKE mutation (c.888+40A>G) that segregated with disease. Sequencing of cDNA from affected individuals revealed aberrant forms of DGKE mRNA predicted to cause profound abnormalities in the protein catalytic site. By whole-genome sequencing, the same mutation was found in compound heterozygosity with a second nonsense DGKE mutation in all affected siblings of another unrelated family. Homozygous and compound heterozygous patients presented similar clinical features, including aHUS presentation in the first year of life, multiple relapsing episodes, and proteinuria, which are prototypical of DGKE-associated aHUS. Conclusions This is the first report of a mutation located beyond the exon-intron boundaries in aHUS. Intronic mutations such as these are underreported because conventional filtering parameters used to process next-generation sequencing data routinely

  14. Alleviating anemia and thrombocytopenia in myelofibrosis patients.

    Science.gov (United States)

    Cervantes, Francisco; Correa, Juan-Gonzalo; Hernandez-Boluda, Juan Carlos

    2016-05-01

    Anemia and thrombocytopenia are frequent clinical manifestations of myelofibrosis as well as important prognostic factors of the disease. Concerning the treatment of anemia, the first step should be the correction of reversible contributing factors, such as possible iron, folate and vitamin B12 deficiency. Then, treatment options include erythropoiesis stimulating agents, androgens, immunomodulating drugs, corticosteroids, and splenectomy. Anemia responses may also be observed in some patients treated with JAK inhibitors. However, most patients eventually fail to such therapies and become transfusion dependent. Some of the aforementioned therapies can also improve thrombocytopenia, but the responses are usually observed in patients with moderate platelet count decrease. Allogeneic hematopoietic stem cell transplantation, the only curative treatment of myelofibrosis, can be an alternative for selected patients with cytopenias who are refractory to conventional therapies. However, for the majority of patients, the management of anemia and severe thrombocytopenia remains an unmet need. PMID:26891375

  15. Anemia caused by low iron - children

    Science.gov (United States)

    Anemia - iron deficiency - children ... able to absorb iron well, even though the child is eating enough iron Slow blood loss over ... bleeding in the digestive tract Iron deficiency in children can also be related to lead poisoning .

  16. Pathophysiology of cardiovascular disease in rare anemias

    Directory of Open Access Journals (Sweden)

    Athanasios Aessopos

    2013-03-01

    Full Text Available Rare anemias encompass a large and markedly heterogeneous group of nearly 90 different conditions, mostly congenital or genetically determined, that, according to the definition of the European Commission, have a global prevalence of less than 5 per 10,000 individuals. However, the geographical distribution of several of those anemias varies considerably and thus their local prevalence may be significantly higher in certain regions...

  17. Megaloblastic, dyserythropoietic anemia following arsenic ingestion.

    Science.gov (United States)

    Lerman, B B; Ali, N; Green, D

    1980-01-01

    Following acute arsenic ingestion, a 35 year old woman experienced multiple organ failure, including renal and respiratory insufficiency, toxic hepatitis, peripheral neuropathy, and encephalopathy. In addition, she developed an anemia; the bone marrow showed a striking dyserythropoiesis with megaloblastic features. Her recovery was heralded by normalization of the bone marrow morphology, followed by improvement in all other organ dysfunction except for the peripheral neuropathy. Arsenic poisoning is a cause of megaloblastic anemia; early hematologic recovery suggests favorable prognosis.

  18. [Anemia in obstetrics and gynecological surgery].

    Science.gov (United States)

    Gredilla Díaz, E

    2015-06-01

    Iron deficiency is more common in women due to uterine bleeding, which affects them throughout their fertile life. Additionally, iron needs increase physiologically during pregnancy and breastfeeding. Pregnant women therefore constitute one of the risk groups for iron deficiency. During the postpartum period, iron deficiency is the most common cause of anemia. Longer hospital stays and greater susceptibility to infections are potential consequences of postpartum anemia.

