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Sample records for anemia aplastic

  1. 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, ...

  2. 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 ...

  3. Aplastic anemia

    Science.gov (United States)

    ... the number of these blood cell types. Aplastic anemia can be caused by: Use of certain drugs or exposure to toxic chemicals (such as benzene) Exposure to radiation or chemotherapy Autoimmune disorders Pregnancy Viruses Sometimes, the cause is unknown. In this ...

  4. 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;}

  5. 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 ...

  6. What Causes Aplastic Anemia?

    Science.gov (United States)

    ... blood and bone marrow diseases, such as aplastic anemia. // Non Object? Updated: August 22, 2012 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  7. Living with Aplastic Anemia

    Science.gov (United States)

    ... blood and bone marrow diseases, such as aplastic anemia. // Non Object? Updated: August 22, 2012 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  8. Aplastic Anemia & MDS International Foundation

    Science.gov (United States)

    ... Menu Donate I'm Like You. "The Aplastic Anemia and MDS International Foundation is helping patients like ... cope with bone marrow failure disease." Diseases Aplastic Anemia Myelodysplastic Syndromes (MDS) Paroxysmal Nocturnal Hemoglobinuria (PNH) Related ...

  9. 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 ...

  10. Aplastic Anemia and Myelodysplastic Syndromes

    Science.gov (United States)

    ... have aplastic anemia. Most new cases of aplastic anemia in children are due to inherited bone marrow failure syndromes. MDS are typically diagnosed between the ages of 70 and 80 years. Although a cause is not found in most cases of aplastic anemia and MDS, the diseases may be triggered by ...

  11. 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 ...

  12. Syngeneic transplantation in aplastic anemia

    DEFF Research Database (Denmark)

    Gerull, Sabine; Stern, Martin; Apperley, Jane

    2013-01-01

    Aplastic anemia is usually treated with immunosuppression or allogeneic transplant, depending on patient and disease characteristics. Syngeneic transplant offers a rare treatment opportunity with minimal transplant-related mortality, and offers an insight into disease mechanisms. We present here...... 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. Acquired aplastic anemia in children.

    Science.gov (United States)

    Hartung, Helge D; Olson, Timothy S; Bessler, Monica

    2013-12-01

    This article provides a practice-based and concise review of the etiology, diagnosis, and management of acquired aplastic anemia in children. Bone marrow transplantation, immunosuppressive therapy, and supportive care are discussed in detail. The aim is to provide the clinician with a better understanding of the disease and to offer guidelines for the management of children with this uncommon yet serious disorder.

  14. Child with aplastic anemia: Anesthetic management

    Directory of Open Access Journals (Sweden)

    Manpreet Kaur

    2012-01-01

    Full Text Available Aplastic anemia is a rare heterogeneous disorder of hematopoietic stem cells causing pancytopenia and marrow hypoplasia with the depletion of all types of blood cells. This results in anemia, neutropenia and thrombocytopenia, which pose a challenge to both surgical and anesthetic management of such cases. We report a child with aplastic anemia who sustained traumatic ulcer on the arm and underwent split-thickness skin grafting under general anesthesia. There are only two case reports on anesthetic considerations in aplastic anemia patients in the literature. The anesthetic management is challenging because of the rarity of the disease, associated pancytopenia and immunosuppression.

  15. Child with aplastic anemia: Anesthetic management

    OpenAIRE

    Manpreet Kaur; Babita Gupta; Aanchal Sharma; Sanjeev Sharma

    2012-01-01

    Aplastic anemia is a rare heterogeneous disorder of hematopoietic stem cells causing pancytopenia and marrow hypoplasia with the depletion of all types of blood cells. This results in anemia, neutropenia and thrombocytopenia, which pose a challenge to both surgical and anesthetic management of such cases. We report a child with aplastic anemia who sustained traumatic ulcer on the arm and underwent split-thickness skin grafting under general anesthesia. There are only two case reports on anest...

  16. What Are the Signs and Symptoms of Aplastic Anemia?

    Science.gov (United States)

    ... What Are the Signs and Symptoms of Aplastic Anemia? Lower than normal numbers of red blood cells, ... most of the signs and symptoms of aplastic anemia. Signs and Symptoms of Low Blood Cell Counts ...

  17. Severe Combined Immunodeficiency (SCID) Presenting with Neonatal Aplastic Anemia

    Science.gov (United States)

    Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy; Xu, Min; Burroughs, Lauri; Woolfrey, Ann; Fleming, Mark; Shimamura, Akiko

    2015-01-01

    Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications. PMID:26011426

  18. Severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia.

    Science.gov (United States)

    Scott, Angela; Glover, Jason; Skoda-Smith, Suzanne; Torgerson, Troy R; Xu, Min; Burroughs, Lauri M; Woolfrey, Ann E; Fleming, Mark D; Shimamura, Akiko

    2015-11-01

    Aplastic anemia in the neonate is rare. We report a case of severe combined immunodeficiency (SCID) presenting with neonatal aplastic anemia. This report highlights the importance of considering SCID early in the evaluation of neonatal aplastic anemia prior to the development of infectious complications.

  19. Idiopathic aplastic anemia: diagnosis and classification.

    Science.gov (United States)

    Dolberg, Osnat Jarchowsky; Levy, Yair

    2014-01-01

    Aplastic anemia (AA) is a disease characterized by pancytopenia and hypoplastic bone marrow caused by the decrease of hematopoietic stem cells. The pathogenesis of AA is complex and involves an abnormal hematopoietic microenvironment, hematopoietic stem cell/progenitor cell deficiencies and immunity disorders. Survival in severe aplastic anemia (SAA) has markedly improved in the past 4 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. Herein, we will update the main issues concern AA according to our literature review.

  20. APLASTIC ANEMIA ET CAUSA OF SUSPECT VIRAL HEPATITIS INFECTION: A CASE REPORT

    OpenAIRE

    I Wayan Wawan Lismana

    2014-01-01

    Aplastic anemia is anemia that occurs because of a failure of hematopoiesis is relatively rarebut can be life threatening. The cause of aplastic anemia itself is still largely unknown oridiopathic. Minority of cases mainly due to a virus infection, one of which is viral hepatitishas long been known to cause symptoms of aplastic anemia. This report discusses thesuspected aplastic anemia caused by hepatitis virus infection. Course of the disease or theprognosis of aplastic anemia varies, but a ...

  1. Aplastic Anemia in Two Patients with Sex Chromosome Aneuploidies.

    Science.gov (United States)

    Rush, Eric T; Schaefer, G Bradley; Sanger, Warren G; Coccia, Peter F

    2015-01-01

    Sex chromosome aneuploidies range in incidence from rather common to exceedingly rare and have a variable phenotype. We report 2 patients with sex chromosome aneuploidies who developed severe aplastic anemia requiring treatment. The first patient had tetrasomy X (48,XXXX) and presented at 9 years of age, and the second patient had trisomy X (47,XXX) and presented at 5 years of age. Although aplastic anemia has been associated with other chromosomal abnormalities, sex chromosome abnormalities have not been traditionally considered a risk factor for this condition. A review of the literature reveals that at least one other patient with a sex chromosome aneuploidy (45,X) has suffered from aplastic anemia and that other autosomal chromosomal anomalies have been described. Despite the uncommon nature of each condition, it is possible that the apparent association is coincidental. A better understanding of the genetic causes of aplastic anemia remains important.

  2. Colon cancer in a patient with underlying aplastic anemia: A clinical challenge

    OpenAIRE

    Wong, Hilda; Chan, Pierre; Yau, Thomas

    2012-01-01

    The association of gastrointestinal malignancy with aplastic anemia has rarely been reported in the literature. Although it is not clear whether there is any direct relationship between aplastic anemia and gastrointestinal cancers, a retrospective analysis did suggest the notion that patients with aplastic anemia might have a higher incidence of colorectal cancer. Here, we report the diagnostic and therapeutic challenges in managing a patient with aplastic anemia and advanced colorectal cance...

  3. Aplastic anemia: A common hematological abnormality among peripheral pancytopenia

    Directory of Open Access Journals (Sweden)

    Haldar Biswajit

    2012-01-01

    Full Text Available Background: Aplastic anemia is a well-recognized form of marrow failure. The incidence of aplastic anemia is subjected to wide variation. Most cases are acquired and immune-mediated but there are also inherited forms. Aim: The study was conducted to assess the magnitude of the problem, morphological changes and determinants of aplastic anemia in North Bengal. Materials and Methods: A cross-sectional study had been conducted for a period of one year among 5 to 70 years age group. Initially complete blood count followed by bone marrow examination was done for diagnosis. Results: Out of 48 cases, 38 (79.17% had hypocellular diagnosed as aplastic anemia, 5 (10.42% each had normocellular and hypercellular bone marrow. Histopathology shows that 30 (78.95% cases had increased iron stores in bone marrow and 8 (21.05% cases had decreased iron stores. Subjects less than 20 years of age mostly (31.58% suffered from non-severe disease in contrast to subjects in higher age groups who had severe or very severe disease, though the trend was not significantly different (χ2 for linear trend 0.171, P > 0.05. Conclusion: The study shows aplastic anemia is a common hematological abnormality among peripheral pancytopenia in North Bengal region where males were affected more than females.

  4. Environmental lead exposure as a risk for childhood aplastic anemia.

    Science.gov (United States)

    Ahamed, M; Akhtar, M J; Verma, S; Kumar, A; Siddiqui, M K J

    2011-01-01

    Concern about environmental lead exposure as a significant public health threat has increased as evidence has accumulated regarding adverse health effects at successively lower levels. Aplastic anemia is a hematological disorder of unknown etiology with a high lethality rate. Lead is a known toxicant for the hematopoietic system. Oxidative stress appears to be the possible mode of lead toxicity. We evaluated the effects of blood lead level on oxidative stress parameters in children suffering from aplastic anemia disease. Seventeen children with aplastic anemia disease (15 male and 2 female, age 3-12 y) were recruited in the study group. Fifty one healthy children (45 male and 6 female, age 3-12 y) having normal blood profiles and not suffering from any chronic disease(s) were used as controls. Blood lead level and oxidative stress parameters were determined. Mean blood lead level was significantly higher while δ-aminolevulinic acid dehydratase (δ-ALAD) activity, a biomarker for lead exposure was significantly lower in the study group as compared to the control group (p lead levels with δ-ALAD (r = -0.45; p lead induces oxidative stress in children suffering from aplastic anemia. Lead-induced oxidative stress as an underlying mechanism for aplastic anemia warrants further research.

  5. Recent advances in treatment of aplastic anemia.

    Science.gov (United States)

    Shin, Seung Hwan; Lee, Sung Eun; Lee, Jong Wook

    2014-11-01

    Recent advances in the treatment of aplastic anemia (AA) made most of patients to expect to achieve a long-term survival. Allogeneic stem cell transplantation (SCT) from HLA-matched sibling donor (MSD-SCT) is a preferred first-line treatment option for younger patients with severe or very severe AA, whereas immunosuppressive treatment (IST) is an alternative option for others. Horse anti-thymocyte globuline (ATG) with cyclosporin A (CsA) had been a standard IST regimen with acceptable response rate. Recently, horse ATG had been not available and replaced with rabbit ATG in most countries. Subsequently, recent comparative studies showed that the outcomes of patients who received rabbit ATG/CsA were similar or inferior compared to those who received horse ATG/CsA. Therefore, further studies to improve the outcomes of IST, including additional eltrombopag, are necessary. On the other hand, the upper age limit of patients who are able to receive MSD-SCT as first-line treatment is a current issue because of favorable outcomes of MSD-SCT of older patients using fludarabine-based conditioning. In addition, further studies to improve the outcomes of patients who receive allogeneic SCT from alternative donors are needed. In this review, current issues and the newly emerging trends that may improve their outcomes in near futures will be discussed focusing the management of patients with AA.

  6. Anemia

    Science.gov (United States)

    ... Hemolytic anemia Idiopathic aplastic anemia Megaloblastic anemia Pernicious anemia Sickle cell anemia Thalassemia Causes Although many parts of the ... anemia Immune hemolytic anemia Iron deficiency anemia Pernicious anemia Sickle cell anemia Vitamin B12 deficiency anemia Review Date 2/ ...

  7. Proton MR spectroscopy of hyperplastic hematopoietic marrow in aplastic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Amano, Yasuo; Kumazaki, Tatsuo [Nippon Medical School, Tokyo (Japan); Arai, Nobuyuki

    1997-04-01

    The purpose of this study was to compare the findings of magnetic resonance (MR) spectroscopy of hyperplastic hematopoietic marrow with those of normal bone marrow. Twenty-four samples of normal marrow from eight control subjects and 19 samples of hyperplastic marrow in aplastic anemia were examined with a 1.5 T MR unit. The former showed low intensity on opposed-phase T1-weighted images, while the latter showed high intensity on both fast STIR and opposed-phase T1-weighted images. MR spectroscopy quantitatively confirmed that the water; fat ratio was increased and the transverse relaxation time of water was changed in hyperplastic bone marrow, compared with normal bone marrow. In summary, MR imaging is able to detect hematopoietic regions among a wide range of bone marrow of aplastic anemia, while MR spectroscopy allowed us to quantitatively analyze the cell population of hyperplastic hematopoietic marrow in aplastic anemia. (author)

  8. Clonal hematopoiesis in acquired aplastic anemia

    Science.gov (United States)

    2016-01-01

    Clonal hematopoiesis (CH) in aplastic anemia (AA) has been closely linked to the evolution of late clonal disorders, including paroxysmal nocturnal hemoglobinuria and myelodysplastic syndromes (MDS)/acute myeloid leukemia (AML), which are common complications after successful immunosuppressive therapy (IST). With the advent of high-throughput sequencing of recent years, the molecular aspect of CH in AA has been clarified by comprehensive detection of somatic mutations that drive clonal evolution. Genetic abnormalities are found in ∼50% of patients with AA and, except for PIGA mutations and copy-neutral loss-of-heterozygosity, or uniparental disomy (UPD) in 6p (6pUPD), are most frequently represented by mutations involving genes commonly mutated in myeloid malignancies, including DNMT3A, ASXL1, and BCOR/BCORL1. Mutations exhibit distinct chronological profiles and clinical impacts. BCOR/BCORL1 and PIGA mutations tend to disappear or show stable clone size and predict a better response to IST and a significantly better clinical outcome compared with mutations in DNMT3A, ASXL1, and other genes, which are likely to increase their clone size, are associated with a faster progression to MDS/AML, and predict an unfavorable survival. High frequency of 6pUPD and overrepresentation of PIGA and BCOR/BCORL1 mutations are unique to AA, suggesting the role of autoimmunity in clonal selection. By contrast, DNMT3A and ASXL1 mutations, also commonly seen in CH in the general population, indicate a close link to CH in the aged bone marrow, in terms of the mechanism for selection. Detection and close monitoring of somatic mutations/evolution may help with prediction and diagnosis of clonal evolution of MDS/AML and better management of patients with AA. PMID:27121470

  9. Worsening Bradycardia Following Antithymocyte Globulin Treatment of Severe Aplastic Anemia

    OpenAIRE

    2011-01-01

    Immunosuppressive regimens, which include antithymocyte globulin (ATG), are widely used for the treatment of severe aplastic anemia (SAA). However, bradycardia has been reported only as a rare side effect of ATG therapy in the manufacturer's product information and, in rare cases, in the adult literature. We present an adolescent with SAA and preexisting bradycardia who underwent immunosuppression therapy with ATG, methylprednisolone, and tacrolimus and developed profound sinus bradycardia wi...

  10. Immunosuppressive therapy for transplant-ineligible aplastic anemia patients.

    Science.gov (United States)

    Schrezenmeier, Hubert; Körper, Sixten; Höchsmann, Britta

    2015-02-01

    Aplastic anemia is a rare life-threatening bone marrow failure that is characterized by bicytopenia or pancytopenia in the peripheral blood and a hypoplastic or aplastic bone marrow. The patients are at risk of infection and hemorrhage due to neutropenia and thrombocytopenia and suffer from symptoms of anemia. The main treatment approaches are allogeneic stem cell transplantation and immunosuppression. Here, we review current standard immunosuppression and the attempts that have been made in the past two decades to improve results: review of recent developments also reveals that sometimes not only the advent of new drugs, good ideas and well-designed clinical trials decide the progress in the field but also marketing considerations of pharmaceutical companies. Aplastic anemia experts unfortunately had to face the situation that efficient drugs were withdrawn simply for marketing considerations. We will discuss the current options and challenges in first-line treatment and management of relapsing and refractory patients with an emphasis on adult patients. Some promising new approaches are currently under investigation in prospective, randomized trials.

  11. Management of aplastic anemia in a woman during pregnancy: A case report

    NARCIS (Netherlands)

    K.J.M. Stibbe (Krista J. M.); H.I.J. Wildschut (Hajo); P.J. Lugtenburg

    2011-01-01

    textabstractIntroduction. Aplastic anemia is a rare disease caused by destruction of pluripotent stem cells in bone marrow. During pregnancy it could be life-threatening for both mother and child. The only causal therapy for aplastic anemia is bone marrow transplantation, which is contraindicated du

  12. MRI of the femoral bone marrow in the assessment of aplastic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Osamu; Matsuura, Katsuhiko; Ichikawa, Tamaki; Kobayashi, Yasuyuki; Nagai, Jun; Takagi, Shojiro [Jichi Medical School, Saitama (Japan) Omiya Medical Center

    1995-11-01

    MR imaging of the femoral bone marrow was performed in 12 patients with untreated aplastic anemia and six patients with hypoplastic myelodysplastic syndrome (MDS). The MRI appearance was classified into four patterns; fatty marrow faint signal, nodular pattern and heterogeneous infiltration. The MRI patterns of aplastic anemia were evaluated and compared with those of hypoplastic MDS. In spite of hypocellular biopsies, MRI of the femoral marrow showed unexpected abnormal signal intensities in aplastic anemia; nodular pattern in five and heterogeneous infiltration pattern in two patients. Completely fatty marrow was depicted in four patients mainly with severe aplastic anemia. The nodular pattern with a background of fatty marrow was commonly seen in moderate or severe cases, while the heterogeneous infiltration pattern was noted in mild cases of the disease. Compared with hypoplastic MDS, asymmetrical nodular pattern suggesting patchy hematopoiesis was thought to be a characteristic finding of aplastic anemia. One patient clinically diagnosed as aplastic anemia, who had shown heterogeneous infiltration pattern, evolved to acute myeloid leukemia. We concluded that MRI of the femoral marrow could be useful in the assessment of aplastic anemia and detection of myelodysplastic or leukemic transformation. (author).

  13. Meta-analysis of Huangqi injection for the adjunctive therapy of aplastic anemia.

    Science.gov (United States)

    Zhu, Changtai; Gao, Yulu; Jiang, Ting; Hao, Cao; Gao, Zongshuai; Sun, Yongning

    2015-01-01

    Aplastic anemia therapy remains difficult, due to lack of effective treatment regimens. In recent years, Huangqi injection for the adjunctive therapy of aplastic anemia has been reported in many clinical trials. Considering that Huangqi injection may be a novel approach to aplastic anemia treatment, we conducted a meta-analysis of clinical controlled trials to assess the clinical value of Huangqi injection in the treatment of aplastic anemia. We searched the Chinese Biomedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), Chinese Scientific Journals Full-text Database (VIP), Wanfang Database, PubMed and EMBASE database to collect the data about the trials of Huangqi injection combined with androgens for treating aplastic anemia. A total of ten studies involving 720 patients with aplastic anemia were included in this study. The meta-analysis showed significant increases in the pool effectiveness rate, white blood cells (WBC), haematoglobin (Hb), platelets (PLT), and reticulocytes (Ret) between the experimental group versus the control group. No severe side effects were found in this study. However, the lower Jadad scores and asymmetric funnel plot degrades the validity of the meta-analysis as the clinical evidence. Therefore, Huangqi injection may significantly enhance the efficacy of androgens for aplastic anemia, suggesting that the novel approach of Chinese traditional medicine combined with Western medicine is promising. The exact outcome required confirmation with rigorously well-designed multi-center trials.

  14. Myelodysplastic syndrome evolving from aplastic anemia treated with immunosuppressive therapy: efficacy of hematopoietic stem cell transplantation.

    Science.gov (United States)

    Kim, Sung-Yong; Le Rademacher, Jennifer; Antin, Joseph H; Anderlini, Paolo; Ayas, Mouhab; Battiwalla, Minoo; Carreras, Jeanette; Kurtzberg, Joanne; Nakamura, Ryotaro; Eapen, Mary; Deeg, H Joachim

    2014-12-01

    A proportion of patients with aplastic anemia who are treated with immunosuppressive therapy develop clonal hematologic disorders, including post-aplastic anemia myelodysplastic syndrome. Many will proceed to allogeneic hematopoietic stem cell transplantation. We identified 123 patients with post-aplastic anemia myelodysplastic syndrome who from 1991 through 2011 underwent allogeneic hematopoietic stem cell transplantation, and in a matched-pair analysis compared outcome to that in 393 patients with de novo myelodysplastic syndrome. There was no difference in overall survival. There were no significant differences with regard to 5-year probabilities of relapse, non-relapse mortality, relapse-free survival and overall survival; these were 14%, 40%, 46% and 49% for post-aplastic anemia myelodysplastic syndrome, and 20%, 33%, 47% and 49% for de novo myelodysplastic syndrome, respectively. In multivariate analysis, relapse (hazard ratio 0.71; P=0.18), non-relapse mortality (hazard ratio 1.28; P=0.18), relapse-free survival (hazard ratio 0.97; P=0.80) and overall survival (hazard ratio 1.02; P=0.88) of post-aplastic anemia myelodysplastic syndrome were similar to those of patients with de novo myelodysplastic syndrome. Cytogenetic risk was independently associated with overall survival in both groups. Thus, transplant success in patients with post-aplastic anemia myelodysplastic syndrome was similar to that in patients with de novo myelodysplastic syndrome, and cytogenetics was the only significant prognostic factor for post-aplastic anemia myelodysplastic syndrome patients.

  15. Aplastic anemia associated to systemic lupus erythematosus in an AIDS patient: a case report.

    Science.gov (United States)

    de Oliveira, Leonardo Rodrigues; Ferreira, Thaís Camargos; Neves, Fernando de Freitas; Meneses, Antônio Carlos de Oliveira

    2013-01-01

    Aplastic anemia is a bone marrow failure syndrome characterized by peripheral cytopenias and hypocellular bone marrow. Although aplastic anemia is idiopathic in most cases, rheumatic diseases such as systemic lupus erythematosus are recognized as causes of aplastic anemia, with their possible etiological mechanisms being T and B lymphocyte dysfunction and pro-inflammatory cytokines and autoantibody production directed against bone marrow components. In the course of the human immunodeficiency virus infection/acquired immunodeficiency syndrome, the identification of autoantibodies and the occurrence of rheumatic events, in addition to the natural course of systemic lupus erythematosus which is modified by immune changes that are characteristic of human immunodeficiency virus infection/acquired immunodeficiency syndrome, make the diagnosis of systemic lupus erythematosus challenging. This study reports the case of a woman with acquired immunodeficiency syndrome treated with a highly active antiretroviral therapy, who had prolonged cytopenias and hypocellular bone marrow consistent with aplastic anemia. The clinical picture, high autoantibodies titers, and sustained remission of the patient's hematological status through immunosuppression supported the diagnosis of systemic lupus erythematosus-associated aplastic anemia. This is the first report of aplastic anemia concurrent with systemic lupus erythematosus and acquired immunodeficiency syndrome, providing additional evidence that immune dysfunction is a key part of the pathophysiological mechanism of aplastic anemia.

  16. Hypertrophic cranial pachymeningitis in a patient with aplastic anemia.

    Science.gov (United States)

    Asano, T; Hayashida, M; Ogawa, K; Adachi, K; Teramoto, A; Yamamoto, M

    1998-12-01

    We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T1- and T2-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/microl) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out. Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI. On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T1-weighted MRI images and was also enhanced by gadolinium. Cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia.

  17. Eltrombopag for the treatment of aplastic anemia: current perspectives

    Directory of Open Access Journals (Sweden)

    Lum SH

    2016-09-01

    Full Text Available Su Han Lum, John D GraingerDepartment of Blood and Marrow Transplant, Royal Manchester Children’s Hospital, Manchester, UK Abstract: Aplastic anemia (AA is a potential life-threatening hematopoietic stem cell (HSC disorder resulting in cytopenia. The mainstays of treatment for AA are definitive therapy to restore HSCs and supportive measures to ameliorate cytopenia-related complications. The standard definitive therapy is HSC transplantation for young and medically fit patients with suitable donors and immunosuppressive therapy (IST with antithymocyte globulin and cyclosporine for the remaining patients. A significant proportion of patients are refractory to IST or relapse after IST. Various strategies have been explored in these patients, including second course of antithymocyte globulin, high-dose cyclophosphamide, and alemtuzumab. Eltrombopag, a thrombopoietin mimetic, has recently emerged as an encouraging and promising agent for patients with refractory AA. It has demonstrated efficacy in restoring trilineage hematopoiesis, and this positive effect continues after discontinuation of the drug. There are ongoing clinical trials exploring the role of eltrombopag as a first-line therapy in moderate to severe AA and a combination of eltrombopag with IST in severe AA.Keywords: eltrombopag, aplastic anemia, thrombopoietin, c-Mpl receptors

  18. Acquired hemoglobin H disease in a patient with aplastic anemia evolving into acute myeloid leukemia

    Directory of Open Access Journals (Sweden)

    Maria Stella Figueiredo

    Full Text Available CONTEXT: The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia. CASE REPORT: We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin.

  19. A plasma microRNA signature as a biomarker for acquired aplastic anemia

    OpenAIRE

    Hosokawa, Kohei; Kajigaya, Sachiko; Feng, Xingmin; Desierto, Marie J.; Fernandez Ibanez, Maria del Pilar; Rios, Olga; Weinstein, Barbara; Scheinberg,Phillip; Townsley, Danielle M.; Young, Neal S.

    2017-01-01

    Aplastic anemia is an acquired bone marrow failure characterized by marrow hypoplasia, a paucity of hematopoietic stem and progenitor cells, and pancytopenia of the peripheral blood, due to immune attack on the bone marrow. In aplastic anemia, a major challenge is to develop immune biomarkers to monitor the disease. We measured circulating microRNAs in plasma samples of aplastic anemia patients in order to identify disease-specific microRNAs. A total of 179 microRNAs were analyzed in 35 plasm...

  20. Acute Lung Injury during Antithymocyte Globulin Therapy for Aplastic Anemia

    Directory of Open Access Journals (Sweden)

    Ewan Christopher Goligher

    2009-01-01

    Full Text Available The case of a 33-year-old man with aplastic anemia who experienced recurrent episodes of hypoxemia and pulmonary infiltrates during infusions of antithymocyte globulin (ATG is described. With the use of high-dose corticosteroids, the patient’s original episodes resolved, and were subsequently prevented before additional administrations of ATG. Rare reports of an association between ATG and acute lung injury are found in the literature, but this is the first report of successful steroid-supported re-exposure. Although the mechanism of ATG-related acute lung injury remains uncertain, it may be parallel to the mechanism of transfusion-related acute lung injury because the pathogenesis of the latter relies, in part, on antileukocyte antibodies. ATG-related toxicity should be included in the differential diagnosis of new, infusion-associated pulmonary infiltrates, and corticosteroids may be a useful therapeutic consideration in the management.

  1. Aplastic anemia as a feature of systemic lupus erythematosus: a case report and literature review.

    Science.gov (United States)

    Chalayer, Émilie; Ffrench, Martine; Cathébras, Pascal

    2015-06-01

    Peripheral cytopenias are common in systemic lupus erythematosus, but bone marrow involvement is rarely reported. Aplastic anemia is the result of immune-mediated destruction of hematopoietic stem cells causing pancytopenia and characterized by an empty bone marrow. This rare but serious disease has been described as an unusual manifestation of systemic lupus erythematosus. We reviewed the 25 cases published in the English language literature and discuss the clinical presentation, outcome, treatment, and pathophysiology of aplastic anemia as a complication of systemic lupus erythematosus. We report here the first case of aplastic anemia associated with systemic lupus erythematosus treated with an allogeneic hematopoietic stem cell transplant. Over one half of patients received concomitantly the diagnoses of systemic lupus erythematosus and aplastic anemia. No clinical or histological features can distinguish primary aplastic anemia from aplastic anemia occurring in systemic lupus erythematosus patients. The overall mortality is about 15% and corticosteroid-based therapy alone or in combination with other immunomodulatory drugs can restore bone marrow function. Systemic lupus erythematosus may be complicated by bone marrow involvement. The diagnosis of peripheral cytopenias should be confirmed by bone marrow aspiration. All these patients should receive cortisone as a first treatment. Plasma exchanges seem to have some efficacy. Other different immunomodulatory therapies were used with variable results.

  2. A plasma microRNA signature as a biomarker for acquired aplastic anemia.

    Science.gov (United States)

    Hosokawa, Kohei; Kajigaya, Sachiko; Feng, Xingmin; Desierto, Marie J; Fernandez Ibanez, Maria Del Pilar; Rios, Olga; Weinstein, Barbara; Scheinberg, Phillip; Townsley, Danielle M; Young, Neal S

    2017-01-01

    Aplastic anemia is an acquired bone marrow failure characterized by marrow hypoplasia, a paucity of hematopoietic stem and progenitor cells, and pancytopenia of the peripheral blood, due to immune attack on the bone marrow. In aplastic anemia, a major challenge is to develop immune biomarkers to monitor the disease. We measured circulating microRNAs in plasma samples of aplastic anemia patients in order to identify disease-specific microRNAs. A total of 179 microRNAs were analyzed in 35 plasma samples from 13 aplastic anemia patients, 11 myelodysplastic syndrome patients, and 11 healthy controls using the Serum/Plasma Focus microRNA Polymerase Chain Reaction Panel. Subsequently, 19 microRNAs from the discovery set were investigated in the 108 plasma samples from 41 aplastic anemia patients, 24 myelodysplastic syndrome patients, and 43 healthy controls for validation, confirming that 3 microRNAs could be validated as dysregulated (>1.5-fold change) in aplastic anemia, compared to healthy controls. MiR-150-5p (induction of T-cell differentiation) and miR-146b-5p (involvement in the feedback regulation of innate immune response) were elevated in aplastic anemia plasma, whereas miR-1 was decreased in aplastic anemia. By receiver operating characteristic curve analysis, we developed a logistic model with these 3 microRNAs that enabled us to predict the probability of a diagnosis of aplastic anemia with an area under the curve of 0.86. Dysregulated expression levels of the microRNAs became normal after immunosuppressive therapy at 6 months. Specifically, miR-150-5p expression was significantly reduced after successful immunosuppressive therapy, but did not change in non-responders. We propose 3 novel plasma biomarkers in aplastic anemia, in which miR-150-5p, miR-146b-5p, and miR-1 can serve for diagnosis and miR-150-5p for disease monitoring. Clinicaltrials.gov identifiers:00260689, 00217594, 00961064.

  3. Antecedent presentation of aplastic anemia in a patient with diffuse large B cell lymphoma

    Directory of Open Access Journals (Sweden)

    Chien-Ting Chen

    2016-12-01

    Full Text Available Immunological manifestation occasionally develops concurrently with lymphoid neoplasms, including immune thrombocytopenia and autoimmune hemolytic anemia, but rarely reported acquired aplastic anemia (AA. Here we present a female case of diffuse large B cell lymphoma (DLBCL with antecedent presentation of AA. Recovery of AA was noted after complete response to lymphoma treatment. Literature regarding this issue was reviewed.

  4. Splenic peliosis in a patient with aplastic anemia during danazol therapy.

    Science.gov (United States)

    Arai, Shunya; Asai, Takashi; Uozaki, Hiroshi; Hangaishi, Akira; Kanda, Yoshinobu; Motokura, Toru; Chiba, Shigeru; Kurokawa, Mineo

    2007-11-01

    We experienced a case of danazol-induced splenic peliosis. A 42-year-old woman had taken danazol for refractory aplastic anemia for 2 years. Her anemia gradually became aggravated, and the patient complained of intermittent left upper-abdominal pain. Because computed tomography scanning showed multiple blood-filled cavities in the enlarged spleen without intraperitoneal hemorrhage, a splenectomy was emergently performed. This report is the first of a case of splenic peliosis developing during danazol therapy for aplastic anemia. The possible association between the administration of anabolic steroids or danazol and the development of peliosis is discussed.

  5. Therapeutic effect of androgen therapy in a mouse model of aplastic anemia produced by short telomeres.

    Science.gov (United States)

    Bär, Christian; Huber, Nicolas; Beier, Fabian; Blasco, Maria A

    2015-10-01

    Aplastic anemia is a rare but life-threatening disorder characterized by cytopenia in at least two of the three blood lineages. A frequent feature of patients with aplastic anemia is that they have shorter telomeres than those of age-matched controls. Testosterone has been used for over half a century in the treatment of aplastic anemia. However, although remissions are frequent following hormone therapy, the molecular mechanism underlying the response to treatment has remained unknown. Here we explored the possibility that the recently described regulation of telomerase activity by sex hormones may be the mechanism responsible. To this end, we used a mouse model of aplastic anemia induced by short telomeres in the bone marrow compartment. We found that testosterone therapy results in telomerase up-regulation, improved blood counts, and a significant extension of life-span of these mice. Importantly, longitudinal follow-up studies revealed longer telomeres in peripheral blood in mice subjected to hormone treatment. Our results demonstrate that testosterone-mediated telomerase activation can attenuate or reverse aplastic anemia disease progression associated with the presence of short telomeres.

  6. Telomerase gene therapy rescues telomere length, bone marrow aplasia, and survival in mice with aplastic anemia.

    Science.gov (United States)

    Bär, Christian; Povedano, Juan Manuel; Serrano, Rosa; Benitez-Buelga, Carlos; Popkes, Miriam; Formentini, Ivan; Bobadilla, Maria; Bosch, Fatima; Blasco, Maria A

    2016-04-07

    Aplastic anemia is a fatal bone marrow disorder characterized by peripheral pancytopenia and marrow hypoplasia. The disease can be hereditary or acquired and develops at any stage of life. A subgroup of the inherited form is caused by replicative impairment of hematopoietic stem and progenitor cells due to very short telomeres as a result of mutations in telomerase and other telomere components. Abnormal telomere shortening is also described in cases of acquired aplastic anemia, most likely secondary to increased turnover of bone marrow stem and progenitor cells. Here, we test the therapeutic efficacy of telomerase activation by using adeno-associated virus (AAV)9 gene therapy vectors carrying the telomerase Tert gene in 2 independent mouse models of aplastic anemia due to short telomeres (Trf1- and Tert-deficient mice). We find that a high dose of AAV9-Tert targets the bone marrow compartment, including hematopoietic stem cells. AAV9-Tert treatment after telomere attrition in bone marrow cells rescues aplastic anemia and mouse survival compared with mice treated with the empty vector. Improved survival is associated with a significant increase in telomere length in peripheral blood and bone marrow cells, as well as improved blood counts. These findings indicate that telomerase gene therapy represents a novel therapeutic strategy to treat aplastic anemia provoked or associated with short telomeres.

  7. RELATIONSHIP BETWEEN HUMAN PARVOVIRUS B19 INFECTION AND APLASTIC ANEMIA

    Institute of Scientific and Technical Information of China (English)

    钱新宏; 郑跃杰; 张国成; 焦西英; 李佐华

    2001-01-01

    Objective. To explore the relationship between human parvovirus B 19 (HPV B 19) infection and aplastic anemia (AA) and to investigate the role of HPV B19 in the occurrence of AA.``Methods. The presence of HPV B19 DNA was detected in the peripheral blood samples of 60 patients with AA (children 38 and adults 22) by nested polymerase chain reaction (PCR) assay, and 30 healthy persons were selected as control.``Results. Sixteen (26. 7 % ) of 60 AA cases were HPV B19 DNA positive, while all the samples in the control group were negative for HPV B19 ( P = 0. 000914). Among the case group, the positive rates of HPV B19DNA were 21.4% (6 /28), 30.0% (3 / 10), 20.0% (1 / 5) and 35.3 % (6 / 17) in children acute AA (AAA), children chronic AA (CAA), adults AAA and adults CAA patients respectively, which were significantly higher than that in the control group. Furthermore, there was no remarkable difference between children AA and adults AA in the 16 HPV B19 DNA positive patients; neither was there between AAA and CAA.``Conclusions. HPV B19 infection is not only correlated with the occurrence of children AAA and CAA, but also with adults AAA and CAA, and might be an important viral cause for AA in humans.

  8. RELATIONSHIP BETWEEN HUMAN PARVOVIRUS B19 INFECTION AND APLASTIC ANEMIA

    Institute of Scientific and Technical Information of China (English)

    钱新宏; 郑跃杰; 张国成; 焦西英; 李佐华

    2001-01-01

    Objective. To explore the relationship between human parvovirus B19 (HPV B19) infection and aplastic anemia (AA) and to investigate the role of HPV B19 in the occurrence of AA. Methods. The presence of HPV B19 DNA was detected in the peripheral blood samples of 60 patients with AA (children 38 and adults 22) by nested polymerase chain reaction (PCR) assay, and 30 healthy persons were selected as control. Results. Sixteen (26. 7 % ) of 60 AA cases were HPV B19 DNA positive, while all the samples in the control group were negative for HPV B19 (P = 0. 000914). Among the case group, the positive rates of HPV B19 DNA were21.4% (6 /28), 30.0% (3 / 10), 20.0% (1 /5) and 35.3% (6 / 17) in children acute AA (AAA), children chronic AA (CAA), adults AAA and adults CAA patients respectively, which were significant-ly higher than that in the control group, Furthermore, there was no remarkable difference between children AA and adults AA in the 16 HPV B19 DNA positive patients; neither was there between AAA and CAA. Conclusions. HPV B19 infection is not only correlated with the occurrence of children AAA and CAA, but also with adults AAA and CAA, and might be an important viral cause for AA in humans.

  9. Alternative donor transplants for severe aplastic anemia: current experience.

    Science.gov (United States)

    Bacigalupo, Andrea; Sica, Simona

    2016-04-01

    Patients with acquired severe aplastic anemia (SAA), who lack a human leukocyte antigen (HLA) identical sibling donor (SIB), have two therapeutic options: immunosuppressive therapy with anti-thymocyte globulin (ATG) and cyclosporine (CsA), or a transplant from an alternative donor. In these patients, the current guidelines of the European Group for Blood and Marrow Transplantation (EBMT) call for a course of ATG + CsA first and transplantation in case of no response. The alternative donor source can be an unrelated donor (UD), a cord blood (CB) unit, or a family mismatched member, in most instances genetically HLA haplo-mismatched (HAPLO). In the present review, we will discuss recent results of transplants from matched UD and SIB donors, with significantly improved outcome, especially with UD in the past decade. We will also be looking at CB transplants, and the problems of limited stem cell dose. Finally HAPLO grafts have been explored in patients lacking or having rejected an unrelated or CB graft: early results seem encouraging, though the procedure should still be considered experimental.

  10. Deferasirox therapy in children with Fanconi aplastic anemia.

    Science.gov (United States)

    Tunç, Bahattin; Tavil, Betul; Karakurt, Neslihan; Yarali, Nese; Azik, Fatih Mehmet; Kara, Abdurrahman; Culha, Vildan; Ozkasap, Serdar

    2012-05-01

    Thirty-nine children with Fanconi aplastic anemia (FAA) have been followed up in our center between January 2008 and November 2010. Eight of these children (20%) with a transfusional iron overload had been undergoing deferasirox treatment during the study period. In the English literature, transfusional iron overload and the use of an iron chelator in children with FAA has not yet been evaluated. Here, we have presented the effectivity and tolerability of deferasirox in children with FAA and a transfusional iron overload. Before the deferasirox treatment, the mean serum ferritin level was 3377 ± 2200 ng/mL. After a mean 13.6-month treatment duration, the mean ferritin level decreased to 2274 ± 1300 ng/mL (Pdeferasirox treatment in patients with FAA. In our series, despite the low number of cases, nephrotoxicity and hepatotoxicity were common side effects instead of gastrointestinal disturbances reported in other studies. Deferasirox is an oral, easily applicable, and effective iron chelator; baseline hepatotoxicity and nephrotoxicity may increase the development of toxic side effects in children with FAA. Patients with FAA receiving deferasirox treatment should be followed up closely for these side effects.

  11. Circulating thrombopoietin levels in normal healthy blood donors and in aplastic anemia patients in relation to disease severity

    Directory of Open Access Journals (Sweden)

    Abhay Singh

    2015-01-01

    Full Text Available Background: Thrombopoietin (TPO is the key hematopoietic growth factor regulating the production of platelets from bone marrow megakaryocytes and maintaining platelet hemostasis. This study was done to find any relationship between the levels of thrombopoietin and the severity of disease in patients with aplastic anemia. Materials and Methods: Serum samples were collected from 52 patients with a confirmed diagnosis of aplastic anemia and 45 normal healthy blood donors of both sexes over a period of 2 years, and TPO was estimated by using commercially available TPO-specific-enzyme-linked immunosorbent assay. Results: The median TPO level of 1190 pg/ml (range 625-7651 pg/ml in aplastic anemia patients was significantly higher than the median TPO level of 121.1 pg/ml (81.25-237.7 pg/ml in normal healthy blood donors (P = 0.000. No significant difference was observed in TPO levels of male and female patients (P = 0.453. The median TPO concentrations observed in very severe aplastic anemia, severe aplastic anemia, and nonsevere aplastic anemia were 2765 pg/ml (range 625-6451 pg/ml, 1190 pg/ml (range 672.1-7651 pg/ml, and 1111.5 pg/ml (range 761.1-2289.2 pg/ml, respectively. TPO in patients of very severe aplastic anemia was significantly higher than patients of nonsevere aplastic anemia (P = 0.043, with no significant relation among rest of the groups. Discussion: TPO levels in aplastic anemia patients were significantly higher than in healthy blood donors; however, in aplastic anemia patients TPO levels were significantly higher only in patients with very severe disease.

  12. Hepatitis G Virus associated aplastic anemia: A recent case from Pakistan

    Directory of Open Access Journals (Sweden)

    Hussain Abrar

    2011-01-01

    Full Text Available Abstract Background Aplastic anemia (AA is a serious and rare disorder characterized by a hypocellular bone marrow. Hepatitis associated aplastic anemia (HAAA is a variant of aplastic anemia in which aplastic anemia follows an acute attack of hepatitis. Several reports have noted an association between HGV and hepatitis-associated aplastic anemia besides other hepatitis causing viruses. Case presentation A female girl of age 11 year with a history of loose motion for one month, vomiting for last 15 days and poor oral intake for last few days is reported here. The physical examination presents fever, pallor whereas bleeding, hepatomegaly, Splenomegaly and bruising were absent, abdominal ultrasonography confirmed the absence of hepatomegaly, Splenomegaly and lymphodenopathy. The laboratory investigation parameters were: haemoglobin 6.2 g/L, total leucocytes count 1.51, neutrophils 0.47%, absolute reticulocyte count 0.5%, Monocytes 0.16%, red cell count 3.2 mil/uL, Picked cell volume (PCV 30.13%, Mean Corpuscular Volume (MCV 78 fL, Mean Corpuscular Hemoglobin (MCH 26.3 pg. The liver enzymes were alanine aminotransferease (ALT 98 IU/L, aspartate aminotransferase (AST 114 IU/L. Serologic and molecular tests for hepatitis A, B, C, D, E, TTV, B19 were negative, whereas HGV RNA PCR test was found positive for hepatitis G virus. The bone marrow aspirate and trephine biopsy examination revealed hypo- cellularity, erythropoiesis, myelopoiesis and megakaryopoiesis. Conclusion HAAA is an uncommon but severe condition, which may occur following idiopathic cases of acute hepatitis. Our finding suggests the involvement of HGV in the development of aplastic anemia. In patients presenting with pancytopenia after an episode of acute hepatitis, the definitive diagnosis should be considered and confirmed by RT-PCR and if possible by bone marrow biopsy.

  13. Incidence of aplastic anemia and agranulocytosis in Latin America: the LATIN study

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    Nelson Hamerschlak

    Full Text Available CONTEXT AND OBJECTIVE: Aplastic anemia and agranulocytosis are rare but life-threatening disorders, often caused by drugs and other environmental exposures. Reported incidence of these diseases seems to vary between different geographic regions, and few data on their incidence are available for Latin American countries. The aim of this work is to determine the incidence of agranulocytosis and aplastic anemia in Brazil. DESIGN AND SETTING: Incidence study. Seven centers took part in the pilot phase, so as to represent all Brazilian regions. METHODS: Each center conducted an active search for new cases in a defined region by means of regular contacts with all hematologists, main clinical laboratories and clinicians in hospitals of the region. RESULTS: 74 patients with aplastic anemia and 16 with agranulocytosis were identified. Patients with agranulocytosis had a median age of 31 years (interquartile range, IQR: 12.5-48.2; 32.2% were male and 81.2% were white. The median age of aplastic anemia patients was 21 years (IQR 15.0-35.2; 62.2% were male, 50.0% were white and 39.2% mulatto. The incidence of agranulocytosis was estimated to be 0.5 cases per million individuals per year, ranging from 0.0 to 1.1 cases per million per year between regions. The incidence of aplastic anemia was 2.7 cases per million per year, ranging from 1.1 to 7.1 cases per million per year between regions. CONCLUSIONS: Aplastic anemia and agranulocytosis are rare diseases in Brazil. However, there is considerable variability in their incidences between different regions.

  14. Current outcome of HLA identical sibling versus unrelated donor transplants in severe aplastic anemia

    DEFF Research Database (Denmark)

    Bacigalupo, Andrea; Socié, Gerard; Hamladji, Rose Marie

    2015-01-01

    We have analyzed 1448 patients with acquired aplastic anemia grafted between 2005 and 2009, and compared outcome of identical sibling (n=940) versus unrelated donor (n=508) transplants. When compared to the latter, sibling transplants were less likely to be performed beyond 180 days from diagnosis.......04). In conclusion, in multivariate analysis, the outcome of unrelated donor transplants for acquired aplastic anemia, is currently not statistically inferior when compared to sibling transplants, although patients are at greater risk of acute and chronic graft-versus-host disease. The use of peripheral blood grafts...

  15. Aplastic anemia as a cause of death in a patient with glioblastoma multiforme treated with temozolomide

    Energy Technology Data Exchange (ETDEWEB)

    Kopecky, Jindrich; Priester, Peter; Slovacek, Ladislav; Petera, Jiri; Macingova, Zuzana [Dept. of Clinical Oncology and Radiotherapy, Charles Univ. Hospital and Faculty of Medicine in Hradec Kralove (Czech Republic); Kopecky, Otakar [Clinical Oncology, Regional Hospital Nachod (Czech Republic)

    2010-08-15

    Background: Standard treatment of glioblastoma multiforme consists of postoperative radiochemotherapy with temozolomide, followed by a 6-month chemotherapy. Serious hematologic complications are rarely reported. Case Report and Results: The authors present the case of a 61-year-old female patient with glioblastoma multiforme treated with external-beam radiation therapy and concomitant temozolomide. After completion of treatment, the patient developed symptoms of serious aplastic anemia that eventually led to death due to prolonged neutro- and thrombocytopenia followed by infectious complications. Conclusion: Lethal complications following temozolomide are, per se, extremely rare, however, a total of four other cases of aplastic anemia have been reported in the literature so far. (orig.)

  16. Differential diagnosis of myelodysplastic syndrome and aplastic anemia using MRI

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Seung Eun; Park, Jung Mi; Lee, Jae Mun; Kim, Ki Tae; Kim, Dong Wook; Kim, Chun Choo; Kim, Chun Yul; Shinn, Kyung Sub [Catholic University Medical College, Seoul (Korea, Republic of)

    1995-04-15

    To assess the patterns of myelodysplastic syndrome (MDS) and aplastic anemia (AA) on MRI of the spinal bone marrow and to find the differential points between the two groups. Fourteen patients with MDS (n=7) and AA (n=7) were studied using magnetic resonance imaging. Sagittal images from the lower thoracic and lumbar vertebral marrow were evaluated on T1-weighted and STIR images. Five distinct patterns of signal intensity of the T1-weighted and STIR images were classified. T1 and T2 relaxation times and T1 marrow/fat signal intensity ratio were measured and analyzed (t-test). The cellularity of bone marrow was evaluated on histologic slides. MDS showed homogeneously low signal intensity on T1WI and high signal intensity on STIR image, indicating hypercellular marrow, whereas AA showed relative high signal intensity on T1WI and low signal intensity on STIR image, representing fatty marrow. T1 and T2 relaxation time (T1 for MDS=750.26 msec {+-} 177.50, T1 for AA=413.21 msec {+-} 167.39 ({rho} < 0.000), T2 for MDS=91.86 msec {+-} 14.16, T2 for AA=81.44 msec {+-} 15.31 ({rho} < 0.001) and T1 marrow/fat signal intensity ratio (0.22 {+-} 0.048 in MDS, 0.30 {+-} 0.083 in AA ({rho} < 0.000) revealed statistically significant difference between the two groups. Although the marrow aspiration and needle biopsy are mandatory in hematologic disease for diagnosis, there are limited in assessing the change of total marrow mass. Therefore MRI of bone marrow might be useful in distinguishing MDS from AA because of its ability of representation of total marrow mass.

  17. Evaluation of severity in aplastic anemia by MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jeong Mi; Lim, Gye Yeon; Kim, Euy Neyng; Lee, Jae Mun; Kim, Dong Wook; Han, Chi Wha; Kim, Chun Choo [The Catholic University College of Medicine, Seoul (Korea, Republic of)

    1999-02-01

    To evaluate the role of bone marrow (BM) magnetic resonance (MR) imaging for assessment of the severity of aplastic anemia (AA). Eighty patients with AA, ranging in age from 16 to 44 years underwent MR imaging. Fifty four patients had clinically severe AA(SAA), while in 26 the condition was moderate(MAA). Sagittal T1-weighted images (T1WI) and short tau inversion recovery (STIR) images of lumbar vertebral BM were analysed. Bulk T1, T2 and rho values (msec) were also measured, with mixed sequences. Signal intensity (SI) on both T1WI and STIR was classified into four patterns according to the amount fatty marrow : pattern I, homogeneous fatty marrow ; 2, fatty marrow with focal cellular nodules ; 3, mixed fatty and cellular marrow ; 4, cellular marrow with focal fatty nodules. These SI patterns and bulk T1, T2 and rho values of the lumbar BM were compared with the clinical severity of AA. On both T1WI and STIR sequences, MR imaging of lumbar vertebral BM in patients with AA showed various SI patterns. Pattern 1, 2 and 3 were much frequently seen in the SAA group (48 of 54 patients on T1WI and 43 of 54 on STIR) and pattern 4 was common in the MAA group (16 of 26 patients on T1WI and 18 of 26 on STIR). The SI patterns of AA seen on both T1WI and STIR sequences closely correlated with clinical severity (x 2 test, p=0.0001). Bulk T1 value was significantly different between SAA and MAA (SAA : 382.82msec {+-} 113.91 ; MAA : 517.99msec {+-} 151.92 ; t test, p=0.0001). The SI pattern seen on MR imaging, and T1 relaxation time of lumbar spinal BM can be useful for assessing the severity of AA.

  18. Anemia Aplástica e Gravidez: Relato de Caso Aplastic Anemia and Pregnancy: A Case Report

    Directory of Open Access Journals (Sweden)

    Rosiane Alves de Sousa Teles

    2002-06-01

    Full Text Available A anemia aplástica é distúrbio caracterizado por pancitopenia e medula óssea hipocelular, com substituição gordurosa dos elementos e sem nenhum sinal de transformação maligna ou doença mieloproliferativa. Acomete geralmente adultos jovens e idosos, sem preferência sexual. A maioria dos casos é adquirida, mas pode ocorrer hereditariamente, por distúrbio molecular (anemia de Fanconi. A associação com gravidez é rara, estando relacionada com alta morbidade e mortalidade materna e fetal. Os autores descrevem o caso de uma paciente com anemia aplástica, diagnosticada previamente, cuja gestação complicou com infecção do trato urinário, doença hipertensiva específica da gestação e restrição de crescimento fetal, com parto prematuro eletivo. Apesar das condições adversas na gravidez e parto, mãe e recém-nascido tiveram evolução clínica satisfatória.Aplastic anemia is characterized by a circulating pancytopenia, hypocellularity, and fatty replacement of cellular marrow elements, without evidence of malignant transformation or myeloproliferative disease. It usually affects young and senior adults, without any sexual preference. Most cases of aplastic anemia are acquired, but the disease may also be inherited due to a molecular disorder (Fanconi's anemia. Aplastic anemia in pregnancy is an extremely rare condition with high maternal and fetal morbidity and mortality rates. The authors describe a case of a patient with previously diagnosed aplastic anemia, whose pregnancy was complicated with urinary tract infection, preeclampsia and fetal growth restriction, with elective preterm birth. In spite of the adverse conditions in pregnancy and delivery, mother and newborn had a satisfactory clinical evolution.

  19. Aloimmunity against HLA class I antigens in patients with myelodysplastic syndrome and aplastic anemia

    OpenAIRE

    2005-01-01

    Myelodysplastic syndrome (MDS) and aplastic anemia (AA) are two of the hematological disorders which present peripheral cytopenias, with extensive clinical manifestations that vary from slight anemia to severe pancytopenia; the latter requiring continuous transfusional reposition of red cell (RC) and platelet concentrates (PC), which can induce aloimunization in patients. Such patients can develop a post-transfusional refractory state, rendering further transfusions unviable. The objective of...