  19. A case of delayed hemolytic transfusion reaction in sickle cell disease patient

    Science.gov (United States)

    Dogra, Ashu; Sidhu, Meena

    2016-01-01

    Sickle cell disease (SCD) is autosomal recessive, genetically transmitted hemoglobinopathy responsible for considerable morbidity and mortality. It is prevalent in many parts of India including Central India, where the prevalence in different communities has ranged from 9.4% to 22%. Perioperative management may include transfusion of red blood cells. Hemolytic transfusion reactions can occur, and these can be either acute or delayed. We present a case of delayed hemolytic transfusion reaction in a patient with SCD. PMID:27605854

  20. Effects of Ca2+ on refolding of the recombinant hemolytic lectin CEL-III.

    Science.gov (United States)

    Hisamatsu, Keigo; Unno, Hideaki; Goda, Shuichiro; Hatakeyama, Tomomitsu

    2009-05-01

    CEL-III is a hemolytic lectin isolated from Cucumaria echinata. Although recombinant CEL-III (rCEL-III) expressed in Escherichia coli showed very weak hemolytic activity compared with native protein, it was considerably enhanced by refolding in the presence of Ca(2+). This suggests that Ca(2+) supported correct folding of the carbohydrate-binding domains of rCEL-III, leading to effective binding to the cell surface and subsequent self-oligomerization. PMID:19420692

  1. A Rare Case; Hemolytic Disease of Newborn Associated with Anti-jkb

    OpenAIRE

    İlknur Tolunay; Meral Oruç; Orkun Tolunay

    2015-01-01

    Jka and Jkb antibodies (Kidd blood group system) can cause acute and delayed type transfusion reactions as well as hemolytic disease of newborn. Jka and Jkb antibodies are seen after events like blood transfusions, pregnancy, abortion and curettage. Hemolytic disease of newborn related to Kidd-Jkb incompatibility is rare and mostly has a good prognosis. The patient was consulted to our department because of 20 hours of jaundice after birth. He was treated with intensive phot...

  2. Family structure and child anemia in Mexico.

    Science.gov (United States)

    Schmeer, Kammi K

    2013-10-01

    Utilizing longitudinal data from the nationally-representative Mexico Family Life Survey, this study assesses the association between family structure and iron-deficient anemia among children ages 3-12 in Mexico. The longitudinal models (n = 4649), which control for baseline anemia status and allow for consideration of family structure transitions, suggest that children living in stable-cohabiting and single-mother families and those who have recently experienced a parental union dissolution have higher odds of anemia than those in stable-married, father-present family structures. Interaction effects indicate that unmarried family contexts have stronger associations with anemia in older children (over age five); and, that the negative effects of parental union dissolution are exacerbated in poorer households. Resident maternal grandparents have a significant beneficial effect on child anemia independent of parental family structure. These results highlight the importance of family structure for child micronutrient deficiencies and suggest that understanding social processes within households may be critical to preventing child anemia in Mexico. PMID:23294876

  3. Mouse models of anemia of cancer.

    Directory of Open Access Journals (Sweden)

    Airie Kim

    Full Text Available Anemia of cancer (AC may contribute to cancer-related fatigue and impair quality of life. Improved understanding of the pathogenesis of AC could facilitate better treatment, but animal models to study AC are lacking. We characterized four syngeneic C57BL/6 mouse cancers that cause AC. Mice with two different rapidly-growing metastatic lung cancers developed the characteristic findings of anemia of inflammation (AI, with dramatically different degrees of anemia. Mice with rapidly-growing metastatic melanoma also developed a severe anemia by 14 days, with hematologic and inflammatory parameters similar to AI. Mice with a slow-growing peritoneal ovarian cancer developed an iron-deficiency anemia, likely secondary to chronically impaired nutrition and bleeding into the peritoneal cavity. Of the four models, hepcidin mRNA levels were increased only in the milder lung cancer model. Unlike in our model of systemic inflammation induced by heat-killed Brucella abortus, ablation of hepcidin in the ovarian cancer and the milder lung cancer mouse models did not affect the severity of anemia. Hepcidin-independent mechanisms play an important role in these murine models of AC.

  4. Myocardial disease,anemia and heart failure

    Institute of Scientific and Technical Information of China (English)

    Donald S Silverberg; Dov Wexler; Adrian Iaina; Doron Schwartz

    2005-01-01

    Abstract Many patients with congestive heart failure (CHF) fail to respond to maximal CHF therapy and progress to end stage CHF with many hospitalizations, very poor quality of life, end stage renal failure, or die of cardiovascular complications within a short time. One factor that has generally been ignored in many of these patients is the fact that they are often anemic.The anemia is due mainly to renal failure but also to the inhibitory effects of cytokines on the bone marrow. Anemia itself may further worsen the cardiac function and make the patients resistant to standard CHF therapies. Indeed anemia has been associated with increased severity of CHF, increased hospitalization, worse cardiac function and functional class, higher doses of diuretics,worsening of renal function and reduced quality of life. In both controlled and uncontrolled studies the correction of the anemia with erythropoietin (EPO) and oral or Ⅳ iron is associated with improvement in all these parameters. EPO itself may also play a direct role in improving the heart unrelated to the improvement of the anemia. Anemia may also play a role in the worsening of coronary heart disease even without CHF.

  5. Effects of co-existing microalgae and grazers on the production of hemolytic toxins in Karenia mikimotoi

    Science.gov (United States)

    Yang, Weidong; Zhang, Naisheng; Cui, Weimin; Xu, Yanyan; Li, Hongye; Liu, Jiesheng

    2011-11-01

    Karenia mikimotoi (Miyake & Kominami ex Oda) Hansen & Moestrup is associated with harmful algal blooms in temperate and subtropical zones of the world. The hemolytic substances produced by K. mikimotoi are thought to cause mortality in fishes and invertebrates. We evaluated the composition of the hemolytic toxin produced by K. mikimotoi cultured in the laboratory using thin-layer chromatography. In addition, we evaluated the effect of co-occuring algae ( Prorocentrum donghaiense and Alexandrium tamarense) and the cladoceran grazer Moina mongolica on hemolytic toxin production in K. mikimotoi. The hemolytic toxins from K. mikimotoi were a mixture of 2 liposaccharides and 1 lipid. Waterborne clues from P. donghaiense and A. tamarense inhibited the growth of K. mikimotoi but increased the production of hemolytic toxins. Conversely, K. mikimotoi strongly inhibited the growth of caged P. donghaiense and A. tamarense. In addition, the ingestion of K. mikimotoi by M. mongolica induced the production of hemolytic toxins in K. mikimotoi. Taken together, our results suggest that the presence of other microalgae and grazers may be as important as environmental factors for controlling the production of hemolytic substances. K. mikimotoi secreted allelochemicals other than unstable fatty acids with hemolytic activity. The production of hemolytic toxins in dinoflagellates was not only dependent on resource availability, but also on the risk of predation. Hemolytic toxins likely play an important role as chemical deterrents secreted by K. mikimotoi.

  6. Effects of co-existing microalgae and grazers on the production of hemolytic toxins in Karenia mikimotoi

    Institute of Scientific and Technical Information of China (English)

    YANG Weidong; ZHANG Naisheng; CUI Weimin; XU Yanyan; LI Hongye; LIU Jiesheng

    2011-01-01

    Karenia mikimotoi (Miyake & Kominami ex Oda) Hansen & Moestrup is associated with harmful algal blooms in temperate and subtropical zones of the world.The hemolytic substances produced by K.mikimotoi are thought to cause mortality in fishes and invertebrates.We evaluated the composition of the hemolytic toxin produced by K.mikimotoi cultured in the laboratory using thin-layer chromatography.In addition,we evaluated the effect of co-occuring algae (Prorocentrum donghaiense and Alexandrium tamarense) and the cladoceran grazer Moina mongolica on hemolytic toxin production in K.mikimotoi.The hemolytic toxins from K.mikimotoi were a mixture of 2 liposaccharides and I lipid.Waterbome clues from P.donghaiense and A.tamarense inhibited the growth of K.mikimotoi but increased the production of hemolytic toxins.Conversely,K.mikimotoi strongly inhibited the growth of caged P.donghaiense and A.tamarense.In addition,the ingestion of K.mikimotoi by M.mongolica induced the production of hemolytic toxins in K.mikimotoi.Taken together,our results suggest that the presence of other microalgae and grazers may be as important as environmental factors for controlling the production of hemolytic substances.K.mikimotoi secreted allelochemicals other than unstable fatty acids with hemolytic activity.The production of hemolytic toxins in dinoflagellates was not only dependent on resource availability,but also on the risk of predation.Hemolytic toxins likely play an important role as chemical deterrents secreted by K.mikimotoi.