  20. Invasive sino-orbital mycosis in an aplastic anemia patient caused by Neosartorya laciniosa.

    Science.gov (United States)

    Malejczyk, Kathy; Sigler, Lynne; Gibas, Connie Fe C; Smith, Stephanie W

    2013-04-01

    We report the first case of Neosartorya laciniosa invasive sinusitis involving the orbit in an immunocompromised male with aplastic anemia. Treatment included surgical debridement with enucleation of the eye and combination voriconazole and micafungin therapy followed by voriconazole alone. The fungus was identified using sequencing of partial benA and calmodulin genes.

  1. Tacrolimus versus Cyclosporine after Hematopoietic Cell Transplantation for Acquired Aplastic Anemia.

    Science.gov (United States)

    Inamoto, Yoshihiro; Flowers, Mary E D; Wang, Tao; Urbano-Ispizua, Alvaro; Hemmer, Michael T; Cutler, Corey S; Couriel, Daniel R; Alousi, Amin M; Antin, Joseph H; Gale, Robert Peter; Gupta, Vikas; Hamilton, Betty K; Kharfan-Dabaja, Mohamed A; Marks, David I; Ringdén, Olle T H; Socié, Gérard; Solh, Melhem M; Akpek, Görgün; Cairo, Mitchell S; Chao, Nelson J; Hayashi, Robert J; Nishihori, Taiga; Reshef, Ran; Saad, Ayman; Shah, Ami; Teshima, Takanori; Tallman, Martin S; Wirk, Baldeep; Spellman, Stephen R; Arora, Mukta; Martin, Paul J

    2015-10-01

    Combinations of cyclosporine (CSP) with methotrexate (MTX) have been widely used for immunosuppression after allogeneic transplantation for acquired aplastic anemia. We compared outcomes with tacrolimus (TAC)+MTX versus CSP+MTX after transplantation from HLA-identical siblings (SIB) or unrelated donors (URD) in a retrospective cohort of 949 patients with severe aplastic anemia. Study endpoints included hematopoietic recovery, graft failure, acute graft-versus-host disease (GVHD), chronic GVHD, and mortality. TAC+MTX was used more frequently in older patients and, in recent years, in both SIB and URD groups. In multivariate analysis, TAC+MTX was associated with a lower risk of mortality in URD recipients and with slightly earlier absolute neutrophil count recovery in SIB recipients. Other outcomes did not differ statistically between the 2 regimens. No firm conclusions were reached regarding the relative merits of TAC+MTX versus CSP+MTX after hematopoietic cell transplantation for acquired aplastic anemia. Prospective studies would be needed to determine whether the use of TAC+MTX is associated with lower risk of mortality in URD recipients with acquired aplastic anemia.

  2. 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).

  3. Susceptibility to aplastic anemia is associated with HLA-DRB1*1501 in an aboriginal population in Sabah, Malaysia.

    Science.gov (United States)

    Dhaliwal, J S; Wong, Lily; Kamaluddin, Muhammad Amir; Yin, Lee Yin; Murad, Shahnaz

    2011-10-01

    The incidence of aplastic anemia is reported to be higher in Asia than elsewhere. We studied the frequency of human leukocyte antigen (HLA) DRB1 alleles in aplastic anemia patients from 2 genetically similar aboriginal groups, the Kadazan and the Dusun, and compared them with genetically matched community and hospital controls. HLA-DRB1*15 was significantly higher in the patients compared with controls (p = 0.005), confirming similar findings in Japanese and Caucasian studies. Further testing indicated a significantly higher frequency of HLA-DRB1*1501 in patients compared with controls (p = 0.0004) but no significant difference in the frequency of HLA-DRB1*1502. The high frequency of HLA-DRB1*15 in the Kadazan and Dusun population combined with the wide variety of environmental factors associated with aplastic anemia could be the reason for the elevated incidence of aplastic anemia in the Kadazan and Dusun in Sabah.

  4. Clinical observation of treating 62 patients with severe aplastic anemia failing in immunosuppressive therapy by integrative medicine

    Institute of Scientific and Technical Information of China (English)

    苏尔云

    2013-01-01

    Objective To explore treatment methods for patients with severe aplastic anemia(SAA) failing in immunosuppressive therapy(IST). Methods Totally 62 SAA patients failing in IST were treated by integrative medicine(IM).

  5. A rare association of celiac disease and aplastic anemia: case report of a child and review of literature.

    Science.gov (United States)

    Badyal, Rama Kumari; Sachdeva, Man Updesh Singh; Varma, Neelam; Thapa, Babu Ram

    2014-01-01

    An association between severe aplastic anemia and other autoimmune diseases is rare and has been described in adults for eosinophilic fasciitis, thymomas, systemic lupus erythematosus, and thyroid disorders. Herein we report a patient with celiac disease who was not strictly following a gluten-free diet and presented with progressive pallor, fever, and weakness of 1 month's duration. On investigation, he had pancytopenia, which on subsequent evaluation revealed aplastic anemia. An association between aplastic anemia and celiac disease has rarely been reported. To the best of author's knowledge, only 1 pediatric case of celiac disease associated with aplastic anemia has been published. This is the second report to suggest such an association in children.

  6. Anemia Due to Excessive Bleeding

    Science.gov (United States)

    ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ...

  7. [Heteroimmune hemolytic anemia associated with antilymphocyte globulin treatment in a patient with aplastic anemia].

    Science.gov (United States)

    Goldztein, S; Carreras Vescio, L A; Salamone, H J; Calahonra, R; Kohan, A I; Sánchez Avalos, J C

    1990-01-01

    A 24-year-old male patient with a severe aplastic anemia (SAA) was treated with equine-antilymphocyte globulin (ALG). As complication of this treatment he developed a severe heteroimmune hemolytic anemia mediated by anti-species pan-agglutinin antibodies present in ALG. In spite of the fact that ALG is absorbed with red-cell stroma and platelets to remove anti-erythrocyte and anti-platelet contaminating antibodies, often only partial absorption is achieved, and the remaining antibodies are passively acquired by the recipient. Neutropenia and especially thrombocytopenia are usual complications of this treatment, but it is also possible to detect anti-erythrocyte antibodies in the serum and on the red cells of those patients. However, the unusual severity of the hemolysis suffered by our patient, with a striking decrease of the hemoglobin levels (Fig. 1) can be ascribed to the administration of ALG at a time at which the hematocrit was close to normal as a result of the previous administration of anabolics. It is likely that in severely anemic patients, with a high transfusional demand, such a hemolytic episode may remain undetected. The patient acquired reactivity to the direct antiglobulin test, as well as the positive results of investigation of unexpected antibodies and compatibility testing can be accounted for by the fact that commercial antihuman globulin serum (AGS) contains antibodies reacting with a globulin component shared by human and horse sera. Neutralization of AGS with ALG administered to the patient removed those cross-reacting antibodies, making it possible to perform reliable transfusion compatibility testing and to rule out the eventual presence of hidden alloantibodies or warm autoantibodies. Neutralized Coombs serum maintained its human antiglobulin properties unaltered (Table 1).(ABSTRACT TRUNCATED AT 250 WORDS)

  8. Salvage therapy for severe aplastic anemia after allogenenic hematopoietic cell transplant

    Institute of Scientific and Technical Information of China (English)

    张静

    2014-01-01

    Objective To probe a practical salvage strategy for relapse or failure patients with severe aplastic anemia(SAA)after allogenenic hematopoietic cell transplant(allo-HSCT).Methods The clinical characteristics and initial treatments of allo-HSCT,and the responses of a novel salvage therapy of cyclosporine alternately combined with levamisole(CsA&LMS regimen)plus danazol(DNZ)in 2 patients were reviewed and evaluated.

  9. Paroxysmal nocturnal hemoglobinuria and telomere length predicts response to immunosuppressive therapy in pediatric aplastic anemia.

    Science.gov (United States)

    Narita, Atsushi; Muramatsu, Hideki; Sekiya, Yuko; Okuno, Yusuke; Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Yoshida, Nao; Wang, Xinan; Xu, Yinyan; Kawashima, Nozomu; Doisaki, Sayoko; Hama, Asahito; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Kobayashi, Masao; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

    2015-12-01

    Acquired aplastic anemia is an immune-mediated disease characterized by severe defects in stem cell number resulting in hypocellular marrow and peripheral blood cytopenias. Minor paroxysmal nocturnal hemoglobinuria populations and a short telomere length were identified as predictive biomarkers of immunosuppressive therapy responsiveness in aplastic anemia. We enrolled 113 aplastic anemia patients (63 boys and 50 girls) in this study to evaluate their response to immunosuppressive therapy. The paroxysmal nocturnal hemoglobinuria populations and telomere length were detected by flow cytometry. Forty-seven patients (42%) carried a minor paroxysmal nocturnal hemoglobinuria population. The median telomere length of aplastic anemia patients was -0.99 standard deviation (SD) (range -4.01-+3.01 SD). Overall, 60 patients (53%) responded to immunosuppressive therapy after six months. Multivariate logistic regression analysis identified the absence of a paroxysmal nocturnal hemoglobinuria population and a shorter telomere length as independent unfavorable predictors of immunosuppressive therapy response at six months. The cohort was stratified into a group of poor prognosis (paroxysmal nocturnal hemoglobinuria negative and shorter telomere length; 37 patients) and good prognosis (paroxysmal nocturnal hemoglobinuria positive and/or longer telomere length; 76 patients), respectively. The response rates of the poor prognosis and good prognosis groups at six months were 19% and 70%, respectively (P<0.001). The combined absence of a minor paroxysmal nocturnal hemoglobinuria population and a short telomere length is an efficient predictor of poor immunosuppressive therapy response, which should be considered while deciding treatment options: immunosuppressive therapy or first-line hematopoietic stem cell transplantation. The trial was registered in www.umin.ac.jp with number UMIN000017972.

  10. Successful treatment of pulmonary mucormycosis in a child with aplastic anemia

    OpenAIRE

    Ye. A. Nikitina; Klimko, N. N.; A. S. Kolbin; E. G. Boychenko; I. A. Garbuzova; M. V. Golubeva; T. S. Bogomolova

    2013-01-01

    Mucormycosis (zygomycosis) is a frequently fatal fungal infection in immunocompromized patients. We describe a case of a successfull treatment of pulmonary mucormycosis in 11-year-child with a very severe aplastic anemia. The diagnosis of invasive mycosis has been proved according to EORTC 2008 criteria. Computed tomography showed bilobar right-sided pulmonary infiltration. Lichtheimia corymbifera cultured from BAL. The child achieved complete clinical, laboratory and instrumental response on...

  11. Recombinant Human Thrombopoietin Treatment Promotes Hematopoiesis Recovery in Patients with Severe Aplastic Anemia Receiving Immunosuppressive Therapy

    OpenAIRE

    2015-01-01

    Objective. To assess the effectiveness of recombinant human thrombopoietin (rhTPO) in severe aplastic anemia (SAA) patients receiving immunosuppressive therapy (IST). Methods. Eighty-eight SAA patients receiving IST from January 2007 to December 2012 were included in this retrospective analysis. Of these, 40 subjects received rhTPO treatment (15000 U, subcutaneously, three times a week). rhTPO treatment was discontinued when the platelet count returned to normal range. Hematologic response, b...

  12. Effect of antithymocyte globulin source on outcomes of bone marrow transplantation for severe aplastic anemia.

    Science.gov (United States)

    Kekre, Natasha; Zhang, Ying; Zhang, Mei-Jie; Carreras, Jeanette; Ahmed, Parvez; Anderlini, Paolo; Atta, Elias Hallack; Ayas, Mouhab; Boelens, Jaap Jan; Bonfim, Carmem; Deeg, H Joachim; Kapoor, Neena; Lee, Jong-Wook; Nakamura, Ryotaro; Pulsipher, Michael A; Eapen, Mary; Antin, Joseph H

    2017-03-24

    For treatment of severe aplastic anemia, immunosuppressive therapy with horse antithymocyte globulin results in superior response and survival compared with rabbit antithymocyte globulin. This relative benefit may be different in the setting of transplantation as rabbit antithymocyte globulin results in more profound immunosuppression. We analyzed 833 severe aplastic anemia transplants between 2008 and 2013 using HLA-matched siblings (n=546) or unrelated donors (n=287) who received antithymocyte globulin as part of their conditioning regimen and bone marrow graft. There were no differences in hematopoietic recovery by type of antithymocyte globulin. Among recipients of HLA-matched sibling transplants, day 100 incidence of acute (17% versus 6%, p<0.001) and chronic (20% versus 9%, p<0.001) graft-versus-host disease were higher with horse compared to rabbit antithymocyte globulin. There were no differences in 3 year overall survival, 87% and 92%, p=0.76. Among recipients of unrelated donor transplants acute graft-versus-host disease was also higher with horse compared to rabbit antithymocyte globulin (42% versus 23%, p<0.001) but not chronic graft-versus-host disease (38% versus 32%, p=0.35). Survival was lower with horse antithymocyte globulin after unrelated donor transplantation, 75% versus 83%, p=0.02. These data support the use of rabbit antithymocyte globulin for bone marrow transplant conditioning for severe aplastic anemia.

  13. [Hepatitis E virus-associated aplastic anemia. Report of a case].

    Science.gov (United States)

    Zylberman, Marcelo; Turdó, Karina; Odzak, Andrea; Arcondo, Florencia; Altabert, Nancy; Munné, Silvina

    2015-01-01

    Hepatitis-associated aplastic anemia (HAAA) is a well-recognized clinical syndrome in which marrow failure follows the development of hepatitis; it can be observed in up to 5% in the aplastic anemia in West Europe and North American countries and 10% in the East Asia. Although hepatotropic and other viruses were suspected of causing HAAA, this hypothesis was rarely confirmed. Currently, the infection with hepatitis E virus represents the first cause of acute hepatitis in the world. Its genotype 3, the most frequent in Argentina and other Latin American countries, was associated with extrahepatic complications (renal, pancreatic, neurologic and hematologic). To our knowledge, only one case of hepatitis E virus-associated aplastic anemia has been previously reported, in Pakistan; the case presented here would be the first in Argentina. The patient was treated with thymoglobulin, cyclosporine, corticosteroids, filgastrim and transfusional support. She developed fungemia due to Candida tropicalis that remitted with equinocandins and therefore fever, pulmonary infiltrates and a solitary nodular cerebral image with serum galactomannan (DO index > 1.0 ng/ml) that resolved with voriconazol. She was discharged three months after her admission without transfusion requirements and normal hepatic values.With this in mind, it would be advisable to investigate hepatitis E (HEV) as a cause of HAAA in Argentina.

  14. Scintigraphic and Radiologic Findings of Pancake Kidney in a Patient with Fanconi Aplastic Anemia

    Directory of Open Access Journals (Sweden)

    Adem Maman

    2016-06-01

    Full Text Available In this case, we have presented that a patient has fankoni aplastic anemia with pancakes kidney in scintigraphy and ultrasonography. The patient is 10 years old and a girl who fanconi aplastic anemia had been diagnosed since three years. In physical examination her general status is good. There was not left hand thumb and she had double the distal phalanx in his right hand thumb in her inspection. We observed 2/6 sistolic murmur in cardiovascular system examınation. Other systems were natural. Abdominal ultrasonography was observed that both the kidney were ectopic location and fused view in the left lower quadrant. Similarly in Tc-99m DTPA and DMSA renal scintigraphy, both kidneys were fused and in the left hemipelvis. The right kidney function were significantly lower by comparison with the left kidney functions. Radiological imaging is necessary in patients with Fanconi aplastic anemia without present clinical symptoms. The renal ultrasonography is important for determining pancakes. In addition, static and dynamic renal scintigraphy plays an important role in revealing the functional status of the kidneys

  15. Listeria monocytogenes meningitis in an atomic bomb survivor receiving corticosteroid therapy for aplastic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Fujihara, Kazuo; Shida, Norihiko; Ohta, Michiya [Hiroshima Atomic Bomb Hospital (Japan)

    1995-12-01

    We report a case of successfully treated Listeria monocytogenes (Lm) meningitis in a atomic bomb survivor receiving steroid therapy for aplastic anemia. The patient was a 62-year-old woman and the past medical history included hypothyroidism due to radioiodide therapy for Basedow disease, breast cancer, aplastic anemia, steroid-induced diabetes mellitus, and pulmonary tuberculosis. At the time of onset, she was receiving corticosteroid, anabolic steroid, an H{sub 2}-blocker (famotidine), and other medication. Since she developed symptoms of meningitis when she visited our hospital for regular medical check-up for aplastic anemia, she was hospitalized and given antibiotic therapy, including ABPC, without delay. With this effective antibiotic therapy and successful management of the co-existing medical conditions, she was cured except for being a little euphoric. Lm meningitis is known to occur in aged and immunocompromised patients. Since most of the atomic bomb survivors are now aged and the prevalence of malignancy, diabetes mellitus, and other diseases which cause immunodeficiency have been rising year by year, Lm meningitis is one of the emergency neurologic conditions whose diagnosis should not be delayed in this population. (author).

  16. Multiparameter FLAER-based flow cytometry for screening of paroxysmal nocturnal hemoglobinuria enhances detection rates in patients with aplastic anemia.

    Science.gov (United States)

    Sachdeva, Man Updesh Singh; Varma, Neelam; Chandra, Dinesh; Bose, Parveen; Malhotra, Pankaj; Varma, Subhash

    2015-05-01

    Flow cytometry is the gold standard methodology for screening of paroxysmal nocturnal hemoglobinuria. In the last few years, proaerolysin conjugated with fluorescein (FLAER) has become an important component of antibody panel used for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clone. This study aimed to compare PNH clone detection by flow cytometry in the pre-FLAER era versus the FLAER era. This was a retrospective analysis of 4 years and included 1004 individuals screened for PNH clone, either presenting as hemolytic anemia or as aplastic anemia. In the pre-FLAER time period, the RBCs and neutrophils were screened with antibodies against CD55 and CD59. With the introduction of FLAER, neutrophils were screened with FLAER/CD24/CD15 and monocytes with FLAER/CD14/CD33 combination. A comparative analysis was done for detection of PNH clone in aplastic anemia patients versus non-aplastic anemia patients, as well as between pre-FLAER and FLAER era. Out of a total of 1004 individuals, 59 (5.8%) were detected to have PNH clone positivity. The frequency of PNH clone detected in aplastic anemia and non-aplastic anemia groups was 12.02 and 3.36%, respectively. The detection rate of PNH clone increased from 4.5% (32/711) in the pre-FLAER era to 9.2% (27/293) with the introduction of FLAER. However, this increase could be attributed to increased detection of PNH clone in the aplastic anemia group, which showed a significant increase from 8.3 to 18.2% after use of FLAER. In the non-aplastic group, PNH clone was detected with similar frequencies before and after use of FLAER (3.2 versus 3.8%, respectively). Mean PNH clone size was lower in the aplastic anemia group when compared with the non-aplastic group. RBCs always showed a lower clone size than neutrophils. PNH clone on neutrophils and monocytes was however similar. Inclusion of FLAER increases the sensitivity of the test which is especially useful in picking up small PNH clones in patients of aplastic anemia.

  17. Identification of novel microRNA signatures linked to acquired aplastic anemia.

    Science.gov (United States)

    Hosokawa, Kohei; Muranski, Pawel; Feng, Xingmin; Keyvanfar, Keyvan; Townsley, Danielle M; Dumitriu, Bogdan; Chen, Jichun; Kajigaya, Sachiko; Taylor, James G; Hourigan, Christopher S; Barrett, A John; Young, Neal S

    2015-12-01

    Emerging evidence indicates that microRNA control and modulate immunity. MicroRNA have not been investigated in acquired aplastic anemia, a T-cell-mediated immune disease. Analysis of 84 microRNA expression levels in CD4(+) and CD8(+) T cells of patients with aplastic anemia revealed concurrent down-regulation of miR-126-3p, miR-145-5p, miR-223-3p, and miR-199a-5p (>3-fold change, Paplastic anemia compared to other hematologic disorders. MiR-126-3p and miR-223-3p were down-regulated in CD4(+) T effector memory cells, and miR-126-3p, miR-145-5p, and miR-223-3p were down-regulated in CD8(+) T effector memory and terminal effector cells. Successful immunosuppressive therapy was associated with restoration to normal expression levels of miR-126-3p, miR-145-5p, and miR-223-3p (>2-fold change, Paplastic anemia patients, MYC and PIK3R2 were up-regulated and proved to be targets of miR-145-5p and miR-126-3p, respectively. MiR-126-3p and miR-145-5p knockdown promoted proliferation and increased interferon-γ and granzyme B production in both CD4(+) and CD8(+) T cells. Our work describes previously unknown regulatory roles of microRNA in T-cell activation in aplastic anemia, which may open a new perspective for development of effective therapy. Clinicaltrials.gov identifier: NCT 01623167.

  18. Outcome of aplastic anemia in adolescence: a survey of the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation.

    Science.gov (United States)

    Dufour, Carlo; Pillon, Marta; Passweg, Jakob; Socié, Gerard; Bacigalupo, Andrea; Franceschetto, Genny; Carraro, Elisa; Oneto, Rosi; Risitano, Antonio Maria; Peffault de Latour, Regis; Tichelli, André; Rovo, Alicia; Peters, Christina; Hoechsmann, Britta; Samarasinghe, Sujith; Kulasekararaj, Austin G; Schrezenmeier, Hubert; Aljurf, Mahmoud; Marsh, Judith

    2014-10-01

    We analyzed the outcome of 537 adolescents (age 12-18 years) with idiopathic aplastic anemia included in the database of the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation comparing: i) matched family donor hematopoietic stem cell transplantation performed as first-line treatment with ii) front-line immunosuppressive therapy not followed by subsequent transplant given for failure and with iii) hematopoietic stem cell transplantation performed after failed front-line immunosuppressive therapy. Overall survival was 86% in the matched family donor hematopoietic stem cell transplantation group, 90% in patients given front-line immunosuppressive alone (those who did not fail this treatment and who did not receive subsequent rescue with hematopoietic stem cell transplantation) and 78% in subjects who underwent hematopoietic stem cell transplantation post failed front-line immunosuppressive therapy (P=0.14). Event-free survival in the same groups was respectively 83%, 64% and 71% (P=0.04). Cumulative incidence of rejection was 8% in matched family donor hematopoietic stem cell transplantation and 9% in transplants post failed front-line immunosuppression (P=0.62). Cumulative incidence of acute graft-versus-host disease was 12% in matched family donor transplants and 18% in transplants post failed immunosuppression (P=0.18). Chronic graft-versus-host disease was higher in matched family donor hematopoietic stem cell transplantation (8%) than in transplants post failed immunosuppressive therapy (20%) (P=0.0009). Cumulative incidence of post-therapy malignancies was 0.7% in matched family donor transplantations, 7% in transplantations post failed immunosuppression and 21% after front-line immunosuppression (P=0.0017). In the whole cohort, under multivariate analysis, the diagnosis to treatment interval of two months or under positively affected overall survival whereas up-front immunosuppression alone (with no subsequent

  19. What Is Fanconi Anemia?

    Science.gov (United States)

    ... Living With Clinical Trials Links Related Topics Anemia Aplastic Anemia Blood and Bone Marrow Transplant Congenital Heart Defects ... red blood cells. FA is a type of aplastic anemia . In aplastic anemia, the bone marrow stops making ...

  20. Dysregulated miR34a/diacylglycerol kinase ζ interaction enhances T-cell activation in acquired aplastic anemia.

    Science.gov (United States)

    Sun, Yuan-Xin; Li, Hui; Feng, Qi; Li, Xin; Yu, Ying-Yi; Zhou, Li-Wei; Gao, Yan; Li, Guo-Sheng; Ren, Juan; Ma, Chun-Hong; Gao, Cheng-Jiang; Peng, Jun

    2017-01-24

    Acquired aplastic anemia is an idiopathic paradigm of human bone marrow failure syndrome, which involves active destruction of hematopoietic stem cells and progenitors by cytotoxic T cells in the bone marrow. Aberrant expression of microRNAs in T cells has been shown to lead to development of certain autoimmune diseases. In the present study, we performed a microarray analysis of miRNA expression in bone marrow CD3+ T cells from patients with aplastic anemia and healthy controls. Overexpression of miR34a and underexpression of its target gene diacylglycerol kinase (DGK) ζ in bone marrow mononuclear cells were validated in 41 patients and associated with the severity of aplastic anemia. Further, the level of miR34a was higher in naïve T cells from patients than from controls. The role of miR34a and DGKζ in aplastic anemia was investigated in a murine model of immune-mediated bone marrow failure using miR34a-/- mice. After T-cell receptor stimulation in vitro, lymph node T cells from miR34a-/- mice demonstrated reduced activation and proliferation accompanied with a less profound down-regulation of DGKζ expression and decreased ERK phosphorylation compared to those from wild-type C57BL6 control mice. Infusion of 5 × 106 miR34a-/- lymph node T cells into sublethally irradiated CB6F1 recipients led to increased Lin-Sca1+CD117+ cells and less vigorous expansion of CD8+ T cells than injection of same number of wild-type lymph node cells. Our study demonstrates that the miR34a/DGKζ dysregulation enhances T-cell activation in aplastic anemia and targeting miR34a may represent a novel molecular therapeutic approach for patients with aplastic anemia.

  1. Iron chelation therapy with deferasirox in patients with aplastic anemia: a subgroup analysis of 116 patients from the EPIC trial

    DEFF Research Database (Denmark)

    Lee, Jong Wook; Yoon, Sung-Soo; Shen, Zhi Xiang

    2010-01-01

    The prospective 1-year Evaluation of Patients' Iron Chelation with Exjade (EPIC) study enrolled a large cohort of 116 patients with aplastic anemia; the present analyses evaluated the efficacy and safety of deferasirox in this patient population. After 1 year, median serum ferritin decreased...... neutrophil and platelet counts remained stable during treatment, and there were no drug-related cytopenias. This prospective dataset confirms the efficacy and well characterizes the tolerability profile of deferasirox in a large population of patients with aplastic anemia. This study was registered at www...