  7. Autoimmune polyglandular syndrome type II in Hospital Universitario del Caribe, Cartagena Colombia.

    Directory of Open Access Journals (Sweden)

    Fortich-Revollo Álvaro José

    2011-06-01

    Full Text Available Autoimmune polyglandular syndrome is a polyendocrinopathy characterized by failureof some endocrine glands as well as nonendocrine organs, caused by actions of theimmune system on endocrine tissues. It has been described two groups and at leasttwo or three variants of them. Autoimmune polyglandular autoimmune syndrome typeII is the most common autoimmune endocrinopathy that is characterized mainly bypresence of Addison’s disease in combination with autoimmune thyroid disease or typeI diabetes mellitus. We review the topic and immunogenetics and etiopathological basesand present a case series. The incidence is 1.2/100.000. The most common finding wasAddison’s disease plus autoimmune thyroiditis (80% and the second most frequentassociation was thyroiditis with pernicious anemia (60%. It is important to note thehigh frequency of vitamin B12 deficiency in patients with severe neuronal impairment. Itmay take up to twenty years between diagnosis of an endocrine disease and emergenceof another disease. It is the duty to perform diagnostic tests to evaluate hormonalfunction correlated with an endocrinopathy until senescence.RESUMEN:El Síndrome Poliglandular Autoinmune es una poliendocrinopatía caracterizada porfalla de algunas glándulas endocrinas así como también en órganos no endocrinos,originada por acciones del sistema inmune sobre tejidos endocrinos. Se han descritodos grandes grupos y al menos dos o tres variantes de ellos. El Síndrome PoliglandularAutoinmune tipo II es la más común de las inmunoendocrinopatias. Se caracteriza porla presencia de la enfermedad de Addison en combinación con enfermedad tiroideaautoinmune y/o diabetes mellitus tipo I. Se realizo una revisión del tema con basesinmunogenéticas y etiopatológicas. Se presenta una serie de casos. La incidenciaacumulada es de 1.2/100.000 habitante. El hallazgo más frecuente fue enfermedadde Addison más Tiroiditis autoinmune, (80 % y la segunda asociación m

  8. Correlative study on anemia and radiotherapy effects in nasopharyngeal carcinoma

    International Nuclear Information System (INIS)

    Objective: To study the effect of oxygen-carrying ability of blood efficacy of radiotherapy for patients with nasopharyngeal carcinoma. Methods: Altogether 161 cases of patients with nasopharyngeal carcinoma were classified according to severity of anemia, and Hb, RBC, MCH, HCT, MCV, MCHC and RDW were tested before, during and after radiotherapy. The patients were followed-up for up to 5 years, the relationship and mechanism among anemia, radiotherapy effects and survival rate was discussed. Results: The survival rate between anemia group and non-anemia group was different significantly (P<0.05). Anemia before radiotherapy, anemia appearance or anemia deterioration during radiotherapy were sensitive factors affecting radiotherapy results. The anemia more severe, the radiotherapy worse. Conclusion: Anemia-hypohemoglobinemia leads to decrease of oxygen-carrying capacity of blood, resulting in oxygen deficiency of tumor cells and their radiotherapy resistance. Therefore this method is worthy of further studies

  9. Historical reflections on autoimmune hepatitis

    Institute of Scientific and Technical Information of China (English)

    Ian R Mackay

    2008-01-01

    Autoimmune hepatitis (AIH),initially known as chronic active or active chronic hepatitis (and by various other names),first came under clinical notice in the late 1940s.However,quite likely,chronic active hepatitis (CAH) had been observed prior to this and was attributed to a persistently destructive virus infection of the liver.An earlier (and controversial) designation in 1956 as lupoid hepatitis was derived from associated L.E.cell test positivity and emphasized accompanying multisystem features and immunological aberrations.Young women featured prominently in early descriptions of CAH.AIH was first applied in 1965 as a descriptive term.Disease-characteristic autoantibodies were defined from the early 1960s,notably antinuclear antibody (ANA),smooth muscle antibody (SMA) and liver-kidney microsomal (LKM) antibody.These are still widely used diagnostically but their relationship to pathogenesis is still not evident.A liver and disease specific autoantigen has long been searched for but unsuccessfully.Prolonged immunosuppressive therapy with predisolone and azathioprine in the 1960s proved beneficial and remains standard therapy today.AIH like many other autoimmune diseases is associated with particular HLA alleles especially with the "ancestral" B8,DR3 haplotype,and also with DR4.Looking forwards,AIH is one of the several enigmatic autoimmune diseases that,despite being (relatively) organ specific,are marked by autoimmune reactivities with non-organ-specific autoantigens.New paradigms are needed to explain the occurrence,expressions and pathogenesis of such diseases.

  10. Autoimmunity: Experimental and clinical aspects

    Energy Technology Data Exchange (ETDEWEB)

    Schwartz, R.S.; Rose, N.R.