  2. Study on abnormal iron metabolism and iron overload in patients with aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    金朋

    2013-01-01

    Objective To investigate the abnormalities of iron metabolism,the prevalence and risk factors of iron overload and clinical characteristics of patients with aplastic anemia (AA) .Methods A cross-sectional study was conducted on 520 newly diagnosed AA patients.Results Iron overload was observed in 66 (13%) of 520 AA patients,in which a higher prevalence of iron overload was seen not only in patients with infections (19/86,22%) than those without infections (47/434,11%,P<0.01) ,but also in patients with hepatitis associated AA

  3. Successful treatment of pulmonary mucormycosis in a child with aplastic anemia

    Directory of Open Access Journals (Sweden)

    Ye. A. Nikitina

    2014-07-01

    Full Text Available Mucormycosis (zygomycosis is a frequently fatal fungal infection in immunocompromized patients. We describe a case of a successfull treatment of pulmonary mucormycosis in 11-year-child with a very severe aplastic anemia. The diagnosis of invasive mycosis has been proved according to EORTC 2008 criteria. Computed tomography showed bilobar right-sided pulmonary infiltration. Lichtheimia corymbifera cultured from BAL. The child achieved complete clinical, laboratory and instrumental response on long-term amphotericin B lipid-formulated therapy combined with posaconazole.

  4. Treatment of Aplastic Anemia by the Kidney-Tonifying and Mediating Method

    Institute of Scientific and Technical Information of China (English)

    俞亚琴; 孙伟正; 曹克俭; 王祥祺

    2001-01-01

    In this series of 65 cases of aplastic anemia, 26 cases were treated by the kidney-tonifying and mediating method, 19 cases by western drugs, and the remaining 20 cases only by tonifying the kidney as controls. The results showed that the kidney-tonifying and mediating method was significantly superior in the total effective rate to the method of western drugs and that of tonifying the kidney alone (P<0.01), and it was also superior in the improvement of symptoms, myelogram, and peripheral blood count to the other two methods.

  5. Successful treatment of pulmonary mucormycosis in a child with aplastic anemia

    Directory of Open Access Journals (Sweden)

    Ye. A. Nikitina

    2013-01-01

    Full Text Available Mucormycosis (zygomycosis is a frequently fatal fungal infection in immunocompromized patients. We describe a case of a successfull treatment of pulmonary mucormycosis in 11-year-child with a very severe aplastic anemia. The diagnosis of invasive mycosis has been proved according to EORTC 2008 criteria. Computed tomography showed bilobar right-sided pulmonary infiltration. Lichtheimia corymbifera cultured from BAL. The child achieved complete clinical, laboratory and instrumental response on long-term amphotericin B lipid-formulated therapy combined with posaconazole.

  6. Deferasirox Decreases Liver Iron Concentration in Iron-Overloaded Patients with Myelodysplastic Syndromes, Aplastic Anemia and Other Rare Anemias.

    Science.gov (United States)

    Kohgo, Yutaka; Urabe, Akio; Kilinç, Yurdanur; Agaoglu, Leyla; Warzocha, Krzysztof; Miyamura, Koichi; Lim, Lay Cheng; Glaser, Sabine; Wang, Candace; Wiktor-Jedrzejczak, Wieslaw

    2015-01-01

    Iron overload in transfusion-dependent patients with rare anemias can be managed with chelation therapy. This study evaluated deferasirox efficacy and safety in patients with myelodysplastic syndromes (MDS), aplastic anemia (AA) or other rare anemias. A 1-year, open-label, multicenter, single-arm, phase II trial was performed with deferasirox (10–40 mg/kg/day, based on transfusion frequency and therapeutic goals), including an optional 1-year extension. The primary end point was a change in liver iron concentration (LIC) after 1 year. Secondary end points included changes in efficacy and safety parameters (including ophthalmologic assessments) overall as well as in a Japanese subpopulation. Overall, 102 patients (42 with MDS, 29 with AA and 31 with other rare anemias) were enrolled; 57 continued into the extension. Mean absolute change in LIC was –10.9 mg Fe/g dry weight (d.w.) after 1 year (baseline: 24.5 mg Fe/g d.w.) and –13.5 mg Fe/g d.w. after 2 years. The most common drug-related adverse event was increased serum creatinine (23.5%), predominantly in MDS patients. Four patients had suspected drug-related ophthalmologic abnormalities. Outcomes in Japanese patients were generally consistent with the overall population. Results confirm deferasirox efficacy in patients with rare anemias, including a Japanese subpopulation. The safety profile was consistent with previous studies and ophthalmologic parameters generally agreed with baseline values (EUDRACT 2006-003337-32).

  7. Hematopoietic Acute Radiation Syndrome (Bone marrow syndrome, Aplastic Anemia): Molecular Mechanisms of Radiation Toxicity.

    Science.gov (United States)

    Popov, Dmitri

    Key Words: Aplastic Anemia (AA), Pluripotential Stem Cells (PSC) Introduction: Aplastic Anemia (AA) is a disorder of the pluripotential stem cells involve a decrease in the number of cells of myeloid, erythroid and megakaryotic lineage [Segel et al. 2000 ]. The etiology of AA include idiopathic cases and secondary aplastic anemia after exposure to drugs, toxins, chemicals, viral infections, lympho-proliferative diseases, radiation, genetic causes, myelodisplastic syndromes and hypoplastic anemias, thymomas, lymphomas. [Brodskyet al. 2005.,Modan et al. 1975., Szklo et al. 1975]. Hematopoietic Acute Radiation Syndrome (or Bone marrow syndrome, or Radiation-Acquired Aplastic Anemia) is the acute toxic syndrome which usually occurs with a dose of irradiation between 0.7 and 10 Gy (70- 1000 rads), depending on the species irradiated. [Waselenko et al., 2004]. The etiology of bone morrow damage from high-level radiation exposure results depends on the radiosensitivity of certain bone marrow cell lines. [Waselenko et al. 2004] Aplastic anemia after radiation exposure is a clinical syndrome that results from a marked disorder of bone marrow blood cell production. [Waselenko et al. 2004] Radiation hematotoxicity is mediated via genotoxic and other specific toxic mechanisms, leading to aplasia, cell apoptosis or necrosis, initiation via genetic mechanisms of clonal disorders, in cases such as the acute radiation-acquired form of AA. AA results from radiation injury to pluripotential and multipotential stem cells in the bone marrow. The clinical signs displayed in reticulocytopenia, anemia, granulocytopenia, monocytopenia, and thrombocytopenia. The number of marrow CD34+ cells (multipotential hematopoietic progenitors) and their derivative colony-forming unit{granulocyte-macrophage (CFU-GM) and burst forming unit {erythroid (BFU{E) are reduced markedly in patients with AA. [Guinan 2011, Brodski et al. 2005, Beutler et al.,2000] Cells expressing CD34 (CD34+ cell) are normally

  8. Prospective study of rabbit antithymocyte globulin and cyclosporine for aplastic anemia from the EBMT Severe Aplastic Anaemia Working Party.

    Science.gov (United States)

    Marsh, Judith C; Bacigalupo, Andrea; Schrezenmeier, Hubert; Tichelli, Andre; Risitano, Antonio M; Passweg, Jakob R; Killick, Sally B; Warren, Alan J; Foukaneli, Theodora; Aljurf, Mahmoud; Al-Zahrani, H A; Höchsmann, Britta; Schafhausen, Philip; Roth, Alexander; Franzke, Anke; Brummendorf, Tim H; Dufour, Carlo; Oneto, Rosi; Sedgwick, Philip; Barrois, Alain; Kordasti, Shahram; Elebute, Modupe O; Mufti, Ghulam J; Socie, Gerard

    2012-06-07

    Rabbit antithymocyte globulin (rATG; thymoglobulin, Genzyme) in combination with cyclosporine, as first-line immunosuppressive therapy, was evaluated prospectively in a multicenter, European, phase 2 pilot study, in 35 patients with aplastic anemia. Results were compared with 105 age- and disease severity-matched patients from the European Blood and Marrow Transplant registry, treated with horse ATG (hATG; lymphoglobulin) and cyclosporine. The primary end point was response at 6 months. At 3 months, no patients had achieved a complete response to rATG. Partial response occurred in 11 (34%). At 6 months, complete response rate was 3% and partial response rate 37%. There were 10 deaths after rATG (28.5%) and 1 after subsequent HSCT. Infections were the main cause of death in 9 of 10 patients. The best response rate was 60% for rATG and 67% for hATG. For rATG, overall survival at 2 years was 68%, compared with 86% for hATG (P = .009). Transplant-free survival was 52% for rATG and 76% for hATG (P = .002). On multivariate analysis, rATG (hazard ratio = 3.9, P = .003) and age more than 37 years (hazard ratio = 4.7, P = .0008) were independent adverse risk factors for survival. This study was registered at www.clinicaltrials.gov as NCT00471848.

  9. Clinical Study of Shengxue Mixture (生血合剂) in Treating Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective: To observe the clinical effect of Shengxue Mixture (SXM) in treating aplastic anemia and study the possible mechanism. Methods: Eighty-four patients in the treated group with Spleen-Kidney Yang deficiency syndrome and Spleen-Kidney Yin deficiency syndrome were treated with SXM-Ⅰ and SXM-Ⅱ respectively, and 30 patients in the control group were treated with Stanozolol. The clinical effect and several experimental parameters were also observed. Results: The basic cure was gained in 18 cases, remission in 23, markedly improved in 32, ineffective 11, total effective rate and cure remission rate of the treated group were 86.90% and 48.81% respectively, which were obviously better than those of the control group (P<0.05) with no obvious side-effect. While the patient's symptoms were alleviated, the peripheral blood cells increased, the ratio of T lymphocyte subsets tended to balance, the level of natural killer cell activity increased, interleukin-2 reduced, and reproduction of the bone marrow were markedly improved in most of the patients treated by SXM. Conclusions: SXM is an effective and safe drug for aplastic anemia. Its mechanism might be likely due to its regulating the immune function, which facilitates the recovery of the bone marrow hematopoiesis function.

  10. Abnormalities of quantities and functions of natural killer cells in severe aplastic anemia.

    Science.gov (United States)

    Liu, Chunyan; Li, Zhishang; Sheng, Weiwei; Fu, Rong; Li, Lijuan; Zhang, Tian; Wu, Yuhong; Xing, Limin; Song, Jia; Wang, Huaquan; Shao, Zonghong

    2014-01-01

    Severe aplastic anemia (SAA) is a rare disease characterized by severe pancytopenia and bone marrow failure. Natural killer (NK) cells are large granular lymphocytes derived from hematopoietic stem cells (HSCs) or common lymphoid progenitors (CLP). They play a key role in n the innate immunity and adaptive immune. In this study, the quantitative and functional changes of natural killer (NK) cell subsets in peripheral blood of severe aplastic anemia (SAA) patients before and after immunosuppressive therapy (IST) were investigated. Results showed that the percentage of NK cells and its subsets in peripheral blood lymphocytes was decreased in SAA patients. After IST, the percentage of NK cells and their subsets increased dramatically. The median expressions of CD158a, NKG2D and NKp46 on NK cells were higher in SAA patients compared to that in normal controls, and the expressions of perforin in newly diagnosed and recovery SAA patients were higher than that in controls. Therefore, we concluded that the decrease of total NK cells, and CD56(bright), CD56(dim) NK cell subsets and the higher expressions of NKp46 and perforin on NK cells may cause the over-function of T lymphocytes and thus lead to hematopoiesis failure in SAA.

  11. Telomere length and gene expression of shelterin in CD3~+ T cell of severe aplastic anemia patients

    Institute of Scientific and Technical Information of China (English)

    王婷

    2013-01-01

    Objective To explore the changes in telomere length and gene expression of complex shelterin (composed of 6core components:TRF1,TRF2,POT1,TIN2,TPP1and RAP1) in severe aplastic anemia (SAA) .Methods Bone marrow samples were obtained from 20 SAA

  12. Short-term therapeutic effects of combined therapy with metformin hydrochloride for aplastic anemia

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    Xue-chun LU

    2012-03-01

    Full Text Available Objective To screen and select new drugs for aplastic anemia (AA and evaluate their clinical efficacy by clinical bioinformatics methods. Methods First, we established genome expression profiles of AA patients, and conducted similarity analyses with the pharmacogenomics database to screen and select drugs with possible efficacy. Intractable AA patients who received immunosuppressors and/or androgen for more than six months showing no clinical efficacy were enrolled in the study to evaluate therapeutic effects of the therapeutic regime. Clinical efficacy and adverse effects were evaluated after six months. Results The clinical bioinformatics results showed therapeutic effects of metformin hydrochloride on AA. Forty-three intractable AA patients (15 with severe AA were treated with metformin hydrochloride combined with cyclosporin A (CsA and stanozolol. Twenty-seven transfusion-dependent patients (100% became transfusion independent after a 6-month therapy. The hemoglobin level completely returned to normal in 37 out of 40 anemia patients (92.5%. In the 40 patients with platelet count lower than 20×109/L, the platelet count of 28 patients (90.3% increased to higher than 50×109/L. The white cell count increased to higher than 3.5×109/L in 30 out of 35 patients (88.6% with white cell count lower than 2.5×109/L. Among 40 anemic patients, 1 was found to have abnormal renal function, but it recovered to the normal range after ending CsA treatment. Eighteen patients were found to have elevated transaminase levels which were lowered to normal range after using liver protectants and reducing the dosage of stanozolol. There were no instances of hypoglycemia in all patients throughout the treatment. Conclusion Combination of metformin hydrochloride, CsA and stanozolol is effective in refractory aplastic anemia with acceptable toxicity.

  13. Management of aplastic anemia in a woman during pregnancy: a case report

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    Lugtenburg Pieternella J

    2011-02-01

    Full Text Available Abstract Introduction Aplastic anemia is a rare disease caused by destruction of pluripotent stem cells in bone marrow. During pregnancy it could be life-threatening for both mother and child. The only causal therapy for aplastic anemia is bone marrow transplantation, which is contraindicated during pregnancy because of potential embryo toxicity. Treatment options are erythrocytes and platelet transfusions and immunosuppressive therapy. There is, however, no agreement about the optimal supportive care and treatment regime for this disorder during pregnancy. Case Presentation A 26-year-old nulliparous Asian woman with an uneventful medical history was admitted to the hospital at 14 weeks' gestation because of excessive vomiting. Routine laboratory tests showed pancytopenia (Hb 3.5 mmol/L, leukocytes 3.5 *109/L, platelets 45 *109L. A bone marrow biopsy confirmed aplastic anemia. Methylprednisolon, cyclosporine A, packed cells and platelet transfusions were initiated. At 33 weeks she developed neutropenia (0.1 *109/L for which oral colistin and tobramycin were given prophylactically. At 35 weeks labor was induced, during which she developed a fever of 38.2°C. She gave birth spontaneously to a healthy son weighing 2415 grams, who had no signs of pancytopenia. After delivery the blood count of the patient did not recover and did not respond to medication. Eighteen weeks after delivery she died of sepsis complicated by cerebral bleeding and infarction due to severe thrombocytopenia and neutropenia, despite optimal supportive treatment. Conclusion This potential life-threatening disease has a relatively good prognosis for both mother and child after optimal treatment. Transfusion during pregnancy is the first choice treatment with recommended hemoglobin levels of >5.5 mmol/L and platelet counts of >20 *109/L. Cyclosporine A seems a reasonable alternative therapy with a reported success rate in non-pregnant patients of 70% when combined with

  14. Nonmyeloablative peripheral blood haploidentical stem cell transplantation for refractory severe aplastic anemia.

    Science.gov (United States)

    Clay, Jennifer; Kulasekararaj, Austin G; Potter, Victoria; Grimaldi, Francesco; McLornan, Donal; Raj, Kavita; de Lavallade, Hugues; Kenyon, Michelle; Pagliuca, Antonio; Mufti, Ghulam J; Marsh, Judith C W

    2014-11-01

    New transplant approaches are urgently needed for patients with refractory severe aplastic anemia (SAA) who lack a matched sibling or unrelated donor (UD) or who have failed UD or cord blood transplant. Patients with refractory SAA are at risk of later clonal evolution to myelodysplastic syndrome and acute leukemia. We report our pilot findings with haploidentical hematopoietic stem cell transplantation (haploHSCT) using uniform reduced-intensity conditioning with postgraft high-dose cyclophosphamide in 8 patients with refractory SAA or patients who rejected a prior UD or cord blood transplant. Six of 8 patients engrafted. Graft failure was associated with donor-directed HLA antibodies, despite intensive pre-HSCT desensitization with plasma exchange and rituximab. There was only 1 case of grade II skin graft-versus-host disease. We show that haploHSCT can successfully rescue refractory SAA patients who lack donor-directed HLA antibodies but not in the presence of donor-directed HLA antibodies. This novel protocol for haploHSCT for SAA has been adopted by the European Group for Blood and Marrow Transplantation Severe Aplastic Anaemia Working Party for a future noninterventional, observational study to further evaluate its efficacy.

  15. Fanconi Anemia — Case Report of Rare Aplastic Anemia at Child

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    Deaconu Alina

    2014-06-01

    Full Text Available Introduction: Fanconi anemia is an autosomal recessive disease characterized by congenital abnormalities, defective haematopoiesis, and a high risk of developing acute myeloid leukaemia, myelodysplastic syndrome and cancers. FA was first described in 1927 by the Swiss pediatrician Guido Fanconi. The diagnosis is based on morphological abnormalities, hematologic abnormalities (pancytopenia, macrocytic anemia and progressive bone marrow failure and genetic tests (cariograma.

  16. Aplastic Anemia & MDS International Foundation (AA&MDSIF): Bone Marrow Failure Disease Scientific Symposium 2014.

    Science.gov (United States)

    Visconte, Valeria; Lindsley, R Coleman; Berlyne, Deborah

    2015-01-01

    Bone marrow failure syndromes (BMFS) are characterized by a failure of the hematopoietic stem cells to produce adequate blood cells, resulting in either cytopenia (defect in one or more blood cell lineages) or pancytopenia (defect in all blood cell lineages). BMFS can be inherited or acquired. The pathogenesis of these diseases is very heterogeneous. Research efforts have been made all over the world to improve the basic knowledge of these diseases. The Aplastic Anemia and MDS International Foundation (AA&MDSIF) is an independent nonprofit organization whose mission is to help patients and family members cope with BMFS. Here, we summarize novel scientific discoveries in several BMFS that were presented at the 4th International Bone Marrow Failure Disease Scientific Symposium 2014 that AA&MDSIF sponsored on March 27-28, 2014, in Rockville, MD.

  17. Bone marrow mesenchymal stem cells from patients with aplastic anemia maintain functional and immune properties and do not contribute to the pathogenesis of the disease.

    Science.gov (United States)

    Bueno, Clara; Roldan, Mar; Anguita, Eduardo; Romero-Moya, Damia; Martín-Antonio, Beatriz; Rosu-Myles, Michael; del Cañizo, Consuelo; Campos, Francisco; García, Regina; Gómez-Casares, Maite; Fuster, Jose Luis; Jurado, Manuel; Delgado, Mario; Menendez, Pablo

    2014-07-01

    Aplastic anemia is a life-threatening bone marrow failure disorder characterized by peripheral pancytopenia and marrow hypoplasia. The majority of cases of aplastic anemia remain idiopathic, although hematopoietic stem cell deficiency and impaired immune responses are hallmarks underlying the bone marrow failure in this condition. Mesenchymal stem/stromal cells constitute an essential component of the bone marrow hematopoietic microenvironment because of their immunomodulatory properties and their ability to support hematopoiesis, and they have been involved in the pathogenesis of several hematologic malignancies. We investigated whether bone marrow mesenchymal stem cells contribute, directly or indirectly, to the pathogenesis of aplastic anemia. We found that mesenchymal stem cell cultures can be established from the bone marrow of aplastic anemia patients and display the same phenotype and differentiation potential as their counterparts from normal bone marrow. Mesenchymal stem cells from aplastic anemia patients support the in vitro homeostasis and the in vivo repopulating function of CD34(+) cells, and maintain their immunosuppressive and anti-inflammatory properties. These data demonstrate that bone marrow mesenchymal stem cells from patients with aplastic anemia do not have impaired functional and immunological properties, suggesting that they do not contribute to the pathogenesis of the disease.

  18. Epstein-Barr virus-related post-transplant lymphoproliferative disorder occurring after bone marrow transplantation for aplastic anemia in Down's syndrome.

    Science.gov (United States)

    Furuya, Aya; Ishida, Mitsuaki; Hodohara, Keiko; Yoshii, Miyuki; Okuno, Hiroko; Horinouchi, Akiko; Nakanishi, Ryota; Harada, Ayumi; Iwai, Muneo; Yoshida, Keiko; Kagotani, Akiko; Yoshida, Takashi; Okabe, Hidetoshi

    2014-01-01

    It is well established that Down's syndrome exhibits a predisposition to development of leukemia, however, association between aplastic anemia and Down's syndrome is exceptional. Herein, we describe a case of aplastic anemia occurring in Down's syndrome following post-transplant lymphoproliferative disorder (PTLD) after bone marrow transplantation (BMT). A 27-year-old Japanese male with Down's syndrome presented with a headache. Laboratory tests revealed severe pancytopenia, and bone marrow biopsy demonstrated hypocellular bone marrow with decrease of trilineage cells, which led to a diagnosis of aplastic anemia. One year after diagnosis, he was incidentally found to have an anterior mediastinal tumor, which was histopathologically diagnosed as seminoma. Subsequently, he received BMT from a female donor, and engraftment was observed. Three months after transplantation, he experienced cough and high fever. Biopsy specimen from the lung revealed diffuse proliferation of large-sized lymphoid cells expressing CD20 and EBER. These lymphoid cells had XY chromosomes. Thus, a diagnosis of EBV-associated PTLD was made. This is the seventh documented case of aplastic anemia occurring in Down's syndrome. Association between aplastic anemia and Down's syndrome has not been established, therefore, additional clinicopathological studies are needed. Moreover, this is the first case to undergo BMT for aplastic anemia in Down's syndrome. Although engraftment was observed, he developed EBV-positive PTLD. The neoplastic cells of the present case were considered to be of recipient origin, although the majority of PTLD cases with BMT are of donor origin.

  19. MR imaging of hematopoietic regions in bone marrow of aplastic anemia. Diagnostic usefulness of opposed phase T1-weighted images

    Energy Technology Data Exchange (ETDEWEB)

    Amano, Yasuo; Tanabe, Yoshihiro; Amano, Maki; Kumazaki, Tatsuo [Nippon Medical School, Tokyo (Japan)

    1996-01-01

    The signal intensity of hematopoietic regions in the marrow of aplastic anemia were investigated on opposed phase T1-weighted images (op-T1WI) with a 0.5-Tesla MR unit. Hematopoietic regions were classified into two groups: low intensity hematopoietic areas (LH) isointense to normal marrow and high intensity hematopoietic regions (HH) with higher intensity than normal marrow on op-T1WI. The signal intensity of LH was significantly lower than that of HH on STIR. LH converted into HH with improvement of laboratory data after therapy, whereas HH decreased with impairment of data. HH were hyperintense to cerebrospinal fluid on op-T1WI. These results indicated that the signal intensity of hematopoietic regions on op-T1WI reflected the cellularity in these regions and that aplastic anemia included hypercellular regions relative to normal marrow. (author).

  20. Quantitative and qualitative assessment of reactive hematopoietic bone marrow in aplastic anemia using MR spectroscopy with variable echo times

    Energy Technology Data Exchange (ETDEWEB)

    Amano, Yasuo; Kumazaki, Tatsuo [Department of Radiology, Nippon Medical School, Tokyo (Japan)

    2002-01-01

    Objective: To assess quantitative and qualitative differences in water components between normal bone marrow and reactive hematopoietic marrow in aplastic anemia using magnetic resonance (MR) spectroscopy with variable echo times (TEs). Design: Water content, T2 value of the water component, and signal change in water related to TE were assessed in normal bone marrow and reactive hematopoietic bone marrow by a stimulated echo acquisition mode with TEs of 30, 45, 60, and 90 ms. Patients: Six patients with aplastic anemia (13-84 years) and seven normal volunteers (25-38 years) were examined. Results and conclusion: Reactive hematopoietic marrow showed significantly higher water content than normal bone marrow. The T2 value of water components tended to be longer in reactive hematopoietic marrow. Water signal ratio related to TE was significantly higher in reactive hematopoietic marrow. These results suggest a quantitative and qualitative difference in water components between normal and reactive hematopoietic bone marrow. (orig.)

  1. Hematopoietic stem cell transplantation in children and young adults with secondary myelodysplastic syndrome and acute myelogenous leukemia after aplastic anemia.

    Science.gov (United States)

    Yoshimi, Ayami; Strahm, Brigitte; Baumann, Irith; Furlan, Ingrid; Schwarz, Stephan; Teigler-Schlegel, Andrea; Walther, Joachim-Ulrich; Schlegelberger, Brigitte; Göhring, Gudrun; Nöllke, Peter; Führer, Monika; Niemeyer, Charlotte M

    2014-03-01

    Secondary myelodysplastic syndrome and acute myelogenous leukemia (sMDS/sAML) are the most serious secondary events occurring after immunosuppressive therapy in patients with aplastic anemia. Here we evaluate the outcome of hematopoietic stem cell transplantation (HSCT) in 17 children and young adults with sMDS/sAML after childhood aplastic anemia. The median interval between the diagnosis of aplastic anemia and the development of sMDS/sAML was 2.9 years (range, 1.2 to 13.0 years). At a median age of 13.1 years (range, 4.4 to 26.7 years), patients underwent HSCT with bone marrow (n = 6) or peripheral blood stem cell (n = 11) grafts from HLA-matched sibling donors (n = 2), mismatched family donors (n = 2), or unrelated donors (n = 13). Monosomy 7 was detected in 13 patients. The preparative regimen consisted of busulfan, cyclophosphamide, and melphalan in 11 patients and other agents in 6 patients. All patients achieved neutrophil engraftment. The cumulative incidence of grade II-IV acute graft-versus-host disease (GVHD) was 47%, and that of chronic GVHD was 70%. Relapse occurred in 1 patient. The major cause of death was transplant-related complication (n = 9). Overall survival and event-free survival at 5 years after HSCT were both 41%. In summary, this study indicates that HSCT is a curative therapy for some patients with sMDS/sAML after aplastic anemia. Future efforts should focus on reducing transplantation-related mortality.

  2. Population pharmacokinetics of ciclosporin in Chinese children with aplastic anemia: effects of weight, renal function and stanozolol administration

    Institute of Scientific and Technical Information of China (English)

    Shao-qing NI; Wei ZHAO; Jue WANG; Su ZENG; Shu-qing CHEN; Evelyne JACQZ-AIGRAIN; Zheng-yan ZHAO

    2013-01-01

    Aim:To develop a population pharmacokinetic model for the immunosuppressant ciclosporin in Chinese children with aplastic anemia and to identify covariates influencing ciclosporin pharmacokinetics.Methods:A total of 102 children with either acquired or congenital aplastic anemia aged 8.8±3.6 years (range 0.9-17.6 years) were included.Therapeutic drug monitoring (TDM) data for ciclosporin were collected.The population pharmacokinetic model of ciclosporin was described using the nonlinear mixed-effects modeling (NONMEM) Vl software.The final model was validated using bootstrap and normalized prediction distribution errors.Results:A one-compartment model with first-order absorption and elimination was developed.The estimated CL/F was 15.1,which was lower than those of children receiving stem cell or kidney transplant reported in the West (16.9-29.3).The weight normalized CL/F was 0.45 (range:0.27-0.70) L.h-1·kg-1.The covariate analysis identified body weight,serum creatinine and concomitant administration of the anabolic steroid stanozolol as individual factors influencing the CL/F of ciclosporin.Conclusion:Our model could be used to optimize the ciclosporin dosing regimen in Chinese children with aplastic anemia.

  3. IMMUNOSUPPRESSIVE TREATMENT OF CHILDHOOD APLASTIC ANEMIA WITH ANTITHYMOCYTE GLOBULIN (ATG) AND CYCLOSPORIN A (CSA)

    Institute of Scientific and Technical Information of China (English)

    谢晓恬; 应大明; 王耀平; 姚慧玉; 林梓; 赵惠君

    1992-01-01

    This paper described the therapeutic efficacy of immunosuppressive (IS) treatment agents (ATG and CSA) and androgens in the treatment of childhood aplastic anemia (AA). The results showed that the overall curative rate was 52.4% in the ATG therapy group (21 cases) and 58.3% in the CSA therapy group (12 cases) respectively. The effective rate of all patients (SAA and CAA) was 58.1% in the IS group (18/31) and 40.4% in the androgens group (42/104), P>0.05. But, in the childhood patients with SAA, the clinical effective rate was 68.4% in the IS group and 7.9% in the androgens group, P<0.01. The laboratory tests revealed that the majority of the AA patients displalyed abnormal immunological states: inversed CD4/ CD8 ratio and increased IL-2 activity. These abnormal immunological states could be normalized in several patients when clinical response was abtained following IS therapy with ATG and CSA.

  4. Role of Stem Cell Factor and Its Receptor in the Pathogenesis of Pediatric Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    WANG Jun; FENG Jianfei; WANG Wei; HU Yu; ZHAO Xuelian; WANG Hong; ZHU Shaoxian

    2005-01-01

    In order to investigate the levels of stem cell factor (SCF) and its receptor c-kit protein and mRNA in pediatric aplastic anemia (AA) and their relevance to the pathogenesis, immunocytochemical and in situ hybridization were utilized to detect the expression of SCF and its receptor c-kit gene protein and mRNA, respectively in 59 children with AA and 51 normal controls. The relationship between SCF and c-kit and the pathogenesis of AA was analyzed subsequently. The results showed that the positive rate of SCF protein and mRNA expression in children with AA was significantly lower than that in healthy controls (P<0.05). However, there was no significant difference in the positive rate of c-kit protein and mRNA expression between children with AA and control group (P>0.05). It was concluded that the expression of SCF is significantly decreased in children with AA, which may be closely associated with the pathogenesis of the AA. c-kit may be unrelated to the development of pediatric AA. Therefore, AA in children may have abnormalities at SCF/ckit signal transduction levels.

  5. CLINICAL AND LABORATORY OBSERVATION ON 31 CHILDREN WITH APLASTIC ANEMIA TREATED BY SPATHOLOBUS SUBERECTUS COMPOSITA

    Institute of Scientific and Technical Information of China (English)

    苏尔云; 陈园妹; 史黎青; 陈辉树; 周永列

    2001-01-01

    To investigate the effect of Spatholobus Suberectus Composita (SSC) on bone marrow hematopoiesis in child aplastic anemia (AA).Methods 31 cases of AA children were treated with SSC for 24 months. The pathological changes of bone marrow and the amount of CD34 positive cells before or after treatment were compared. 14 cases of normal volunteers were enrolled as control.Results The overall response rate was 87.1% (27 in 31 patients). The volume of nucleated cell in bone marrow (27.4±9.9vol% before treatment vs 44.6±31.5 vol% after treatment), granulocytes (36.0±9.9% vs 46.2±12.9%), megakaryocytes(3.3±7.2 vs 28.5±40.5 cells in bone marrow sections) and CD34+ cells (0.9±0.5% vs 2.0±0.7%) significantly increased after treatment compared with those before treatment (P<0.01 or P<0.05).Conclusion SSC has stimulative effect, which possibly acts at the level of stem/progenitor cells, on the bone marrow hematopoesis.

  6. [Clinical study of TCM-WM on aplastic anemia complicated with hepatitis C].

    Science.gov (United States)

    Liu, Q C; Zheng, B R; Zhang, C L

    1995-04-01

    The testing kit of second generation for serum anti-HCV was used in 82 cases of aplastic anemia (AA). The results showed that positive rate was 69.4% (43/62) in the patients of AA with transfusion, this was significantly higher than that in the patients of AA without transfusion. There was no difference of anti-HCV antibody positive rate between chronic AA and acute AA (P > 0.05), incidence rate of post-transfusion hepatitis C (PTHC) in AA was 33.9% (21/62), among which the incidence rate in acute and chronic AA were 68.8% (11/16) and 21.7% (10/46) respectively (P PTHC and non-PTHC, there was no statistical difference of their transfusion volume, hemoglobin, white blood cell between these groups. Response rate of AA was lower in anti-HCV positive patients than that in negative patients (P PTHC was predominant in patients with AA. The patients with AA complicated with PTHC was liable to bleed and be infected. PTHC has been an important complication in patients with AA. The better response was obtained by TCM-WM therapy in the patients.

  7. Distinct Overexpression of Fas Ligand on T Lymphocytes in Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    Wenxin Li; Jinxiang Fu; Fengming Wang; Gehua Yu; Yong Wang; Xueguang Zhang

    2004-01-01

    Increased expression of Fas by hematopoietic progenitors in aplastic anemia (AA) suggests that Fas/Fas ligand (FasL) system plays a key role in the formation of severe pancytopenia. To further confirm the above hypothesis, T cells from 8 patients with AA were systematically studied for their FasL's distribution pattern,releasing manner and proapoptotic activity, compared with normal resting T cells and artificially activated Tcell blasts. The results demonstrated that AA T cells abnormally expressed low levels of membrane-bound FasL and contained high levels of intracellular FasL which could be triggered to release by high-dose phytohemagglutinin (PHA) pulse-stimulation. The supernatants from the PHA-stimulated AA T cells had apparent cytotoxicity against FasL-sensitive Jurkat cells, which could be significantly inhibited by monoclonal antibody against FasL in a dose-dependent manner, or nearly completely abrogated by ultracentrifugation. The above phenomena also appeared on artificially activated T cell blasts, but this was not the case on normal resting Tcells. These results indicate that AA T cell is a type of "preactivated" T lymphocyte, characterized by overexpression of FasL, especially intracellular FasL which can be stimulated to release in bioavtive exosomesbound form. Taken together, our data provide further and direct evidence for the hypothesis that T cells might mediate the destruction of hematopietic progenitor in AA through Fas/FasL system.

  8. Identification of novel regulators in T-cell differentiation of aplastic anemia patients

    Directory of Open Access Journals (Sweden)

    Probst-Kepper Michael

    2006-10-01

    Full Text Available Abstract Background Aplastic anemia (AA is a bone marrow failure syndrome mostly characterized by an immune-mediated destruction of marrow hematopoietic progenitor/stem cells. The resulting hypocellularity limits a detailed analysis of the cellular immune response. To overcome this technical problem we performed a microarray analysis of CD3+ T-cells derived from bone marrow aspirates and peripheral blood samples of newly diagnosed AA patients and healthy volunteers. Two AA patients were additionally analyzed after achieving a partial remission following immunosuppression. The regulation of selected candidate genes was confirmed by real-time RT-PCR. Results Among more than 22.200 transcripts, 583 genes were differentially expressed in the bone marrow of AA patients compared to healthy controls. Dysregulated genes are involved in T-cell mediated cytotoxicity, immune response of Th1 differentiated T-cells, and major regulators of immune function. In hematological remission the expression levels of several candidate genes tend to normalize, such as immune regulators and genes involved in proinflammatory immune response. Conclusion Our study suggests a pivotal role of Th1/Tc1 differentiated T-cells in immune-mediated marrow destruction of AA patients. Most importantly, immune regulatory genes could be identified, which are likely involved in the recovery of hematopoiesis and may help to design new therapeutic strategies in bone marrow failure syndromes.

  9. llogeneic peripheral blood stem cell transplantation in the treatment of severe aplastic anemia and severe infection

    Institute of Scientific and Technical Information of China (English)

    万理萍; 颜式可; 王椿; 杨新潮; 周柱; 高彦荣; 蔡琦; 张冰

    2003-01-01

    Objective To investigate the efficacy of allogeneic peripheral blood stem cell transplantation (PBSCT) in the treatment of severe aplastic anemia (SAA) and severe infection. Methods A patient with SAA and pseudomonas aeruginosa septicemia was treated with PBSCT from an HLA-identical sibling with cyclophosphamide (CY) and total body irradiation (TBI) for conditioning. The patient was infused with 20.3×108/kg mononuclear cells including 61.0×106/kg CD34+cells following the conditioning regimen. Results Twenty days after PBSCT, the absolute neutrophil count (ANC) of 1.0×109/L was achieved, with platelet count >50×109/L. The donor origin of engraftment was confirmed by polymerase chain reaction (PCR) analysis of short tandem repeats at the end of the first, sixth and twelfth month. The patient's body temperature dropped to normal level when her ANC reached 0.5×109/L on day 10, and the bacterial culture of blood sample became negative subsequently. Symptoms and signs of acute or chronic graft versus host disease (GVHD) were not observed in 30 months after PBSCT. Conclusions Hematopoiesis was reconstituted shortly after PBSCT. The combination of CY and TBI and the infusion of sufficient peripheral blood stem cells may contribute to the successful engraftment. PBSCT may be considered as the first choice when hematopoietic stem cell transplantation is needed for SAA patients complicated with severe infection.

  10. Bone marrow transplantation versus immunosuppressive therapy in patients with acquired severe aplastic anemia.

    Science.gov (United States)

    Bacigalupo, Andrea; Giammarco, Sabrina; Sica, Simona

    2016-08-01

    Standard front-line treatment for acquired aplastic anemia (AA) for patients is either immunosuppressive therapy (IST) or bone marrow transplantation (BMT), usually from an HLA identical sibling. Whereas long-term survival is comparable with either treatment, important differences remain: IST patients may have incomplete or no recovery, are exposed to late clonal disorders and relapse of the original disease. Transplantation is a curative treatment, but patients are exposed to transplant-related complications both acute and chronic, such as chronic graft versus host disease (cGvHD). In the year 2000, a study by the European Group for Blood and Marrow Transplantation (EBMT), looked at failure free survival (FFS), in patients receiving first-line BMT from an HLA identical sibling, or the first-line IST. Young patients with low neutrophil counts benefited of the first-line BMT; the opposite was true for older patients with higher neutrophil counts; and a third intermediate group of patients had comparable survival irrespective of the first-line therapy. We have now studied a more recent cohort of patients to assess whether things have changed over the years. We have found similar results, although overall survival has improved, as a consequence of changes in the IST and BMT protocols.

  11. Recombinant Human Thrombopoietin Treatment Promotes Hematopoiesis Recovery in Patients with Severe Aplastic Anemia Receiving Immunosuppressive Therapy

    Directory of Open Access Journals (Sweden)

    Huaquan Wang

    2015-01-01

    Full Text Available Objective. To assess the effectiveness of recombinant human thrombopoietin (rhTPO in severe aplastic anemia (SAA patients receiving immunosuppressive therapy (IST. Methods. Eighty-eight SAA patients receiving IST from January 2007 to December 2012 were included in this retrospective analysis. Of these, 40 subjects received rhTPO treatment (15000 U, subcutaneously, three times a week. rhTPO treatment was discontinued when the platelet count returned to normal range. Hematologic response, bone marrow megakaryocyte recovery, and time to transfusion independence were compared. Results. Hematologic response was achieved in 42.5%, 62.5%, and 67.5% of patients receiving rhTPO and 22.9%, 41.6%, and 47.9% of patients not receiving rhTPO at 3, 6, and 9 months after treatment, respectively (P = 0.0665, P = 0.0579, and P = 0.0847, resp.. Subjects receiving rhTPO presented an elevated number of megakaryocytes at 3, 6, and 9 months when compared with those without treatment (P = 0.025, P = 0.021, and P = 0.011, resp.. The time to platelet and red blood cell transfusion independence was shorter in patients who received rhTPO than in those without rhTPO treatment. Overall survival rate presented no differences between the two groups. Conclusion. rhTPO could improve hematologic response and promote bone marrow recovery in SAA patients receiving IST.

  12. Th17 immune responses contribute to the pathophysiology of aplastic anemia.

    Science.gov (United States)

    de Latour, Regis Peffault; Visconte, Valeria; Takaku, Tomoiku; Wu, Colin; Erie, Andrew J; Sarcon, Annahita K; Desierto, Marie J; Scheinberg, Phillip; Keyvanfar, Keyvan; Nunez, Olga; Chen, Jichun; Young, Neal S

    2010-11-18

    T helper type 17 (Th17) cells have been characterized based on production of interleukin-17 (IL-17) and association with autoimmune diseases. We studied the role of Th17 cells in aplastic anemia (AA) by isolating Th17 cells from patients blood (n = 41) and bone marrow (BM) mononuclear cells (n = 7). The frequency and total number of CD3(+)CD4(+)IL-17-producing T cells were increased in AA patients at presentation compared with healthy controls (P = .0007 and .02, respectively) and correlated with disease activity. There was an inverse relationship between the numbers of Th17 cells and CD4(+)CD25(high)FoxP3(+) regulatory T cells (Tregs) in the blood of AA patients. Concomitant with the classical Th1 response, we detected the presence of CD4(+) and CD8(+) IL-17-producing T cells in a mouse model of lymph node infusion-induced BM failure. Although anti-IL-17 treatment did not abrogate BM failure, early treatment with the anti-IL-17 antibody reduced the severity of BM failure with significantly higher platelet (P < .01) and total BM cell (P < .05) counts at day 10. Recipients that received anti-IL-17 treatment had significantly fewer Th1 cells (P < .01) and more Treg cells (P < .05) at day 10 after lymph node infusion. Th17 immune responses contribute to AA pathophysiology, especially at the early stage during disease progression.

  13. Outcome of a novel immunosuppressive strategy of cyclosporine, levamisole and danazol for severe aplastic anemia.

    Science.gov (United States)

    Wang, Min; Li, Xingxin; Shi, Jun; Shao, Yingqi; Ge, Meili; Huang, Jinbo; Huang, Zhendong; Zhang, Jing; Nie, Neng; Zheng, Yizhou

    2015-08-01

    Treatment options for patients with severe aplastic anemia (SAA) in developing countries are limited. A cohort of 261 patients with SAA received a novel immunosuppressive strategy of cyclosporine alternately combined with levamisole plus danazol (CSA&LMS-based regimen), which included 70 VSAA and 191 moderate SAA [initial absolute neutrophil count (ANC) >200/μL] cases. The CSA&LMS-based regimen was administrated orally with an initial dose of CSA 3 mg/kg in adults and 5 mg/kg in children every other day, LMS 150 mg in adults and 2.5 mg/kg in children every other day, and danazol (5.0-10.0) mg/kg daily, continued for 12 more months, followed by slow tapering. The 6-month response rates were 24.3 and 52.9 % for VSAA and moderate SAA (P < 0.001), respectively. Univariate and multivariate analyses demonstrated that younger age, higher pretreatment absolute reticulocyte count and ANC were favorable factors for achieving response at 6 months. The estimated 5-year overall survival rates were 33.8 % (95 % CI 20.6-47 %) and 80.5 % (95 % CI 69.7-91.3 %) for VSAA and moderate SAA, respectively (P < 0.001). To date, nine patients relapsed, and six patients evolved to clonal disorders. Thus, CSA&LMS-based regimen may represent a promising immunosuppressive strategy for moderate SAA.

  14. Peripheral blood lymphocyte telomere length as a predictor of response to immunosuppressive therapy in childhood aplastic anemia.

    Science.gov (United States)

    Sakaguchi, Hirotoshi; Nishio, Nobuhiro; Hama, Asahito; Kawashima, Nozomu; Wang, Xinan; Narita, Atsushi; Doisaki, Sayoko; Xu, Yinyan; Muramatsu, Hideki; Yoshida, Nao; Takahashi, Yoshiyuki; Kudo, Kazuko; Moritake, Hiroshi; Nakamura, Kazuhiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

    2014-08-01

    Predicting the response to immunosuppressive therapy could provide useful information to help the clinician define treatment strategies for patients with aplastic anemia. In our current study, we evaluated the relationship between telomere length of lymphocytes at diagnosis and the response to immunosuppressive therapy in 64 children with aplastic anemia, using flow fluorescence in situ hybridization. Median age of patients was ten years (range 1.5-16.2 years). Severity of the disease was classified as very severe in 23, severe in 21, and moderate in 20 patients. All patients were enrolled in multicenter studies using antithymocyte globulin and cyclosporine. The response rate to immunosuppressive therapy at six months was 52% (33 of 64). The probability of 5-year failure-free survival and overall survival were 56% (95% confidence interval (CI): 41-69%) and 97% (95%CI: 87-99%), respectively. Median telomere length in responders was -0.4 standard deviation (SD) (-2.7 to +3.0 SD) and -1.5 SD (-4.0 to +1.6 (SD)) in non-responders (Paplastic anemia.

  15. Pre-existing anti-HLA antibodies negatively impact survival of pediatric aplastic anemia patients undergoing HSCT.

    Science.gov (United States)

    Zhu, Hua; He, Jun; Cai, Junchao; Yuan, Xiaoni; Jiang, Hua; Luo, Changying; Wang, Jianmin; Luo, Chengjuan; Pan, Zhijuan; Terasaki, Paul I; Ding, Lixia; Chen, Jing

    2014-11-01

    Graft failure and survival are the major problems for patients with aplastic anemia undergoing hematopoietic stem cell transplantation (HSCT). Previous studies showed that anti-HLA antibodies negatively impact engraftment in HSCT. This retrospective study of 51 pediatric patients with acquired aplastic anemia who underwent allogeneic HSCT at a single institution between 2006 and 2012 investigated the influence of anti-HLA antibodies on the outcome of HSCT. Serum samples collected before HSCT were tested for the presence of anti-HLA antibodies. Pre-existing anti-HLA antibodies were detected in 54.9% (28/51) of patients, among whom 39.2% (20/51) had anti-HLA class I antibodies. Anti-HLA antibodies were associated with worse five-yr survival (78.6% vs. 100%, p = 0.021) and higher treatment-related mortality (21.4% vs. 0%, p = 0.028) compared with antibody-negative patients. Anti-HLA class I antibody-positive patients had poorer five-yr survival (75.0%) than anti-HLA class I&II antibody-positive and antibody-negative patients (87.5% and 100.0%, respectively, p = 0.039). Presence of anti-HLA class I antibodies (p = 0.024) and older age (10 yr or more; p = 0.027) significantly increased the risk of post-HSCT mortality. Pre-existing anti-HLA antibodies negatively affect the outcome of HSCT in pediatric patients with aplastic anemia. Routine testing for anti-HLA antibodies concurrent with efficient treatment should be conducted prior to HSCT.

  16. Iron chelation therapy with deferasirox in patients with aplastic anemia: a subgroup analysis of 116 patients from the EPIC trial

    DEFF Research Database (Denmark)

    Lee, Jong Wook; Yoon, Sung-Soo; Shen, Zhi Xiang;

    2010-01-01

    adjustments and ongoing iron intake. Baseline labile plasma iron levels were within normal range despite high serum ferritin levels. The most common drug-related adverse events were nausea (22%) and diarrhea (16%). Serum creatinine increases more than 33% above baseline and the upper limit of normal occurred...... neutrophil and platelet counts remained stable during treatment, and there were no drug-related cytopenias. This prospective dataset confirms the efficacy and well characterizes the tolerability profile of deferasirox in a large population of patients with aplastic anemia. This study was registered at www...

  17. Ocular manifestations of idiopathic aplastic anemia: retrospective study and literature review

    Directory of Open Access Journals (Sweden)

    Mansour AM

    2014-04-01

    Full Text Available Ahmad M Mansour,1 Jong Wook Lee,2 Seung Ah Yahng,2 Kyu Seop Kim,3 Maha Shahin,4 Nelson Hamerschlak,5 Rubens N Belfort,6 Shree K Kurup71Department of Ophthalmology, American University of Beirut, Rafic Hariri University Hospital, Beirut, Lebanon; 2Division of Hematology, Seoul St Mary’s Hospital, The Catholic University of Korea, Seoul, Republic of Korea; 3Department of Ophthalmology, Seoul St Mary’s Hospital, The Catholic University of Korea, Seoul, Republic of Korea; 4Department of Ophthalmology, Mansoura University, Mansoura City, Egypt; 5Oncology and Hematology Program, Instituto Israelita de Ensino e Pesquisa Albert Einstein, São Paulo, Brazil; 6Vision Institute, Hospital São Paulo, Federal University of São Paulo, Brazil; 7Department of Ophthalmology, Wake Forest University Baptist Medical Center, Winston-Salem, NC, USAAbstract: Aplastic anemia (AA is a rare disease with few reports on its ophthalmic ­manifestations. The ocular findings are described in a retrospective consecutive series of 719 AA Korean patients followed at the Hematology Clinic of The Catholic University of Korea. Out of a total of 719 patients, 269 patients had eye examinations, 156 patients had retinal evaluation, and 37 (23.7% had retinal findings. These 37 patients had unilateral retinal ­hemorrhage in seven and bilateral retinal hemorrhage in 30 with mean hemoglobin of 6.6 g/dL (range 2.7–12.6 g/dL and platelet counts of 18.8×109/L (range 4–157×109/L; central retinal vein occlusion-like picture occurred in nine patients and these had similar rheology to the rest of the subjects; optic disc edema, cotton-wool spots, macular edema, and dry eyes occurred in two, three, five, and three patients, respectively. In this Korean series of 141 subjects with AA, systemic bleeding occurred in 24.8% of subjects, retinal hemorrhage in 37% of subjects, and any bleeding site (eye or elsewhere occurred in 47.5% of subjects with AA. A

  18. Total lymphoid irradiation based conditioning for hematopoietic stem cell transplantation in severe aplastic anemia

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yun Hee; Kim, Ji Yoon; Choi, Byung Ock; Ryu, Mi Ryeong; Chung, Su Mi [Dept. of Radiation Oncology, The Catholic University of Korea College of Medicine, Seoul (Korea, Republic of)

    2012-12-15

    To retrospectively evaluate the outcome and toxicity of total lymphoid irradiation (TLI) based conditioning regimen for allogeneic hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) patients who experienced an engraftment failure from prior HSCT or were heavily transfused. Between 1995 and 2006, 20 SAA patients received TLI for conditioning of HSCT. All patients were multi-transfused or had long duration of disease. Fifteen (75%) patients had graft failure from prior HSCT. In 18 (90%) patients, the donors were human leukocyte antigen identical siblings. The stem cell source was the peripheral blood stem cell in 15 (75%) patients. The conditioning regimen was composed of antithymocyte globulin plus TLI with a median dose of 750 cGy in 1 fraction. The graft-versus-host disease (GVHD) prophylaxis used cyclosporine with methotrexate. With a median follow-up of 10.8 years, graft failures developed in 6 patients. Among them, 3 patients received their third HSCT to be engrafted finally. The Kaplan-Meier overall survival rate was 85.0% and 83.1% at 5 and 10 years, respectively. The incidence of acute and chronic GVHD was 20% and 20%, respectively. None of the patients have developed a malignancy after HSCT. In our study, TLI based conditioning in allogeneic HSCT was feasible with acceptable rates of GVHD in SAA patients who experienced graft failure from prior HSCT or was at a high risk of graft rejection. We achieved relatively better results of engraftment and survival with a long term follow-up.

  19. Association of severe thrombocytopenia and poor prognosis in pregnancies with aplastic anemia.

    Directory of Open Access Journals (Sweden)

    Jae Eun Shin

    Full Text Available PURPOSE: We sought to estimate the risks of adverse obstetric outcomes and disease outcomes associated with severe thrombocytopenia in pregnant women with aplastic anemia (AA. METHODS: In a retrospective study, we compared demographics, clinical characteristics, laboratory results, and outcomes between severe thrombocytopenia (ST and non-severe thrombocytopenia (non-ST groups comprising pregnant women with AA. RESULTS: Of 61 AA patients, 43 (70% were diagnosed as AA before pregnancy and 18 (30% were AA during pregnancy. The ST group exhibited lower gestational age at nadir of platelet count (26.0 versus 37.0 weeks, p<0.001 and at delivery (37.3 versus 39.1 weeks, p = 0.008, and a higher rate of bleeding gums (33.8 versus 7.7%, p = 0.015 than the non-ST group. In addition, the ST group exhibited more transfusions during pregnancy (72.7 versus 15.4%, p<0.001 and postpartum period (45.0 versus 2.7%, p<0.001, and more bone marrow transplant after delivery (25.0 versus 0.0%, p<0.001 than the non-ST group. The ST group had a higher odds ratio of composite disease complications (OR, 9.63; 95% CI, 2.82-32.9; p<0.001 and composite obstetric complications (OR, 6.78; 95% CI, 2.11-21.8; p = 0.001 than the non-ST group. CONCLUSIONS: Severe thrombocytopenia is more associated with obstetric and disease complications than is non-severe thrombocytopenia in pregnant women with AA.

  20. Immunosuppressive therapy in patients with aplastic anemia: a single-center retrospective study.

    Directory of Open Access Journals (Sweden)

    Hasan Jalaeikhoo

    Full Text Available Aplastic anemia (AA is a rare disease in which hematopoietic stem cells are severely diminished resulting in hypocellular bone marrow and pancytopenia. Etiology of AA includes auto immunity, toxins, infection, ionizing radiation, drugs and rare genetic disorders, but in the majority of cases no cause can be identified. In the present study we assessed response rate, survival, relapse and clonal evolution in patients with AA treated with immunosuppressive therapy.Patients with AA who received immunosuppressive therapy between May 1998 and September 2013 were included in this study. Patients with non-severe AA (NSAA were treated with cyclosporine (CsA and danazol while patients with severe AA (SAA as well as patients with NSAA who progressed to SAA after beginning of the treatment, were candidates for receiving antithymocyte globulin in addition to CsA and danazol.Among the 63 studied patients, 29 (46% had NSAA and 34 (54% had SAA. Three months after treatment, overall response was 58.6% in NSAA and 12.9% in patients with SAA. Survival of all patients at 5, 10 and 15 years were 73%, 55% and 49%, respectively. Survival rates were significantly higher in patients with NSAA compared to patients with SAA as well as in patients who responded at 6 months compared to non-responders. The relapse risk was 39.7% at 10 years. Relapse occurred in patients who discontinued the therapy more than those who continued taking CsA (p value<0.01. The risk of clonal evolution was 9.9% at 10 years and 22.8% at 15 years after treatment.This long-term retrospective study indicated that immunosuppressive therapy should be recommended to patients with AA. Also, our experience indicated that immunosuppressive therapy should not be discontinued after response to therapy in patients with both NSAA and SAA due to high risk of relapse. Low dose of CsA should be continued indefinitely.