    1986-01-01

    This book contains five parts and a section of poster papers. Each part contains several papers. Some of the papers are: Molecular Genetics and T Cells in Autoimmunity; Gene Conversion: A Mechanism to Explain HLA-D Region and Disease Association; Genetics of the Complement System; Speculation on the Role of Somatic Mutation in the Generation of Anti-DNA Antibodies; and Monoclonal Anti-DNA Antibodies: The Targets and Origins of SLE.

  11. Cystic Lesions in Autoimmune Pancreatitis

    OpenAIRE

    Gompertz, Macarena; Morales, Claudia; Aldana, Hernán; Castillo, Jaime; Berger, Zoltán

    2015-01-01

    Autoimmune pancreatitis (AIP) can be chronic or recurrent, but frequently completely reversible after steroid treatment. A cystic lesion in AIP is a rare finding, and it can mimic a pancreatic cystic neoplasm. Difficulties in an exact diagnosis interfere with treatment, and surgery cannot be avoided in some cases. We report the history of a 63-year-old male presenting with jaundice and pruritus. AIP was confirmed by imaging and elevated IgG4 blood levels, and the patient completely recovered ...

  12. Autoimmunity in chronic lymphocytic leukaemia.

    OpenAIRE

    Lischner, M.; Prokocimer, M.; Zolberg, A.; Shaklai, M.

    1988-01-01

    Seventy-nine patients with chronic lymphocytic leukaemia were evaluated for the presence of autoimmune diseases and autoantibodies. One patient has polymyositis and two additional patients presented with features suggestive of pernicious anaemia and chronic active hepatitis. The Coombs' direct test was positive in 7% and immune thrombocytopenia was present in 8.1% of patients. Five (7%) patients had M-protein in the serum. No increased frequency of other autoantibodies was noted in our study ...

  13. Historical reflections on autoimmune hepatitis

    OpenAIRE

    Mackay, Ian R.

    2008-01-01

    Autoimmune hepatitis (AIH), initially known as chronic active or active chronic hepatitis (and by various other names), first came under clinical notice in the late 1940s. However, quite likely, chronic active hepatitis (CAH) had been observed prior to this and was attributed to a persistently destructive virus infection of the liver. An earlier (and controversial) designation in 1956 as lupoid hepatitis was derived from associated L.E. cell test positivity and emphasized accompanying multisy...

  14. Pancreatic Tuberculosis or Autoimmune Pancreatitis

    Directory of Open Access Journals (Sweden)

    Ayesha Salahuddin

    2014-01-01

    Full Text Available Introduction. Isolated pancreatic and peripancreatic tuberculosis is a challenging diagnosis due to its rarity and variable presentation. Pancreatic tuberculosis can mimic pancreatic carcinoma. Similarly, autoimmune pancreatitis can appear as a focal lesion resembling pancreatic malignancy. Endoscopic ultrasound-guided fine needle aspiration provides an effective tool for differentiating between benign and malignant pancreatic lesions. The immune processes involved in immunoglobulin G4 related systemic diseases and tuberculosis appear to have some similarities. Case Report. We report a case of a 59-year-old Southeast Asian male who presented with fever, weight loss, and obstructive jaundice. CT scan revealed pancreatic mass and enlarged peripancreatic lymph nodes. Endoscopic ultrasound-guided fine needle aspiration confirmed the presence of mycobacterium tuberculosis. Patient also had high immunoglobulin G4 levels suggestive of autoimmune pancreatitis. He was started on antituberculosis medications and steroids. Clinically, he responded to treatment. Follow-up imaging showed findings suggestive of chronic pancreatitis. Discussion. Pancreatic tuberculosis and autoimmune pancreatitis can mimic pancreatic malignancy. Accurate diagnosis is imperative as unnecessary surgical intervention can be avoided. Endoscopic ultrasound-guided fine needle aspiration seems to be the diagnostic test of choice for pancreatic masses. Long-term follow-up is warranted in cases of chronic pancreatitis.

  15. [Autoimmune hepatitis induced by isotretionine].