  1. Complete sequence analysis of mitochondrial DNA and telomere length in aplastic anemia.

    Science.gov (United States)

    Cui, Xing; Wang, Junqiang; Cai, Zhiguo; Wang, Jingyi; Liu, Kui; Cui, Siyuan; Zhang, Jie; Luo, Yaqin; Wang, Xin; Li, Weiwei; Jing, Jingyan

    2014-11-01

    The present study was primarily undertaken to examine the hypothesis that mitochondrial DNA (mtDNA) mutations and telomere length may be associated with aplastic anemia (AA). Our study included a single institution analysis of 40 patients presenting with AA first diagnosed at the Affiliated Hospital of Shandong, University of Traditional Chinese Medicine between 2010 and 2013. Bone marrow and oral epithelial samples were collected from patients with AA (n=40) for mtDNA mutation and telomere length determinations. Bone marrow specimens were collected from 40 healthy volunteers as controls for the examination of telomere length. The mitochondrial genome was amplified by polymerase chain reaction (PCR), and the products were used for sequencing and analysis. We detected 146 heteroplasmic mutations in 18 genes from 40 patients with AA, including 39 silent mutations and 28 frameshift mutations. We used the gamma globin gene (HBG) as the control gene in real-time PCR to survey the relative telomere length measurements of the patients with AA and the healthy volunteers. Telomere length was expressed as the relative T/S value. We observed a negative correlation between the mtDNA non-silent mutation and the white blood cell (WBC) count, hemoglobin and platelet count. Of note, there was a positive correlation between the relative T/S value and WBC count, hemoglobin and platelet count, and a negative correlation between the non-silent mutation and the relative T/S value. We conclude that the functional impairment of the mitochondrial respiratory chain induced by mutation and telomere length shortening may play an important role in the process of hematopoietic failure in patients with AA. Additionally, mtDNA mutations and telomere length shortening influenced each other.

  2. First-line treatment for severe aplastic anemia in children: bone marrow transplantation from a matched family donor versus immunosuppressive therapy.

    Science.gov (United States)

    Yoshida, Nao; Kobayashi, Ryoji; Yabe, Hiromasa; Kosaka, Yoshiyuki; Yagasaki, Hiroshi; Watanabe, Ken-Ichiro; Kudo, Kazuko; Morimoto, Akira; Ohga, Shouichi; Muramatsu, Hideki; Takahashi, Yoshiyuki; Kato, Koji; Suzuki, Ritsuro; Ohara, Akira; Kojima, Seiji

    2014-12-01

    The current treatment approach for severe aplastic anemia in children is based on studies performed in the 1980s, and updated evidence is required. We retrospectively compared the outcomes of children with acquired severe aplastic anemia who received immunosuppressive therapy within prospective trials conducted by the Japanese Childhood Aplastic Anemia Study Group or who underwent bone marrow transplantation from an HLA-matched family donor registered in the Japanese Society for Hematopoietic Cell Transplantation Registry. Between 1992 and 2009, 599 children (younger than 17 years) with severe aplastic anemia received a bone marrow transplant from an HLA-matched family donor (n=213) or immunosuppressive therapy (n=386) as first-line treatment. While the overall survival did not differ between patients treated with immunosuppressive therapy or bone marrow transplantation [88% (95% confidence interval: 86-90) versus 92% (90-94)], failure-free survival was significantly inferior in patients receiving immunosuppressive therapy than in those undergoing bone marrow transplantation [56% (54-59) versus 87% (85-90); Paplastic anemia.

  3. Comparison of long-term outcomes between children with aplastic anemia and refractory cytopenia of childhood who received immunosuppressive therapy with antithymocyte globulin and cyclosporine.

    Science.gov (United States)

    Hama, Asahito; Takahashi, Yoshiyuki; Muramatsu, Hideki; Ito, Masafumi; Narita, Atsushi; Kosaka, Yoshiyuki; Tsuchida, Masahiro; Kobayashi, Ryoji; Ito, Etsuro; Yabe, Hiromasa; Ohga, Shouichi; Ohara, Akira; Kojima, Seiji

    2015-11-01

    The 2008 World Health Organization classification proposed a new entity in childhood myelodysplastic syndrome, refractory cytopenia of childhood. However, it is unclear whether this morphological classification reflects clinical outcomes. We retrospectively reviewed bone marrow morphology in 186 children (median age 8 years; range 1-16 years) who were enrolled in the prospective study and received horse antithymocyte globulin and cyclosporine between July 1999 and November 2008. The median follow-up period was 87 months (range 1-146 months). Out of 186 patients, 62 (33%) were classified with aplastic anemia, 94 (49%) with refractory cytopenia of childhood, and 34 (18%) with refractory cytopenia with multilineage dysplasia. Aplastic anemia patients received granulocyte colony-stimulating factor more frequently and for longer durations than other patients (Paplastic anemia, 4 patients with refractory cytopenia of childhood, and 3 patients with refractory cytopenia with multilineage dysplasia. Although the cumulative incidence of total clonal evolution at ten years was not significantly different among the 3 groups, the cumulative incidence of monosomy 7 development was significantly higher in aplastic anemia than in the other groups (P=0.02). Multivariate analysis revealed that only granulocyte colony-stimulating factor administration duration of 40 days or more was a significant risk factor for monosomy 7 development (P=0.02). These findings suggest that even the introduction of a strict morphological distinction from hypoplastic myelodysplastic syndrome cannot eradicate clonal evolution in children with aplastic anemia.

  4. Analysis on Traditional Chinese Medicine Syndrome of Aplastic Anemia%再生障碍性贫血中医证候分析

    Institute of Scientific and Technical Information of China (English)

    丁晓庆; 唐晓波; 孙海燕; 郭明; 郎海燕; 刘军霞; 李玲; 苏伟

    2013-01-01

    Objective: Analysis on traditional Chinese medicine syndrome of 220 cases of patients with aplastic anemia (110 severe aplastic anemia and 110 light aplastic anemia) was made,hoping to explore basic syndromes of the aplastic a-nemia through the comparative analysis,to observe the similarities syndrome characteristics or differences between severe and light aplastic anemia, and to provide theoretical basis of Chinese medicine treatment for aplastic anemia. Methods: The retrospective analysis on the general conditions( sex, age, course of disease, peripheral blood) , symptoms, syndromes and factors which may be associated with the syndromes in 220 cases of aplastic anemia patients was made. Conclusion: Deficiency of Qi, deficiency of blood, deficiency of kidney - yang and deficiency of kidney - yin are basic syndromes of aplastic anemia. While the deficiency of Qi and blood is more prominent than deficiency of yin and yang, especially in the severe aplastic anemia. Drugs for supplementing Qi and nourishing blood must be reused in treatment of traditional Chinese medicine. The frequency of intense noxious heat syndrome in severe aplastic anemia was higher than that in light aplastic anemia,and in daily life infection protection for SAA should be taken seriously attention.%目的:对220例再生障碍性贫血患者中医证候进行统计分析,其中重型再障和轻型再障各110例,希望通过分析对比,了解再生障碍性贫血的基本证候,并观察重型再障与轻型再障证候特点的异同,为中医治疗再生障碍性贫血提供理论依据.方法:采用病例回顾性分析方法,对220例再障患者的一般情况(性别、年龄、病程、外周血象)、症状、证候特点及可能与证候相关的因素进行统计分析.结论:①气虚、血虚、肾阳虚、肾阴虚是再生障碍性贫血的基本证候.而气血亏虚比阴阳不足更加突出,尤其是重型再障气血亏虚的临床表现更加严重,中医治疗时一定

  5. Therapeutic Effects of Hydrogen-Rich Solution on Aplastic Anemia in Vivo

    Directory of Open Access Journals (Sweden)

    Sanhu Zhao

    2013-08-01

    recovery of either hematological or immunological recovery on aplastic anemia mice. This finding suggests hydrogen-rich solution as a potential clinical therapeutic agent for AA.

  6. Severe Aplastic Anemia following Acute Hepatitis from Toxic Liver Injury: Literature Review and Case Report of a Successful Outcome

    Directory of Open Access Journals (Sweden)

    Kamran Qureshi

    2014-01-01

    Full Text Available Hepatitis associated aplastic anemia (HAAA is a rare syndrome in which severe aplastic anemia (SAA complicates the recovery of acute hepatitis (AH. HAAA is described to occur with AH caused by viral infections and also with idiopathic cases of AH and no clear etiology of liver injury. Clinically, AH can be mild to fulminant and transient to persistent and precedes the onset SAA. It is assumed that immunologic dysregulation following AH leads to the development of SAA. Several observations have been made to elucidate the immune mediated injury mechanisms, ensuing from liver injury and progressing to trigger bone marrow failure with the involvement of activated lymphocytes and severe T-cell imbalance. HAAA has a very poor outcome and often requires bone marrow transplant (BMT. The findings of immune related myeloid injury implied the use of immunosuppressive therapy (IST and led to improved survival from HAAA. We report a case of young male who presented with AH resulting from the intake of muscle building protein supplements and anabolic steroids. The liver injury slowly resolved with supportive care and after 4 months of attack of AH, he developed SAA. He was treated with IST with successful outcome without the need for a BMT.

  7. Trichosporon faecale invasive infection in a patient with severe aplastic anemia: Efficacy of voriconazole and liposomal amphotericin B before neutrophil recovery

    Directory of Open Access Journals (Sweden)

    Baptiste Pérard

    2015-09-01

    Full Text Available We report a case of a 51-year old man with a severe aplastic anemia who developed an invasive trichosporonosis to Trichosporon faecale with fungemia and skin lesions during severe neutropenia. The treatment was successful before neutrophil recovery with a combination of voriconazole and liposomal amphotericin B.

  8. F-18 FLT PET : A Noninvasive Diagnostic Tool for Visualization of the Bone Marrow Compartment in Patients With Aplastic Anemia A Pilot Study

    NARCIS (Netherlands)

    Agool, Ali; Slart, Riemer H. J. A.; Kluin, Philip M.; de Wolf, Joost Th. M.; Dierckx, Rudi A. J. O.; Vellenga, Edo

    2011-01-01

    Rationale: A discordant relationship between bone marrow cellularity and peripheral blood findings is regularly noticed in patients with aplastic anemia (AA). Therefore, the feasibility of 3-F-18 fluoro-3-deoxy-L-thymidine (F-18 FLT PET was tested as a noninvasive tool to visualize the total distrib

  9. Alternative Donor Transplantation with High-Dose Post-Transplantation Cyclophosphamide for Refractory Severe Aplastic Anemia

    Science.gov (United States)

    DeZern, Amy E.; Zahurak, Marianna; Symons, Heather; Cooke, Kenneth; Jones, Richard J.; Brodsky, Robert A.

    2017-01-01

    Severe aplastic anemia (SAA) is a life-threatening hematopoietic stem cell disorder that is treated with bone marrow transplantation (BMT) or immunosuppressive therapy (IST). The management of patients with refractory SAA after IST is a major challenge. Alternative donor BMT is the best chance for cure in refractory SAA, but morbidity and mortality from graft failure and complications of graft-versus-host disease (GVHD) have limited enthusiasm for this approach. Here, we employed post-transplantation high-dose cyclophosphamide in an effort to safely expand the donor pool in 16 consecutive patients with refractory SAA who did not have a matched sibling donor. Between July 2011 and August 2016, 16 patients underwent allogeneic (allo) BMT for refractory SAA from 13 haploidentical donors and 3 unrelated donors. The nonmyeloablative conditioning regimen consisted of antithymocyte globulin, fludarabine, low-dose cyclophosphamide, and total body irradiation. Post-transplantation cyclophosphamide 50 mg/kg/day i.v. on days +3 and +4 was administered for GVHD prophylaxis. Additionally, patients received mycophenolate mofetil on days +5 through 35 and tacrolimus from day +5 through 1 year. The median age of the patients at the time of transplantation was 30 (range, 11 to 69) years. The median time to neutrophil recovery over 1000 × 103/mm3 for 3 consecutive days was 19 (range, 16 to 27) days, to red cell engraftment was 25 (range, 2 to 58) days, and to last platelet transfusion to keep platelets counts over 50 × 103/mm3 was 27.5 (range, 22 to 108) days. Graft failure, primary or secondary, was not seen in any of the patients. All 16 patients are alive, transfusion independent, and without evidence of clonality. The median follow-up is 21 (range, 3 to 64) months. Two patients had grade 1 or 2 skin-only acute GVHD. These same 2 also had mild chronic GVHD of the skin/mouth requiring systemic steroids. One of these GVHD patients was able to come off all IST by 15 months and the

  10. 再生障碍性贫血的临床护理分析%Analysis Clinical Nursing Care of Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    王辉

    2015-01-01

    目的:探讨再生障碍性贫血的临床护理。方法选取临床2012年3月~2014年10月收治的再生障碍性贫血患者30例临床护理方法进行分析。结果30例再生障碍性贫血患者基本治愈10例,缓解9例,明显进步9例,无效2例,总有效率93.33%。结论了解再障的病因及治疗,让患者以积极的态度配合治疗。同时护士应熟练掌握再生障碍性贫血的基础护理,以满足患者的生存需要。%Objective To investigate clinical nursing care of aplastic anemia. Methods Selected 30 cases with aplastic anemia from March 2012 to October 2014 in clinical nursing methods were analyzed. Results 30 cases of aplastic anemia patients basically cured 10 cases, 9 cases of remission, 9 cases signiifcant progress , 2 cases invalid, the total efifciency is 93.33%. Conclusion Patients comprehend the etiology and treatment of aplastic anemia with a positive attitude to treatment. Nurses should primary nursing care on top of aplastic anemia, to satisfy patients survive.

  11. Aplasia medular após transplante hepático em pediatria Aplastic anemia after pediatric liver transplantation

    Directory of Open Access Journals (Sweden)

    Marlene P. Garanito

    2009-01-01

    Full Text Available A aplasia de medula é uma das mais raras (Aplastic anemia (AA is one of the rarest (<1% and most serious complications of liver transplantation for fulminant non-A, non-B and non-C hepatitis. It was first described in 1987 by Stock; the mechanism involved is an immunologically mediated condition secondary to an unknown viral infection. The disease is associated with a dismal prognosis. Spontaneous recovery from acquired AA is very rare however some patients (50-70% recover after immunosuppressive therapy, such as Cyclosporin A (CsA and Antithymocyte globulin (ATG, even after liver transplantation. Another treatment option is bone marrow transplantation. We report on a child who developed AA following liver transplantation for fulminant viral hepatitis that was treated with intensive immunosuppression including CsA and ATG and achieved complete recovery.

  12. 再生障碍性贫血免疫学发病机制的研究进展%Progress of immunological mechanism of aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    刘满菊

    2015-01-01

    Aplastic anemia is a more common pediatrics marrow failure disease.The main clinical manifestations are bone marrow dysfunction,pancytopenia,anemia,bleeding and infection.Althrough its pathogenesis has not been fully elucidated so far,more and more people think that it is associated with the disorder of immunological mechanisms, including the abnormalities of T cells, the level of negative regulator,humoral immune abnormalities and apoptosis.This review discusses the progress of immunological mechanism of aplastic anemia.%再生障碍性贫血(aplastic anemia,AA)是儿科较为常见的骨髓衰竭性疾病,临床上主要表现为骨髓造血功能低下、全血细胞减少以及贫血、出血、感染.其发病机制目前尚未完全阐明,越来越多的研究认为AA的发病与免疫学机制的紊乱有关,主要包括T细胞亚群的异常、负调控因子水平的改变、体液免疫异常及细胞凋亡等.该文就AA的免疫学发病机制的研究进展作一综述.

  13. Progress of pathogenesis of aplastic anemia%再生障碍性贫血发病机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    王方; 金润铭

    2010-01-01

    Aplastic anemia is an acquired bone marrow failure syndrome,characterized by an empty bone marrow,pancytopenia,as well as anemia,bleeding,infection syndrome.The pathogenesis of aplastic anemia has not yet been completely clear.Some of the proposed causes include hematopoietic stem/ progenitor cell deficiency,immune disorders,and abnormalities in the hematopoietic microenvironment.In recent years,further clinical and experimental studies have accumulated to recognize the pathogenesis of aplastic anemia.%再生障碍性贫血是一种获得性骨髓造血功能衰竭综合征.主要表现为骨髓造血功能低下、全血细胞减少以及贫血、出血、感染综合征.其发病机制尚未完全明确,主要涉及造血干/祖细胞缺陷、免疫功能紊乱、骨髓造血微环境异常三个方面.近年来各国学者对再生障碍性贫血进行了大量的临床以及实验研究,对其发病机制有了更进一步的认识,该文对此方面的研究进展做简要综述.

  14. Primitive Sca-1 Positive Bone Marrow HSC in Mouse Model of Aplastic Anemia: A Comparative Study through Flowcytometric Analysis and Scanning Electron Microscopy

    Directory of Open Access Journals (Sweden)

    Sumanta Chatterjee

    2010-01-01

    Full Text Available Self-renewing Hematopoietic Stem Cells (HSCs are responsible for reconstitution of all blood cell lineages. Sca-1 is the “stem cell antigen” marker used to identify the primitive murine HSC population, the expression of which decreases upon differentiation to other mature cell types. Sca-1+ HSCs maintain the bone marrow stem cell pool throughout the life. Aplastic anemia is a disease considered to involve primary stem cell deficiency and is characterized by severe pancytopenia and a decline in healthy blood cell generation system. Studies conducted in our laboratory revealed that the primitive Sca-1+ BM-HSCs (bone marrow hematopoietic stem cell are significantly affected in experimental Aplastic animals pretreated with chemotherapeutic drugs (Busulfan and Cyclophosphamide and there is increased Caspase-3 activity with consecutive high Annexin-V positivity leading to premature apoptosis in the bone marrow hematopoietic stem cell population in Aplastic condition. The Sca-1bright, that is, “more primitive” BM-HSC population was more affected than the “less primitive” BM-HSC Sca-1dim⁡ population. The decreased cell population and the receptor expression were directly associated with an empty and deranged marrow microenvironment, which is evident from scanning electron microscopy (SEM. The above experimental evidences hint toward the manipulation of receptor expression for the benefit of cytotherapy by primitive stem cell population in Aplastic anemia cases.

  15. Outcome of Allogeneic Stem Cell Transplantation for Patients Transformed to Myelodysplastic Syndrome or Leukemia from Severe Aplastic Anemia: A Report from the MDS Subcommittee of the Chronic Malignancies Working Party and the Severe Aplastic Anemia Working Party of the European Group for Blood and Marrow Transplantation

    NARCIS (Netherlands)

    Hussein, A.A.; Halkes, C.M.; Socie, G.; Tichelli, A.; Borne, P.A. von dem; Schaap, M.N.; Foa, R.; Ganser, A.; Dufour, C.; Bacigalupo, A.; Locasciulli, A.; Aljurf, M.; Peters, C.; Robin, M.; Biezen, A.A. van; Volin, L.; Witte, T.J. de; Marsh, J.; Passweg, J.R.; Kroger, N.

    2014-01-01

    One hundred and forty patients who had undergone hematopoietic stem cell transplantation (HSCT) for myelodysplastic syndrome (MDS) or acute myelogenous leukemia (AML) transformation after treatment of severe aplastic anemia (SAA) were identified in the European Group for Blood and Marrow Transplanta

  16. Anemia aplásica adquirida e anemia de Fanconi - Diretrizes Brasileiras em Transplante de Células-Tronco Hematopoéticas Acquired aplastic anemia and Fanconi anemia - Brazilian Guidelines in Hematopoietic Stem Cell Transplantation

    Directory of Open Access Journals (Sweden)

    Larissa A. Medeiros

    2010-05-01

    Full Text Available As diretrizes apresentadas neste trabalho foram elaboradas e aprovadas na I Reunião de Diretrizes Brasileiras em Transplante de Células-Tronco Hematopoéticas (TCTH realizada no Rio de Janeiro, entre os dias 19 e 21 de julho de 2009. O evento foi promovido pela SBTMO (Sociedade Brasileira de Transplante de Medula Óssea. Neste artigo, tratamos da anemia aplásica severa (AAS, considerada uma urgência hematológica, que, identificada e manejada de forma precoce, apresenta grande possibilidade de recuperação da hematopoese seja através de transplante de medula óssea ou terapia imunossupressora. Objetiva-se nortear o manejo terapêutico no contexto do transplante e indicar formas de condicionamento, de acordo com as características clínicas dos pacientes, como o número de transfusões, com intuito de minimizar a rejeição primária e secundária, garantindo a melhora da sobrevida global e livre de doença (observadas pela literatura e já validadas por resultados na população brasileira. No que concerne à anemia de Fanconi, o transplante é a única modalidade curativa para o componente aplásico de medula óssea; embora não modificando as outras características da síndrome também demanda perícia e agilidade na busca de um doador com resultados expressivos de sobrevida.The guidelines presented in this article have been prepared and approved in the I Meeting of Brazilian Guidelines in Hematopoietic Stem Cell Transplantation (HSCT - Rio de Janeiro, July 19-21, 2009. The event was sponsored by SBTMO (Brazilian Society of Bone Marrow Transplantation. In this paper, we treat the severe aplastic anemia (SAA, considered a hematological emergency, that when identified and medically treated early, shows a great chance of recovery of the hematopoiesis, either through bone marrow transplantation or immunosuppressive therapy. Its objective is to guide the management of the transplantation, and indicate methods of conditioning, according to

  17. Current outcome of HLA identical sibling versus unrelated donor transplants in severe aplastic anemia: an EBMT analysis.

    Science.gov (United States)

    Bacigalupo, Andrea; Socié, Gerard; Hamladji, Rose Marie; Aljurf, Mahmoud; Maschan, Alexei; Kyrcz-Krzemien, Slawomira; Cybicka, Alicja; Sengelov, Henrik; Unal, Ali; Beelen, Dietrich; Locasciulli, Anna; Dufour, Carlo; Passweg, Jakob R; Oneto, Rosi; Signori, Alessio; Marsh, Judith C W

    2015-05-01

    We have analyzed 1448 patients with acquired aplastic anemia grafted between 2005 and 2009, and compared outcome of identical sibling (n=940) versus unrelated donor (n=508) transplants. When compared to the latter, sibling transplants were less likely to be performed beyond 180 days from diagnosis (39% vs. 85%), to have a cytomegalovirus negative donor/recipient status (15% vs. 23%), to receive antithymocyte globulin in the conditioning (52% vs. 61%), and more frequently received marrow as a stem cell source (60% vs. 52%). Unrelated donor grafts had significantly more acute grade II-IV (25% vs. 13%) and significantly more chronic graft-versus-host disease (26% vs. 14%). In multivariate analysis, the risk of death of unrelated donor grafts was higher, but not significantly higher, compared to a sibling donor (P=0.16). The strongest negative predictor of survival was the use of peripheral blood as a stem cell source (Paplastic anemia, is currently not statistically inferior when compared to sibling transplants, although patients are at greater risk of acute and chronic graft-versus-host disease. The use of peripheral blood grafts remains the strongest negative predictor of survival.

  18. Comparison of immune manifestations between refractory cytopenia of childhood and aplastic anemia in children: A single-center retrospective study.

    Science.gov (United States)

    Wu, Jun; Cheng, Yifei; Zhang, Leping

    2015-12-01

    This retrospective single-center study assessed the incidence and clinical features of immune manifestations of refractory cytopenia of childhood (RCC) and childhood aplastic anemia (AA). We evaluated 72 children with RCC and 123 with AA between February 2008 and March 2013. RCC was associated with autoimmune disease in 4 children, including 1 case each with autoimmune hemolytic anemia, rheumatoid arthritis, systemic lupus erythematosus, and anaphylactoid purpura. No children with AA were diagnosed with autoimmune diseases. Immune abnormalities were common in both RCC and AA; the most significant reductions were in the relative numbers of CD3-CD56+ subsets found in RCC. Despite the many similar immunologic abnormalities in AA and RCC, the rate of autoimmune disease was significantly lower in childhood AA than RCC (p=0.008, χ2=6.976). The relative numbers of natural killer cells were significantly lower in RCC patients than AA patients. By month 6, there was no significant difference in autoimmune manifestations between RCC and AA in relation to the response to immunosuppressive therapy (p=0.907, χ2=0.014). The large overlap of analogous immunologic abnormalities indicates that RCC and childhood AA may share the same pathogenesis.

  19. Combined transplantation of G-CSF primed allogeneic bone marrow cells and peripheral blood stem cells in treatment of severe aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    黄晓军; 陈育红; 许兰平; 张耀臣; 刘代红; 郭乃榄; 陆道培

    2004-01-01

    @@ The major causes of unsuccessful transplantations for severe aplastic anemia (SAA) are graft-versus-host disease (GVHD), infection, and graft failure.1,2 The latter is particularly associated with SAA in that various methods have been developed to overcome it.Intensification of immunosupression during conditioning and high-dosage stem cell infusion can overcome sensitization to transplant antigens and improve engraftment after transplantation.

  20. Relationship between aplastic anemia and infection of virus in children%儿童再生障碍性贫血与病毒感染的关系

    Institute of Scientific and Technical Information of China (English)

    刘月东; 张洪涛

    2003-01-01

    @@ 为分析病毒感染与再生障碍性贫血(aplastic anemia,简称AA,再障)发病的关系,收集了1997年10月至2002年8月55例再障病例,均进行了病毒学检测,现将其结果报告如下.

  1. 再生障碍性贫血的诊断治疗进展%Progress of diagnosis and treatment for aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    杨颖哲; 汤静燕

    2010-01-01

    As the techruque of molecular biology developed, the research of aplastic anemia turns to the cytogenetical level. Scientists focused on irwestigating the relationship between the mutation of gene or chromosome and the curative effect of aplastic anemia.The results show that abnormality of particular chrromosome or gene will cause different outcome . On the other hand, the first line therapy of aplastic anemia is still IST or HSCT according to the different situation.The antibody special for T cell is still in studying, and the effect shows well .Through the typing diagnosis consummated, the treating method developed, the survival rate of the patient with apLastic anemia will be advanced.%随着分子生物学技术的发展,有关再生障碍性贫血(再障)的研究热点转向细胞遗传学,着力于探讨染色体及基因异常对再障的疗效影响.研究发现特定染色体及基因异常会对再障预后的诊断产生影响.另一方面,再障的首选治疗仍是根据不同情况选择免疫抑制治疗或骨髓移植,针对T细胞的特异性抗体正在研究中且疗效肯定.通过完善诊断分型和发展治疗手段,再障患者的生存率将继续提高.