    Science.gov (United States)

    Guzman Rojas, Patricia; Gallegos Lopez, Roxana; Ciliotta Chehade, Alessandra; Scavino, Yolanda; Morales, Alejandro; Tagle, Martín

    2016-01-01

    We describe a case of a teenage patient with the diagnosis of drug induced autoimmune hepatitis. The patient is a 16 years old female, with the past medical history of Hashimoto’s hypothyroidism controlled with levothyroxine, who started treatment with Isotretionin (®Accutane) 20 mg q/12 hours for a total of 3 months for the treatment of severe acne. The physical examination was within normal limits and the results of the laboratory exams are: Baseline values of ALT 28 U/L, AST 28 U/L. Three months later: AST 756 U/L, ALT 1199U/L, alkaline phosphatase 114 U/L, with normal bilirrubin levels throughout the process. The serology studies were negative for all viral hepatitis; ANA titers were positive (1/160) and igG levels were also elevated. A liver biopsy was performed, and was compatible with the diagnosis of autoimmune hepatitis. Corticosteroid therapy was started with Prednisone 40 mg per day one week after stopping the treatment with isotretionin, observing an improvement in the laboratory values. We describe this case and review the world literature since there are no reported cases of Isotretinoin-induced autoimmune hepatitis. PMID:27131947

  16. Effects of Testosterone on Erythropoiesis in a Female Mouse Model of Anemia of Inflammation.

    Science.gov (United States)

    Guo, Wen; Schmidt, Paul J; Fleming, Mark D; Bhasin, Shalender

    2016-07-01

    The anemia of inflammation is a common problem in inflammatory and autoimmune diseases. We characterized a mouse model of anemia of chronic inflammation induced by repeated injections of low doses of heat-killed Brucella abortus (HKBA), and determined the effects of T administration on erythropoiesis in this model. Female C57BL/6NCrl mice were injected weekly with HKBA for 10 wk. Weekly injections of T or vehicle oil were started 4 wk later. Control mice were injected with saline and vehicle oil in parallel. HKBA-injected mice had significantly lower hemoglobin, hematocrit, mean corpuscular volume, reticulocyte hemoglobin, transferrin saturation (TSAT), and tissue nonheme iron in liver and spleen, enlarged spleen, and up-regulated hepatic expression of inflammatory markers, serum amyloid A1, and TNFα, but down-regulated IL-6, bone morphogenic protein 6, and hepcidin compared with saline controls. HKBA also reduced serum hepcidin and increased serum erythropoietin. Bone marrow erythroid precursors were substantially reduced in HKBA-injected mice. Cotreatment with T increased the percentage of late-stage erythroid precursors in the bone marrow relative to HKBA-injected and saline controls and reversed HKBA-induced suppression of hemoglobin and hematocrit. T also normalized serum erythropoietin, TSAT, and reticulocyte hemoglobin without correcting the expression of the hepatic inflammation markers. Conclusions are that low-dose HKBA induces moderate anemia characterized by chronic inflammation, decreased iron stores, and suppression of erythroid precursors in the bone marrow. T administration reverses HKBA-induced anemia by stimulating erythropoiesis, which is associated with a shift toward accelerated maturation of erythroid precursors in the bone marrow. PMID:27074351

  17. Cholesterol-dependent hemolytic activity of Passiflora quadrangularis leaves

    Directory of Open Access Journals (Sweden)

    L.N. Yuldasheva

    2005-07-01

    Full Text Available Plants used in traditional medicine are rich sources of hemolysins and cytolysins, which are potential bactericidal and anticancer drugs. The present study demonstrates for the first time the presence of a hemolysin in the leaves of Passiflora quadrangularis L. This hemolysin is heat stable, resistant to trypsin treatment, has the capacity to froth, and acts very rapidly. The hemolysin activity is dose-dependent, with a slope greater than 1 in a double-logarithmic plot. Polyethylene glycols of high molecular weight were able to reduce the rate of hemolysis, while liposomes containing cholesterol completely inhibited it. In contrast, liposomes containing phosphatidylcholine were ineffective. The Passiflora hemolysin markedly increased the conductance of planar lipid bilayers containing cholesterol but was ineffective in cholesterol-free bilayers. Successive extraction of the crude hemolysin with n-hexane, chloroform, ethyl acetate, and n-butanol resulted in a 10-fold purification, with the hemolytic activity being recovered in the n-butanol fraction. The data suggest that membrane cholesterol is the primary target for this hemolysin and that several hemolysin molecules form a large transmembrane water pore. The properties of the Passiflora hemolysin, such as its frothing ability, positive color reaction with vanillin, selective extraction with n-butanol, HPLC profile, cholesterol-dependent membrane susceptibility, formation of a stable complex with cholesterol, and rapid erythrocyte lysis kinetics indicate that it is probably a saponin.