  2. 柯微君治疗慢性再生障碍性贫血经验%Ke Weijun's Experience in Treatment of Chronic Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    刘晓翔; 高飞; 柯微君

    2015-01-01

    Chronic aplastic anemia is the difficultly - treated disease in blood system. Prof. Ke Wei-jing has accumulated rich experience in the treatment of chronic aplastic anemia. In the paper,the etiology and pathogenesis,syndrome differentiation and medication characteristics were analyzed. Prof. Ke thought that spleen and kidney deficiency and disharmony of yin and yang were the basic pathogenesis of chronic aplastic anemia,heat - toxin - stasis was the causative factor and also the pathological production of chronic aplastic anemia. In the treatment,the basic principle was strengthening the spleen,tonifying the kidney and regulating yin and yang,associated with clearing heat,detoxification,cooling blood and activating blood circulation. The therapeutic effect was very excellent.%慢性再生障碍性贫血是临床上较为难治的血液系统疾病。柯微君老师对慢性再生障碍性贫血的治疗经验丰富。文章介绍了柯老师对慢性再生障碍性贫血的病因病机、辨证分型以及用药特点分析。柯老师认为脾肾两虚、阴阳失调是慢性再生障碍性贫血的基本病机,热毒瘀是慢性再生障碍性贫血的致病因素,也是慢性再生障碍性贫血的病理产物。治疗上以健脾补肾、调补阴阳为本,配合清热解毒、凉血活血之剂,疗效甚佳。

  3. Deregulation of vital mitotic kinase-phosphatase signaling in hematopoietic stem/progenitor compartment leads to cellular catastrophe in experimental aplastic anemia.

    Science.gov (United States)

    Chatterjee, Ritam; Chattopadhyay, Sukalpa; Law, Sujata

    2016-11-01

    Aplastic anemia, the paradigm of bone marrow failure, is characterized by pancytopenic peripheral blood and hypoplastic bone marrow. Among various etiologies, inappropriate use of DNA alkylating drugs like cyclophosphamide and busulfan often causes the manifestation of the dreadful disease. Cell cycle impairment in marrow hematopoietic stem/progenitor compartment together with cellular apoptosis has been recognized as culpable factors behind aplastic pathophysiologies. However, the intricate molecular mechanisms remain unrevealed till date. In the present study, we have dealt with the mechanistic intervention of the disease by peripheral blood hemogram, bone marrow histopathology, cytopathology, hematopoietic kinetic study, scanning electron microscopy, DNA damage assessment and flowcytometric analysis of cellular proliferation and apoptosis in hematopoietic stem/progenitor cell (HSPC) rich marrow compartment using busulfan and cyclophosphamidemediated mouse model. To unveil the molecular mechanisms behind aplastic pathophysiology, we further investigated the role of some crucial mitotic and apoptotic regulators like Protein kinase-B (PKB), Gsk-3β, Cyclin-D1, PP2A, Cdc25c, Plk-1, Aurora kinase-A, Chk-1 regarding the hematopoietic catastrophe. Our observations revealed that the alteration of PKB-GSK-3β axis, Plk-1, and Aurora kinase-A expressions in HSPC compartment due to DNA damage response was associated with the proliferative impairment and apoptosis during aplastic anemia. The study established the correlation between the accumulation of DNA damage and alteration of the mentioned molecules in aplastic HSPCs that lead to the hematopoietic catastrophe. We anticipate that our findings will be beneficial for developing better therapeutic strategies for the dreadful disease concerned.

  4. Differential gene expression profile associated with the abnormality of bone marrow mesenchymal stem cells in aplastic anemia.

    Directory of Open Access Journals (Sweden)

    Jianping Li

    Full Text Available Aplastic anemia (AA is generally considered as an immune-mediated bone marrow failure syndrome with defective hematopoietic stem cells (HSCs and marrow microenvironment. Previous studies have demonstrated the defective HSCs and aberrant T cellular-immunity in AA using a microarray approach. However, little is known about the overall specialty of bone marrow mesenchymal stem cells (BM-MSCs. In the present study, we comprehensively compared the biological features and gene expression profile of BM-MSCs between AA patients and healthy volunteers. In comparison with healthy controls, BM-MSCs from AA patients showed aberrant morphology, decreased proliferation and clonogenic potential and increased apoptosis. BM-MSCs from AA patients were susceptible to be induced to differentiate into adipocytes but more difficult to differentiate into osteoblasts. Consistent with abnormal biological features, a large number of genes implicated in cell cycle, cell division, proliferation, chemotaxis and hematopoietic cell lineage showed markedly decreased expression in BM-MSCs from AA patients. Conversely, more related genes with apoptosis, adipogenesis and immune response showed increased expression in BM-MSCs from AA patients. The gene expression profile of BM-MSCs further confirmed the abnormal biological properties and provided significant evidence for the possible mechanism of the destruction of the bone marrow microenvironment in AA.

  5. Efficacy of rabbit antithymocyte globulin as first-line treatment of severe aplastic anemia: an Asian multicenter retrospective study.

    Science.gov (United States)

    Chuncharunee, Suporn; Wong, Raymond; Rojnuckarin, Ponlapat; Chang, Cheng-Shyong; Chang, Kian Meng; Lu, Meng-Yao; Hwang, Wen-Li; Koh, Liang Piu; Chen, Tsai-Yun; Leung, Anskar Yh; Norasetthada, Lalita; Wang, Shih-Chung; Chang, Ming-Chih; Wu, Kang-Hsi; Issaragrisil, Surapol

    2016-10-01

    Due to the unavailability of horse antithymocyte globulin (ATG) in many markets worldwide, patients with severe aplastic anemia (SAA) are limited to the use of rabbit ATG. We aimed to analyze hematologic response and overall survival (OS) of Asian patients treated with rabbit ATG as first-line therapy of SAA. We retrospectively reviewed the medical records of 97 consecutive patients who received rabbit ATG as first-line treatment of SAA from 2006 to 2012 at centers in four Asian countries. The primary endpoint was 6- and 12-month overall response rates (ORR) for patients receiving rabbit ATG within the recommended dose range (2.5-3.75 mg/kg/day). Secondary endpoints included ORR in patients receiving any dose of rabbit ATG and 2-year OS. For patients who received rabbit ATG within the recommended dose range, 6- and 12-month ORRs were 17.4 and 63.6 %, respectively. For patients who received any dose of rabbit ATG, 6- and 12-month ORRs were 24.3 and 68.6 %, respectively. The 2-year OS rate was 86.3 %. Rabbit ATG is effective for treatment of SAA in Asian patients. The 12-month ORR and 2-year OS with rabbit ATG were comparable to historical results obtained with horse ATG.

  6. Memory Stem T Cells in Autoimmune Disease: High Frequency of Circulating CD8+ Memory Stem Cells in Acquired Aplastic Anemia.

    Science.gov (United States)

    Hosokawa, Kohei; Muranski, Pawel; Feng, Xingmin; Townsley, Danielle M; Liu, Baoying; Knickelbein, Jared; Keyvanfar, Keyvan; Dumitriu, Bogdan; Ito, Sawa; Kajigaya, Sachiko; Taylor, James G; Kaplan, Mariana J; Nussenblatt, Robert B; Barrett, A John; O'Shea, John; Young, Neal S

    2016-02-15

    Memory stem T cells (TSCMs) constitute a long-lived, self-renewing lymphocyte population essential for the maintenance of functional immunity. Hallmarks of autoimmune disease pathogenesis are abnormal CD4(+) and CD8(+) T cell activation. We investigated the TSCM subset in 55, 34, 43, and 5 patients with acquired aplastic anemia (AA), autoimmune uveitis, systemic lupus erythematosus, and sickle cell disease, respectively, as well as in 41 age-matched healthy controls. CD8(+) TSCM frequency was significantly increased in AA compared with healthy controls. An increased CD8(+) TSCM frequency at diagnosis was associated with responsiveness to immunosuppressive therapy, and an elevated CD8(+) TSCM population after immunosuppressive therapy correlated with treatment failure or relapse in AA patients. IFN-γ and IL-2 production was significantly increased in various CD8(+) and CD4(+) T cell subsets in AA patients, including CD8(+) and CD4(+) TSCMs. CD8(+) TSCM frequency was also increased in patients with autoimmune uveitis or sickle cell disease. A positive correlation between CD4(+) and CD8(+) TSCM frequencies was found in AA, autoimmune uveitis, and systemic lupus erythematosus. Evaluation of PD-1, CD160, and CD244 expression revealed that TSCMs were less exhausted compared with other types of memory T cells. Our results suggest that the CD8(+) TSCM subset is a novel biomarker and a potential therapeutic target for AA.

  7. Therapeutic Effect of Shenfu Injection(参附注射液)on Secondary Aplastic Anemia of Tumor Patients after Chemotherapy

    Institute of Scientific and Technical Information of China (English)

    潘敬新; 郭奕斌; 郭熙哲; 郭健欣; 陈云峰; 王志东

    2004-01-01

    Objective: To explore the effect of Shenfu Injection (参附注射液, SFI) on the secondary aplastic anemia (AA) of tumor patients after chemotherapy (CT). Methods: The 15 cases of SFI treated group, 10 cases of control group, 25 cases of SFI + granulocyte-macrophage colony stimulating factor (GMCSF) treated group and the group of 23 GM-CSF-treated tumor cases of secondary AA after CT were compared, with their increasing rate and the rebound speed of neutrophil, platelet, bone marrow nucleated RBC,granulocyte, and megakaryocyte all being investigated. Results: The increasing rate and rebound speed of granulocyte, platelet, and the increasing rate of bone marrow nucleated RBC, granulocyte, megakaryocyte were obviously higher than those of the control, and the clinical manifestations were also obviously improved.The increasing rate of platelet, bone marrow nucleated RBC, megakaryocyte of the SFI + GM-CSF group were higher than those of the group which used GM-CSF alone, while the increasing rate of granulocyte in blood and bone marrow in both groups was similar. Conclusion: Significant efficacy was shown in SFI for the treatment of secondary AA of tumor patients after CT.

  8. Aplastic anemia and severe pancytopenia during treatment with peg-interferon, ribavirin and telaprevir for chronic hepatitis C.

    Science.gov (United States)

    Lens, Sabela; Calleja, Jose L; Campillo, Ana; Carrión, Jose A; Broquetas, Teresa; Perello, Christie; de la Revilla, Juan; Mariño, Zoe; Londoño, María-Carlota; Sánchez-Tapias, Jose M; Urbano-Ispizua, Álvaro; Forns, Xavier

    2015-05-07

    Telaprevir and Boceprevir are the first direct acting antivirals approved for chronic hepatitis C in combination with peg-interferon alfa and ribavirin. Pancytopenia due to myelotoxicity caused by these drugs may occur, but severe hematological abnormalities or aplastic anemia (AA) have not been described. We collected all cases of severe pancytopenia observed during triple therapy with telaprevir in four Spanish centers since approval of the drug in 2011. Among 142 cirrhotic patients receiving treatment, 7 cases of severe pancytopenia (5%) were identified and three were consistent with the diagnosis of AA. Mean age was 59 years, five patients had compensated cirrhosis and two patients had severe hepatitis C recurrence after liver transplantation. Severe pancytopenia was diagnosed a median of 10 wk after the initiation of therapy. Three patients had pre-treatment hematological abnormalities related to splenomegaly. In six patients, antiviral treatment was interrupted at the onset of hematological abnormalities. Two patients died due to septic complications and one patient due to acute alveolar hemorrhage. The remaining patients recovered. Severe pancytopenia and especially AA, are not rare during triple therapy with telaprevir in patients with advanced liver disease. Close monitoring is imperative in this setting to promptly detect serious hematological disorders and to prevent further complications.

  9. Clinical Observation on Treatment of Chronic Aplastic Anemia by Shengxuening (生血宁) and Cyclosporin A

    Institute of Scientific and Technical Information of China (English)

    ZHANG Xue-zhong; XU Yan-li; JIN Juan; ZHANG Xiu-qun; ZHANG Lei; SU Ai-ling

    2006-01-01

    Objective: To explore the therapy to further elevate the efficacy of the treatment of chronic aplastic anemia (CAA). Methods: Forty-five patients with CCA were assigned into two groups, the 26 paporin A (CsA), and the 19 patients in the control group were treated with androgen alone, with the therapeutic course lasting for over 3 months. Changes of peripheral blood picture, and the colony productivity of burst forming unit-erythroid (BFU-E), colony forming unit-erythroid (CFU-E) and colony forming unit-granulocyte macrophage (CFU-GM) in bone marrow were observed before and after 3 months treatment. The amount of erythrocyte and platelet infusion, frequency of infection, condition of hemorrhage and relevant death were also observed. The follow-up study was conducted for over half a year. Results: The total effective rate in the treated group was 84.6%, which was significantly higher than that in the control group (52.6%, P<0.05).Levels of hemoglobin, reticulocyte, neutrophil and platelet increased after treatment in the treated group, as compared with those before treatment, with significant difference ( P<0.05), and the colony productivity of BFU-E, CFU-E and CFU-GM in bone marrow also got significantly increased ( P<0.01 ), and showed significant difference from those in the control group (P<0.05). Conclusion: Shengxuening-assisting CsA therapy is an effective measure for treatment of CAA.

  10. Expression of CD28 and CTLA4 on T Cells in Bone Morrow of Immune-mediated Aplastic Anemia Mice

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    To investigate the expression and significance of CD28 and CTLA4 on T cells in bone marrow of aplastic anemia (AA) mice, in vitro bone marrow mononuclear cells (BMMNCs) were activated through being incubated with PHA (15μg/mL). The expression of CD28 and CTLA4 on T cells incubated with or without PHA was detected by two-color flow cytometry. The expression of CD28 and CTLA4 was significantly increased after PHA stimulation. In the AA mice, the expression of CD28 with or without PHA stimulation was both higher than that in the normal mice (both P<0.01), but the expression of CTLA4 with or without PHA stimulation showed no significant difference in comparison to that in the normal mice (both P>0.05). In the AA mice, there were more activation and activated potential of T cells than the normal, and the abnormal expression of CD28 and CTLA4 may participate in immunological disorder mediated by T cells.

  11. Quantitative Detection of ID4 Gene Aberrant Methylation in the Differentiation of Myelodysplastic Syndrome from Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    Mian-Yang Li; Yuan-Yuan Xu; Hui-Yuan Kang; Xin-Rong Wang; Li Gao; Jian Cen; Wei Wang

    2015-01-01

    Background:The diagnosis of myelodysplastic syndrome (MDS),especially hypoplastic MDS,and MDS with low blast counts or normal karyotype may be problematic.This study characterized ID4 gene methylation in patients with MDS and aplastic anemia (AA).Methods:The methylation status ofID4 was analyzed by bisulfite sequencing polymerase chain reaction (PCR) and quantitative real-time methylation-specific PCR (MethyLight PCR) in 100 patients with MDS and 31 patients with AA.Results:The MDS group had a higher ID4 gene methylation positivity rate (22.22%) and higher methylation levels (0.21 [0-3.79]) than the AA group (P < 0.05).Furthermore,there were significant differences between the hypoplastic MDS and AA groups,the MDS with low blast count and the AA groups,and the MDS with normal karyotype and the AA groups.The combination of genetic and epigenetic markers was used in much more patients with MDS (62.5% [35/56]) than the use of genetic markers only (51.79% [29/56]).Conclusions:These results showed that the detection ofID4 methylation positivity rates and levels could be a useful biomarker for MDS diagnosis.

  12. Thought o Treatment Based on Syndrome Differentiation of TCM on Chronic Aplastic Anemia%慢性再生障碍性贫血中医辨治思路

    Institute of Scientific and Technical Information of China (English)

    王兴丽; 杨文华

    2015-01-01

    再生障碍性贫血简称“再障”,是由多种原因引起骨髓造血功能衰竭,临床以贫血、出血、感染为主要表现的疾病。根据临床表现分为急性再障与慢性再障,中医药在治疗慢性再障方面有较好的疗效。杨文华教授现为天津中医药大学硕士研究生导师、博士研究生导师,从事中医药治疗血液病临床及科研工作40余年,在治疗慢性再生障碍性贫血方面具有独特建树,现将杨文华教授辨治慢性再生障碍性贫血思路及经验作一总结。%The aplastic anemia is caused by a variety of reasons for bone marrow hematopoietic failure .Main features are anemia , hemorrhage and infection .According to clinical feature , it is divided into acute aplastic anemia and chronic aplastic anemia .The Chinese herbal medicine had better effect in the treatment of chronic aplastic anemia .Professor Yang Wenhua is a supervisor of postgraduate student and PHD student in Tianjin University of Traditional Chinese Medicine , and was engaged in clinical Hematology and research over 40 years. Professor Yang Wenhua had distinctive method in the treatment of chronic aplastic anemia .We presented a summary of Professor Yang Wenhua's thought and experience of treatment based on syndrome differentiation of TCM on chronic aplastic anemia .

  13. 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 ...

  14. Analysis the risk factors of pregnancy complicated with chronic aplastic anemia and pre-eclampsia%妊娠合并慢性再生障碍性贫血并发子痫前期的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    谭大勇

    2013-01-01

      Objective To investigate the risk factor of pregnancy with chronic aplastic anemia and pre-eclampsia factors, for provide the help for prevention of pregnancy complicated with chronic aplastic anemia and pre-eclampsia. Methods 27 cases of pregnancy complicated with chronic aplastic anemia clinical data in our hospital from 2007 May and analysis to 2012 December, and the statistical analysis, the age, first diagnosed during pregnancy, bleeding tendency, aplastic anemia severity, white blood cell, hemoglobin, platelet 0.05). Conclusion Pregnancy complicated with chronic aplastic anemia and pre-eclampsia may affected by many factors, but the 0.05)。结论妊娠合并慢性再生障碍性贫血并发子痫前期受到多种因素影响,血红蛋白<60g/L、血小板<10×109/L是主要的相关危险因素。

  15. 基于络病理论的再生障碍性贫血“络瘀致障”病机探讨%Pathogenesis Research of Collateral Stasis to Aplastic Anemia Based on the Theory of Collateral Disease

    Institute of Scientific and Technical Information of China (English)

    王海霞; 李琳; 田丛丛

    2013-01-01

      本文以络病理论为基础,对再生障碍性贫血的病机进行探讨,从“络瘀致障”来探讨其发病机制,力求揭示再障的病机规律,为再障的治疗寻求新的思路与方法。%This article is based on the theory of collateral disease, to explore the pathogenesis of aplastic anemia from collateral, and strive to reveal the the pathogenesis of aplastic anemia, and to seek the new ideas and methods for aplastic anemia treatment.

  16. Impact of cyclophosphamide dose of conditioning on the outcome of allogeneic hematopoietic stem cell transplantation for aplastic anemia from human leukocyte antigen-identical sibling.

    Science.gov (United States)

    Mori, Takehiko; Koh, Hideo; Onishi, Yasushi; Kako, Shinichi; Onizuka, Makoto; Kanamori, Heiwa; Ozawa, Yukiyasu; Kato, Chiaki; Iida, Hiroatsu; Suzuki, Ritsuro; Ichinohe, Tatsuo; Kanda, Yoshinobu; Maeda, Tetsuo; Nakao, Shinji; Yamazaki, Hirohito

    2016-04-01

    The standard conditioning regimen in allogeneic hematopoietic stem cell transplantation (HSCT) for aplastic anemia from a human leukocyte antigen (HLA)-identical sibling has been high-dose cyclophosphamide (CY 200 mg/kg). In the present study, results for 203 patients with aplastic anemia aged 16 years or older who underwent allogeneic HSCT from HLA-identical siblings were retrospectively analyzed using the registry database of Japan Society for Hematopoietic Cell Transplantation. Conditioning regimens were defined as a (1) high-dose CY (200 mg/kg or greater)-based (n = 117); (2) reduced-dose CY (100 mg/kg or greater, but less than 200 mg/kg)-based (n = 38); and (3) low-dose CY (less than 100 mg/kg)-based (n = 48) regimen. Patient age and the proportion of patients receiving fludarabine were significantly higher in the reduced- and low-dose CY groups than the high-dose CY group. Engraftment was comparable among the groups. Five-year overall survival (OS) tended to be higher in the low-dose CY group [93.0 % (95 % CI 85.1-100.0 %)] than the high-dose CY [84.2 % (95 % CI 77.1-91.3 %)] or reduced-dose CY groups [83.8 % (95 % CI 71.8-95.8 %); P = 0.214]. Age-adjusted OS was higher in the low-dose CY group than the high- and reduced-dose CY groups with borderline significance (P = 0.067). These results suggest that CY dose can safely be reduced without increasing graft rejection by adding fludarabine in allogeneic HSCT for aplastic anemia from an HLA-identical sibling.

  17. Treatment of Severe Aplastic Anemia by Immunosuppressor Anti-lymphocyte Globulin/Anti-thymus Globulin as the Chief Medicine in Combination with Chinese Drugs

    Institute of Scientific and Technical Information of China (English)

    郑兵荣; 沈建平; 庄海峰; 林圣云; 沈一平; 周郁鸿

    2009-01-01

    Objective:To study the therapeutic effect of combined therapy with Chinese drugs and immunosuppressors, mainly anti-lymphocyte globulin/anti-thymus globulin(ALG/ATG),for the treatment of severe aplastic anemia(SAA),the efficacy associated factors and adverse effects as well.Methods:A retrospective analysis was conducted on 65 patients with SAA treated by combined therapy which was supplemented with cyclosporin A,androgen,hematopoietic growth factor,etc.Results:Of the 57 patients followed-up,26 (45.6%) we...

  18. 再生障碍性贫血相关的恶性疾病及克隆改变%Malignant or clonal diseases evoluted from aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    王婷; 邵宗鸿

    2011-01-01

    再生障碍性贫血(aplastic anemia,AA)是多种病因引起的造血障碍综合征,近年来生存率得到明显改善,生存时间明显延长.人们长期随访发现部分AA有向恶性疾病及克隆性疾病进展或"转变"的风险,发病机制可能包括造血干细胞自身缺陷、造血微环境影响、治疗干预等.

  19. Monosomy 7 in donor cell-derived leukemia after bone marrow transplantation for severe aplastic anemia: report of a new case and review of the literature

    Directory of Open Access Journals (Sweden)

    Luize Otero

    2012-01-01

    Full Text Available Monosomy 7 arises as a recurrent chromosome aberration in donor cell leukemia after hematopoietic stem cell transplantation. We report a new case of donor cell leukemia with monosomy 7 following HLA-identical allogenic bone marrow transplantation for severe aplastic anemia (SAA. The male patient received a bone marrow graft from his sister, and monosomy 7 was detected only in the XX donor cells, 34 months after transplantation. The patient's bone marrow microenvironment may have played a role in the leukemic transformation of the donor hematopoietic cells.

  20. T-cell receptor Vbeta CDR3 oligoclonality frequently occurs in childhood refractory cytopenia (MDS-RC) and severe aplastic anemia

    DEFF Research Database (Denmark)

    Vries, A.C. de; Langerak, A.W.; Verhaaf, B.

    2008-01-01

    (Very) severe acquired aplastic anemia ((v)SAA) and myelodysplastic syndrome (MDS) are rare diseases in childhood. (V)SAA is a bone marrow (BM) failure syndrome characterized by immune-mediated destruction of hematopoietic progenitors. MDS is a malignant clonal stem cell disorder, of which...... and the discriminative value of TCR Vbeta CDR3 oligoclonality in pediatric (v)SAA and MDS patients. Peripheral blood (PB) and/or BM mononuclear cell samples of pediatric patients with (v)SAA (n=38), refractory cytopenia (MDS-RC) (n=28) and 18 controls were analysed via TCR Vbeta heteroduplex PCR analysis of extracted...

  1. A retrospective comparison of cyclophosphamide plus antithymocyte globulin with cyclophosphamide plus busulfan as the conditioning regimen for severe aplastic anemia

    Directory of Open Access Journals (Sweden)

    L.V.M. Ommati

    2009-03-01

    Full Text Available Allogeneic hematopoietic stem cell transplantation (AHSCT is the treatment of choice for young patients with severe aplastic anemia (SAA. The association of antithymocyte globulin (ATG and cyclophosphamide (CY is the most frequently used conditioning regimen for this disease. We performed this retrospective study in order to compare the outcomes of HLA-matched sibling donor AHSCT in 41 patients with SAA receiving cyclophosphamide plus ATG (ATG-CY, N = 17 or cyclophosphamide plus busulfan (BU-CY, N = 24. The substitution of BU for ATG was motivated by the high cost of ATG. There were no differences in the clinical features between the two groups, including age, gender, cytomegalovirus status, ABO match, interval between diagnosis and transplant, and number of total nucleated cells infused. No differences were observed in the time to neutrophil and platelet engraftment, or in the risk of veno-occlusive disease and hemorrhage. However, there was a higher risk of mucositis in the BU-CY group (71 vs 24%, P = 0.004. There were no differences in the incidence of neutrophil and platelet engraftment, acute and chronic graft-versus-host disease, and transplant-related mortality. There was a higher incidence of late rejection in the ATG-CY group (41 vs 4%, P = 0.009. Although the ATG-CY group had a longer follow-up (101 months than the BU-CY group (67 months, P = 0.04, overall survival was similar between the groups (69 vs 58%, respectively, P = 0.32. We conclude that the association BU-CY is a feasible option to the conventional ATG-CY regimen in this population.

  2. Changes and significances of Th1/Th2, related cytokines and T cell subgroup in aplastic anemia patients

    Institute of Scientific and Technical Information of China (English)

    Hong-Xia Zhang; Guang-Sheng Wu; Wei-Ling Guo

    2016-01-01

    Objective:To investigate the changes and significances of Th1/Th2, related cytokines and T cell subgroup in aplastic anemia(AA) patients.Methods:A total of 87 cases AA patients were chosen. They were set as observation group, and they were divided into acute group (n=21) and chronic group (n=66) according to the condition, chose another 30 cases healthy volunteers as control group, detected the Th1 cytokine interferon-γ (IFN-γ) and Th2 cytokines interleukin-4 (IL-4) with nzyme-linked immunosorbent method, detected the Th1/Th2 cells and T cells subgroup with flow cytometry instrument between groups.Results:The Th1 cells and Th1/Th2 cells in observation group were significantly higher than control group. Th1 cells and Th1/Th2 cells in acute group were significantly higher than chronic group; The IFN-γ, IL-4 and IFN-γ/IL-4 in observation group were significantly higher than that of control group, and the IFN-γ, IL-4 and IFN-γ/IL-4 in acute group were significantly higher than chronic group; The blood CD4+, CD4+/CD8+ in observation group were significantly lower than the control group, CD8+ was significantly higher than the control group, and the blood CD4+, CD4+/CD8+ in acute group were significantly lower than the chronic group, CD8+ was significantly higher than the chronic group, all the difference was statistically significant.Conclusion:T cell subgroup imbalances and Th1 migration plays the important role in the onset of AA, and may relate to a certain degree of disease state.