  18. Atypical Hemolytic Uremic Syndrome Recurrence after Renal Transplantation

    Science.gov (United States)

    Bouatou, Yassine; Bacchi, Véronique Frémeaux; Villard, Jean; Moll, Solange; Martin, Pierre-Yves; Hadaya, Karine

    2015-01-01

    Abstract Risk for atypical hemolytic uremic syndrome (aHUS) recurrence after renal transplantation is low with an isolated membrane cofactor protein mutation (MCP). We report the case of a 32-year-old woman with a MCP who underwent kidney transplantation with a good evolution at 12 months. At 15 and 35 months, 2 episodes of thrombotic microangiopathy (TMA), after a miscarriage and a preeclampsia, were misinterpreted as triggered by tacrolimus. After each episode however serum creatinine returned to baseline. Five years after transplantation, she had a self-limited rhinosinusitis followed 3 weeks later by an oliguric renal failure. Her complement profile was normal. Graft biopsy showed C3 glomerulonephritis with no “humps” on electron microscopy. No significant renal function improvement followed methylprednisolone pulsing. A second biopsy showed severe acute TMA lesions with C3 glomerular deposits. Despite weekly eculizumab for 1 month, dialysis was resumed. A new workup identified the “at-risk” complement factor H haplotype. Thus, aHUS recurrence should be ruled out in aHUS patients considered at low recurrence risk when a TMA is found in graft biopsy. Prompt eculizumab therapy should be considered to avoid graft loss as aHUS recurrence can first present as a C3 glomerulonephritis. PMID:27500215

  19. Hemorrhagic retinopathy in an infant with hemolytic-uremic syndrome

    Directory of Open Access Journals (Sweden)

    João Caetano Ávila Geraissate

    2014-12-01

    Full Text Available We describe the case of a 23-month-old female infant with a diagnosis of hemolytic uremic syndrome (HUS and hemorrhagic retinopathy. The patient had a past history of abdominal pain, bloody diarrhea, and acute renal failure. On ophthalmologic examination, indirect ophthalmoscopy revealed extensive areas of flame-shaped hemorrhage, cotton wool spots, macular edema and optic nerve head neovascularization in both eyes. Fluorescein angiography showed severe bilateral retinal ischemia and neovascularization leakage in disk. The patient, who had the visual acuity of 20/1000 in the right eye (OD and 20/540 in the left eye (OS at the first examination, was treated with panretinal photocoagulation (PRP and presented at the end of the 6th month of follow-up improvement to 20/540 in OD and 20/270 in OS. There was also a regression of disc neovascularization, hemorrhages and macular edema. Despite intense retinal ischemia, there were no complications related to angiogenesis such as vitreous hemorrhage and/or neovascular glaucoma. We describe, in this report, the association between hemorrhagic retinopathy with features of Purtscher-like disease and HUS.

  20. Anemia in patients with diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Dimković Nada

    2007-01-01

    Full Text Available Introduction: Anemia is more common and pronounced in patients with diabetic, than in patients with non-diabetic renal disease. While several factors contribute to its pathogenesis, the failure of the kidney to increase erythropoietin in response to falling hemoglobin appears to be the dominant factor. The most frequent complications of anemia in diabetic patients include decreased quality of life and work capacity and increased cardiovascular morbidity and mortality. Material and Methods: This cross-sectional multicenter study included a total of 539 patients with type I (~20% and type II diabetes (~80% classified into five stages according to the glomerular filtration rate. Results Diabetic nephropathy appears in stage I, and progresses in all patients to the stage V (p=0.045. The presence of anemia progressively increased from stage I to stage V (from 60% to 100%, p=0.008. Only 62% of patients with anemia were treated (mainly with iron and only 3.4% received erythropoietin treatment. Hypertension was present in 90% of patients in stage I and in 100% of patients in stage V nephropathy. The presence of heart failure increased from 0% (stage I to 51% (stage IV, p=0.03. Around 62% of patients were referred to a nephrologist, and according to the logistic regression model, renal failure and presence of anemia were significant predictors of patients' referral to nephrologist. Conclusion: In a primary care setting, anemia is a frequent finding, even in the very beginning of diabetic renal disease. Currently available guidelines for management of anemia are not followed; this may explain high percentage of patients with heart failure in pre-dialysis stage. Early referral to a nephrologist and regular follow-up by an endocrinologist and cardiologist are the best way for the prevention of diabetic complications and comorbidity.