  3. Altered expression of the TCR signaling related genes CD3 and FcεRIγ in patients with aplastic anemia

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    Li Bo

    2012-03-01

    Full Text Available Abstract Background Aplastic anemia (AA is characterized by pancytopenia and bone marrow hypoplasia, which results from immune-mediated hematopoiesis suppression. Understanding the pathophysiology of the immune system, particularly T cells immunity, has led to improved AA treatment over the past decades. However, primary and secondary failure after immunosuppressive therapy is frequent. Thus, knowledge of the immune mechanisms leading to AA is crucial to fundamentally understand the disease. Findings To elucidate the T cell receptor (TCR signal transduction features in AA, the expression levels of CD3γ, δ, ε and ζ chain and FcεRIγ genes, which are involved in TCR signal transduction, and the negative correlation of the expression levels between the CD3ζ and FcεRIγ genes in T cells from peripheral blood mononuclear cells (PBMCs were analyzed. Real-time RT-PCR using the SYBR Green method was used to detect the expression level of these genes in PBMCs from 18 patients with AA and 14 healthy individuals. The β2microglobulin gene (β2M was used as an endogenous reference. The expression levels of the CD3γ, CD3δ, CD3ε and CD3ζ genes in patients with AA were significantly increased compared to a healthy control group, whereas the FcεRIγ gene expression level was significantly decreased in patients with AA in comparison with the healthy control group. Moreover, the negative correlation of the expression levels between the CD3ζ and FcεRIγ genes was lost. Conclusions To our knowledge, this is the first report of the CD3γ, CD3δ, CD3ε, CD3ζ and FcεRIγ gene expression in patients with AA. The abnormally expressed TCR signaling related genes may relate to T cells dysfunction in AA.

  4. Long-term outcome of 25 children and adolescents with severe aplastic anemia treated with antithymocyte globulin

    Directory of Open Access Journals (Sweden)

    de-Medeiros C.R.

    2000-01-01

    Full Text Available Severe aplastic anemia (SAA is probably an immune-mediated disorder, and immunosuppressive therapy is recommended for patients with no available donor for bone marrow transplant. Between October 1984 and November 1987, 25 consecutive children and adolescents with SAA with no HLA-compatible marrow donor received equine antithymocyte globulin (ATG (15 mg kg-1 day-1 for 10 days. The patients were evaluated 6 weeks, 6 months, and 12 months after starting ATG treatment. Thereafter, patients were evaluated yearly until July 1998. Median age was 10 years (range, 1.5-20 years, granulocyte counts on referral ranged from 0.032 to 1.4 x 10(9/l (median 0.256 x 10(9/l, and 12 patients had granulocyte counts <0.2 x 10(9/l. At a median follow-up of 9.6 years (range, 8.6-11.8 years, 10 patients (40% remained alive with good marrow function. No morphologic evidence of hematological clonal disorders has been observed, although two patients probably have acquired clonal chromosomal abnormalities (trisomy 8 and del(6q21, respectively. Responses to ATG were observed between 6 weeks and 6 months from the start of treatment in 60% of evaluable patients. The response rate was not different in patients whose granulocyte count at diagnosis was <0.2 x 10(9/l, or in those who were <10 years of age. This study supports the view that, when compared with supportive measures, ATG is an effective treatment for children or adolescents with SAA. Although these results are inferior to those reported for marrow transplantation or more intensive immunosuppressive regimens, these patients who responded to ATG are long-term survivors with stable peripheral blood counts and a low rate of relapse.

  5. Reduced intensity conditioning, combined transplantation of haploidentical hematopoietic stem cells and mesenchymal stem cells in patients with severe aplastic anemia.

    Directory of Open Access Journals (Sweden)

    Xiao-Hong Li

    Full Text Available We examined if transplantation of combined haploidentical hematopoietic stem cells (HSC and mesenchymal stem cells (MSC affected graft failure and graft-versus-host disease (GVHD in patients with severe aplastic anemia (SAA. Patients with SAA-I (N = 17 received haploidentical HSCT plus MSC infusion. Stem cell grafts used a combination of granulocyte colony-stimulating factor (G-CSF-primed bone marrow and G-CSF-mobilized peripheral blood stem cells of haploidentical donors and the culture-expanded third-party donor-derived umbilical cord MSCs (UC-MSCs, respectively. Reduced intensity conditioning consisted of fludarabine (30 mg/m2·d+cyclosphamide (500 mg/m2·d+anti-human thymocyte IgG. Transplant recipients also received cyclosporin A, mycophenolatemofetil, and CD25 monoclonal antibody. A total of 16 patients achieved hematopoietic reconstitution. The median mononuclear cell and CD34 count was 9.3×10(8/kg and 4.5×10(6/kg. Median time to ANC was >0.5×10(9/L and PLT count >20×10(9/L were 12 and 14 days, respectively. Grade III-IV acute GVHD was seen in 23.5% of the cases, while moderate and severe chronic GVHD were seen in 14.2% of the cases. The 3-month and 6-month survival rates for all patients were 88.2% and 76.5%, respectively; mean survival time was 56.5 months. Combined transplantation of haploidentical HSCs and MSCs on SAA without an HLA-identical sibling donor was safe, effectively reduced the incidence of severe GVHD, and improved patient survival.

  6. Decreased expression of vitamin D receptor may contribute to the hyperimmune status of patients with acquired aplastic anemia.

    Science.gov (United States)

    Yu, Wei; Ge, Meili; Lu, Shihong; Shi, Jun; Feng, Sizhou; Li, Xingxin; Zhang, Jizhou; Wang, Min; Huang, Jinbo; Shao, Yingqi; Huang, Zhendong; Zhang, Jing; Nie, Neng; Zheng, Yizhou

    2016-05-01

    Acquired aplastic anemia (AA) is an immune-mediated bone marrow failure syndrome. 1α,25-Dihydroxyvitamin D3 [1,25(OH)2 D3 ], the biologically active metabolite of vitamin D, is a critical modulator of immune response via binding with vitamin D receptor (VDR). Previous studies have established that 1,25(OH)2 D3 and VDR were involved in the pathogenesis of some autoimmune diseases. In this study, we evaluated the involvement of 1,25(OH)2 D3 and VDR on T-cell responses in AA. Plasma 25(OH)D3 levels were comparable between patients with AA and healthy controls. Surprisingly, VDR mRNA was significantly lower in untreated patients with AA than in healthy controls. Subsequent in vitro experiments revealed that 1,25(OH)2 D3 treatment suppressed the proliferation of lymphocytes and inhibited the secretion of interferon-γ, tumor necrosis factor-α, and interleukin-17A, meanwhile promoting the production of transforming growth factor-β1 in patients with AA. Moreover, 1,25(OH)2 D3 inhibited the differentiation of type 1 and Th17 cells but induced the differentiation of type 2 and regulatory T cells. Interestingly, VDR mRNA was elevated in healthy controls after 1,25(OH)2 D3 treatment, but not in patients with AA. In conclusion, decreased expression of VDR might contribute to the hyperimmune status of AA and appropriate vitamin D supplementation could partly correct the immune dysfunction by strengthening signal transduction through VDR in patients with AA.

  7. Cyclosporine restores hematopoietic function by compensating for decreased Tregs in patients with pure red cell aplasia and acquired aplastic anemia.

    Science.gov (United States)

    Dao, An T T; Yamazaki, Hirohito; Takamatsu, Hiroyuki; Sugimori, Chiharu; Katagiri, Takamasa; Maruyama, Hiroyuki; Zaimoku, Yoshitaka; Maruyama, Kana; Ly, Trung Q; Espinoza, Luis; Nakao, Shinji

    2016-04-01

    Most patients with acquired pure red cell aplasia (PRCA) and some with acquired aplastic anemia (AA) respond well to cyclosporine (CsA), but thereafter often show CsA dependency. The mechanism underlying this dependency remains unknown. We established a reliable method for measuring the regulatory T cell (Treg) count using FoxP3 and Helios expression as markers and determined the balance between Tregs and other helper T cell subsets in 16 PRCA and 29 AA patients. The ratios of interferon-γ-producing CD4(+) (Th1) T cells to Tregs in untreated patients and CsA-dependent patients were significantly higher (PRCA 5.77 ± 1.47 and 7.38 ± 2.58; AA 6.18 ± 2.35 and 8.94 ± 4.06) than in healthy volunteers (HVs; 3.33 ± 0.90) due to the profound decrease in the percentage of Tregs. In contrast, the ratios were comparable to HVs in convalescent CsA-treated AA patients (4.74 ± 2.10) and AA patients in remission after the cessation of CsA treatment (4.24 ± 1.67). Low-dose CsA (100 ng/ml) inhibited the proliferation of conventional T cells (Tconv) to a similar degree to the inhibition by Tregs in a co-culture with a 1:1 Treg/Tconv ratio. The data suggest that CsA may reverse the hematopoietic suppression in PRCA and AA patients by compensating for the inadequate immune regulatory function that occurs due to a profound decrease in the Treg count.

  8. Alteration in Marrow Stromal Microenvironment and Apoptosis Mechanisms Involved in Aplastic Anemia: An Animal Model to Study the Possible Disease Pathology

    Directory of Open Access Journals (Sweden)

    Sumanta Chatterjee

    2010-01-01

    Full Text Available Aplastic anemia (AA is a heterogeneous disorder of bone marrow failure syndrome. Suggested mechanisms include a primary stem cell deficiency or defect, a secondary stem cell defect due to abnormal regulation between cell death and differentiation, or a deficient microenvironment. In this study, we have tried to investigate the alterations in hematopoietic microenvironment and underlying mechanisms involved in such alterations in an animal model of drug induced AA. We presented the results of studying long term marrow culture, marrow ultra-structure, marrow adherent and hematopoietic progenitor cell colony formation, flowcytometric analysis of marrow stem and stromal progenitor populations and apoptosis mechanism involved in aplastic anemia. The AA marrow showed impairment in cellular proliferation and maturation and failed to generate a functional stromal microenvironment even after 19 days of culture. Ultra-structural analysis showed a degenerated and deformed marrow cellular association in AA. Colony forming units (CFUs were also severely reduced in AA. Significantly decreased marrow stem and stromal progenitor population with subsequently increased expression levels of both the extracellular and intracellular apoptosis inducer markers in the AA marrow cells essentially pointed towards the defective hematopoiesis; moreover, a deficient and apoptotic microenvironment and the microenvironmental components might have played the important role in the possible pathogenesis of AA.

  9. Ileal mass-like lesion induced by Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a patient with aplastic anemia.

    Science.gov (United States)

    Min, Kyueng-Whan; Jung, Ho Young; Han, Hye Seung; Hwang, Tae Sook; Kim, Sung-Yong; Kim, Wan Seop; Lim, So Dug; Kim, Wook Youn

    2015-01-01

    Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening hyperinflammatory syndrome characterized by activated macrophages engulfing erythrocytes, leukocytes, platelets, and their precursor cells in bone marrow, liver, spleen, or lymph nodes. We report a case of Epstein-Barr virus (EBV)-associated HLH unusually presenting as an ileal mass. A 23-year-old man presented initially with persistent fever unresponsive to antibiotics and pancytopenia. A bone marrow aspiration and biopsy were used to diagnose the patient with aplastic anemia and HLH. A relatively well-defined low-density mass was radiologically noted in the terminal ileum, along with enlarged lymph nodes, and was suspected to be malignant lymphoma or an abscess. The ileocecectomy specimen revealed a transmural hemorrhagic infarction with numerous activated macrophages phagocytosing erythrocytes, plasma cells, and lymphocytes, and he was diagnosed with EBV-associated HLH. The patient received an allo-unrelated peripheral blood stem-cell transplantation and expired due to graft-versus-host disease following liver failure. The present case is very unique, in that EBV-associated HLH presented with an unusual ileal mass resulting from hemorrhagic infarction in a patient with aplastic anemia, suggesting variability in the biological behavior of EBV-associated disease.

  10. Concurrent Epstein-Barr virus associated NK/T cell lymphoma after immunosuppressive therapy for aplastic anemia: report of a case and review of literature.

    Science.gov (United States)

    Yin, Guangli; Ni, Ying; Xiao, Zhengrui; He, Guangsheng; Miao, Kourong

    2015-01-01

    Aplastic anemia (AA) patients with prolonged immunosuppression have a risk of development of lymphoproliferative disorders (LPDs), especially combined with Epstein-Barr virus (EBV) infection. However, development of nature killer/T (NK/T) cell lymphoma, in a nontransplantation setting, has not been documented for AA patients with immunosuppressive therapy (IST). Herein, we described a middle-aged man, Han ethnic, who presented with swelled parotid gland after a long history of IST for AA. Fever, night sweating, weight loss had not been found. Increased heterotypic lymphocytes had been detected in the left side of parotid gland demonstrated as cCD3(+), CD56(+), GranB(+), TIA-1(+), MUM-1(+), KI-67 (50%-75%)(++), Bcl-6(-), MPO(-) by immunohistochemistry, and in-situ hybridization (ISH) indicated EBER positive. Chromosome analysis by R banding method revealed 46, XY [20]. NK/T cell lymphoma concurrent with aplastic anemia was diagnosed and a mild chemotherapy regimen including vincristine, prednisone, L-asparaginase was administered. The parotid mass was gradually regressed after the first cycle of chemotherapy. The patient discharged from the hospital voluntarily and lost the follow-up.

  11. Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP).

    Science.gov (United States)

    Barone, Angelica; Lucarelli, Annunziata; Onofrillo, Daniela; Verzegnassi, Federico; Bonanomi, Sonia; Cesaro, Simone; Fioredda, Francesca; Iori, Anna Paola; Ladogana, Saverio; Locasciulli, Anna; Longoni, Daniela; Lanciotti, Marina; Macaluso, Alessandra; Mandaglio, Rosalba; Marra, Nicoletta; Martire, Baldo; Maruzzi, Matteo; Menna, Giuseppe; Notarangelo, Lucia Dora; Palazzi, Giovanni; Pillon, Marta; Ramenghi, Ugo; Russo, Giovanna; Svahn, Johanna; Timeus, Fabio; Tucci, Fabio; Cugno, Chiara; Zecca, Marco; Farruggia, Piero; Dufour, Carlo; Saracco, Paola

    2015-06-01

    Acquired aplastic anemia (AA) is a rare heterogeneous disease characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3/million inhabitants/year, in Europe, but higher in East Asia. Survival in severe aplastic anemia (SAA) has markedly improved in the past 2 decades because of advances in hematopoietic stem cell transplantation, immunosuppressive and biologic drugs, and supportive care. In SAA hematopoietic stem cell transplant (HSCT) from a matched sibling donor (MSD) is the treatment of choice. If a MSD is not available, the options include immunosuppressive therapy (IST) or unrelated donor HSCT. The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary, evidence-based document issued by a group of pediatric hematologists was discussed, modified and approved during a series of "Consensus Conferences" according to procedures previously validated by the AIEOP Board. The guidelines highlight the importance of referring pediatric patients with AA to pediatric centers with long experience in diagnosis, differential diagnosis, management, supportive care and follow-up of AA.

  12. Saprochaete clavata invasive infection in a patient with severe aplastic anemia: Efficacy of voriconazole and liposomal amphotericin B with adjuvant granulocyte transfusions before neutrophil recovery following allogeneic bone marrow transplantation

    Directory of Open Access Journals (Sweden)

    Simon Favre

    2016-03-01

    Full Text Available We report a case of a 27-year old man with severe aplastic anemia who developed a Saprochaete clavata (Geotrichum clavatum disseminated invasive infection shortly prior a scheduled allogeneic bone marrow transplantation. Treatment with a combination of voriconazole, liposomal amphotericin B and adjuvant granulocyte transfusions was successful before neutrophil recovery.

  13. Worse outcome and more chronic GVHD with peripheral blood progenitor cells than bone marrow in HLA-matched sibling donor transplants for young patients with severe acquired aplastic anemia.

    NARCIS (Netherlands)

    Schrezenmeier, H.; Passweg, J.R.; Marsh, J.C.; Bacigalupo, A.; Bredeson, C.N.; Bullorsky, E.; Camitta, B.M.; Champlin, R.E.; Gale, R.P.; Fuhrer, M.; Klein, J.P.; Locasciulli, A.; Oneto, R.; Schattenberg, A.V.M.B.; Socie, G.; Eapen, M.

    2007-01-01

    We analyzed the outcome of 692 patients with severe aplastic anemia (SAA) receiving transplants from HLA-matched siblings. A total of 134 grafts were peripheral blood progenitor cell (PBPC) grafts, and 558 were bone marrow (BM) grafts. Rates of hematopoietic recovery and grades 2 to 4 chronic graft-

  14. 获得性再生障碍性贫血患者端粒-端粒酶活性的变化及其意义%Changes of telomere-telomerase activity in patients with acquired aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    宋佳音; 王耀春; 肖扬

    2013-01-01

    Summary To summarize (he research progress of iclomcrc length and telomerase activity in acquired aplastic anemia(Aaa). A full text database system based on PUBMED and CNK1 was adopted for searching relevant literature from 1996 to 2012 with the key words of "telomere.telomerase and acquired aplastic anemia". Criterion for data selection-The literature about advances of telomere, telomerase and acquired aplastic anemia was selected for a-nalysis. According to the standard.236 articles were analyzed. Acquired aplastic anemia is hone marrow failure syndromes that mainly results from immune-mediated destruction of hematopoiesis. About one-third of patients with Aaa have short telomeres in peripheral white blood cells.some of which have heterozygous mutations in genes encoding the telomerase components of TERT or TERC Clinical observation found that most Aaa patients with telomere shortening were insensitive to immunosuppressive therapy;the disease duration increased and the prognosis was poor. Short telomeres may cause malignant clonal diseases in the advanced .stage.such as MDS.AML. Treatment plan for these Aaa patients with shortening telomere should he adjusted. They must undergo haematopoietic stem cell transplant ( HSCT) as soon as possible,and family donors with relevant mutations must he avoided. The Aaa patients with telomere related abnormality are different from other patients regarding pathological mechanism,disease progress, treatment and prognosis. Only a small proportion of these eases could be explained by TERT or TERC mutation. Other mutation or causes may remain unknown .

  15. Detection and Significance of CD4+CD25+CD127dim Regulatory T Cells in Individuals with Severe Aplastic Anemia

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    Weiwei Qi

    2015-09-01

    Full Text Available Objective: To investigate the relationship between CD4+CD25+CD127dim regulatory T cells (Tregs and immune imbalance in acquired severe aplastic anemia (SAA. Materials and Methods: The quantity of CD4+CD25+CD127dim Tregs in 44 SAA patients and 23 normal controls was measured by flow cytometry. Correlations between Tregs and T cell subsets, dendritic cell (DC subsets, granulocyte counts, and percentage of reticulocytes (RET% were analyzed. Results: The percentage of CD4+CD25+CD127dim Tregs in peripheral blood lymphocytes (PBLs of untreated patients was lower than in recovery patients and normal controls (0.83±0.44% vs. 2.91±1.24% and 2.18±0.55%, respectively, p<0.05. The percentage of CD4+CD25+CD127dim Tregs in CD4+ T lymphocytes of recovery patients was higher than that of untreated patients and normal controls (9.39±3.51% vs. 7.61±5.3% and 6.83±1.4%, respectively, p<0.05. The percentage of CD4+ T lymphocytes in PBLs of untreated patients was lower than in recovery patients and normal controls (13.55±7.37% vs. 31.82±8.43% and 32.12±5.88%, respectively, p<0.05. T cell subset (CD4+/CD8+ ratio was 0.41±0.24 in untreated patients, which was lower than in recovery patients (1.2±0.4 and normal controls (1.11±0.23 (p<0.05. DC subset (myeloid DC/plasmacytoid DC ratio, DC1/DC2 ratio was 3.08±0.72 in untreated patients, which was higher than in recovery patients (1.61±0.49 and normal controls (1.39±0.36 (p<0.05. The percentage of CD4+CD25+CD127dim Tregs in PBLs was positively associated with T cell subset (r=0.955, p<0.01 and negatively associated with DC subset (r=-0.765, p<0.01. There were significant positive correlations between CD4+CD25+CD127dim Tregs/PBL and granulocyte counts and RET% (r=0.739 and r=0.749, respectively, p<0.01. Conclusion: The decrease of CD4+CD25+CD127dim Tregs in SAA patients may cause excessive functioning of T lymphocytes and thus lead to hematopoiesis failure in SAA.

  16. Studies of the response in mixed leukocyte culture of cells from patients with aplastic anemia to cells from HLA-identical siblings.

    Science.gov (United States)

    Mickelson, E M; Clift, R A; Fefer, A; Storb, R; Thomas, E D; Warren, R P; Hansen, J A

    1981-08-01

    We have studied the mixed leukocyte culture (MLC) reactions of 64 patients with severe aplastic anemia. Their peripheral blood mononuclear cells showed an increased relative response (RR) to cells from HLA-identical siblings as compared to cells from normal HLA-identical siblings, confirming the results reported in an earlier series of 34 patients. Elevated RRs were associated with patient antidonor lymphocyte antibodies as detected by the antibody-dependent cell-mediated cytotoxicity assay, but were not associated with antidonor complement-dependent cytotoxic antibodies or with antidonor cytotoxic T lymphocytes. Among 98 patients receiving marrow grafts from HLA-identical sibling donors, those with elevated RRs before transplantation were more apt to reject the transplant than those without (P less than 0.0001). There was no elevation of the RR in 10 untransfused patients, although positive RRs were noted in 2 patients within 12 to 24 hr of their first transfusions. Five patients with identical twins were also tested, and elevated RRs were noted in three. Although blood transfusion appears to be responsible for the increased RRs observed in some aplastic patients, genetic differences between donor and recipient were not always necessary for eliciting an increased MLC response, suggesting that mechanisms other than alloimmunization are involved.

  17. Effects of Serum from Aplastic Anemia patients on the Expression of Cyclin D3 Isoform in Umbilical Cord Blood CD34+ Cells

    Institute of Scientific and Technical Information of China (English)

    孟凡凯; 谭细友; 刘文励; 孙汉英; 周剑锋; 李春蕊; 刘丹; 何莉; 孙岚

    2004-01-01

    Summary: The pathogenesis of aplastic anemia (AA) was explored and the effects of AA serum on the expression of crucial cyclin D isoform (cyclin D3) in umbilical cord blood hematopoietic stem/progenitor cells were observed. The CD34+ cells were isolated from the cord blood with MIDIMACS Semi-solid methylcellulose culture technique was used to measure the formation of CFUGM;The expression level of cyclin D3 was assayed by semi-quantitative RT-PCR and Western-blot after the hematopoietic stem/progenitor cells were incubated in AA serum. The results showed that the AA serum could inhibit the formation of CFU-GM and down regulate the expression level of the cyclin D3 at the mRNA and protein level respectively. In conclusion, the AA serum could inhibit the proliferation of hematopoietic stem cells and down regulate level of cyclin D3, which might be one mechanism of hematopoiesis inhibition in AA.

  18. Dynamics of Graft Function Measured by DNA-Technology in a Patient with Severe Aplastic Anemia and Repeated Stem Cell Transplantation

    Directory of Open Access Journals (Sweden)

    Anna Karastaneva

    2014-01-01

    Full Text Available Although bone marrow transplantation (BMT from an HLA identical sibling is considered as treatment of choice in pediatric patients with severe aplastic anemia (SAA, a significant number of them experience graft failure (GF after BMT. We report a case of an 8-year-old male patient with SAA who presented with a complicated posttransplant course due to parvovirus B19 infection and GF. A subsequent attempt to support the graft by antithymocyte globulin (ATG and a peripheral stem cell boost resulted in transitory autologous recovery of hematopoiesis followed by mixed chimerism, supported by donor lymphocyte infusions (DLIs and finally graft rejection with relapse of SAA. Permanent complete chimerism was achieved by a second BMT. Dynamics of graft function, measured by a single nucleotide polymorphism (SNPs analysis, are discussed.

  19. 中西医结合规范化诊治再生障碍性贫血的思路探讨%Discussion on Treatment and Diagnosis of Aplastic Anemia in Combination of Chinese and Western Medicine

    Institute of Scientific and Technical Information of China (English)

    魏学礼; 马济佩; 胡明辉

    2012-01-01

    再生障碍性贫血是一种严重危害人类健康的血液病,主要的治疗方法有骨髓移植、免疫抑制疗法,以及中医药等.近年来随着循证医学兴起,人们对再障认识的更加深入,再障的定义、分型以及治疗路径等都发生了一些新的变化,如何利用这些新进展加快再障中医规范化诊治,如何对起病缓急不同、轻重程度各异的再障患者选择恰当的中西医治疗方案,常常是中医界面临的困惑之一.目前我国尚无治疗再障的规范诊治指南,结合再障诊疗的新进展和近期的中医文献探讨再障的中西医结合规范化诊疗的思路和方法.%Aplastic anemia is a serious blood disease, the main treatments include bone marrow transplantation, immunosup-pressive therapy and traditional Chinese medicine. With the rise of evidence - based medicine in recent years,people know more about aplastic anemia. The definition, classification and treatment paths of aplastic anemia have undergone some new changes. How to use these new developments to speed up the TCM standardization of diagnosis and treatment of aplastic anemia, how to select proper treatment for diseases with different degrees, these problems are faced by the Chinese medicine physicians. At present, Chinese medicine has no diagnosis and treatment guidelines of aplastic anemia. In this paper,based on the latest progress and the recent Chinese literatures, it explored the ideas of diagnosis and treatment of aplastic anemia.

  20. 浅谈妊娠期再生障碍性贫血的诊疗效果%To Explore the Effect of Treatment for Pregnancy Combine With Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    靳宝霞

    2015-01-01

    ObjectiveTo investigate the treatment of aplastic anemia in pregnancy.Methods20 cases of pregnant patients with aplastic anemia in our hospital from January 2014 to December 2014 were selected in this study.Results In the 20 cases of pregnancy with aplastic anemia patients, six cases had vaginal delivery, 14 cases had cesarean section. All of them received intraoperative blood transfusion without stillbirth. All of the babies were alive before they left hospital.Conclusion Pregnant patients with aplastic anemia need cautious treatment. During the pregnancy, they should receive rigor guardianship, for early detection and early treatment, in order to reduce the harm for both of the mother and baby.%目的:探讨妊娠期合并贫血的治疗及疗效。方法随机选取我院2014年1月~2014年12月间收治的经妊娠期合并贫血的患者20例进行研究分析。结果20例患有妊娠合并再生障碍性贫血的患者,经阴道分娩6例,剖宫产14例,全部进行术中输血没有死胎现象,至出院前婴儿全部存活。结论再障患者妊娠需谨慎,对孕期要进行严密监护,争取对病情早发现早治疗,减少对母儿的危害。

  1. 异基因造血干细胞移植治疗儿童再生障碍性贫血%Allogeneic hematopoietic stem cell transplantation in children with aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    罗成娟; 陈静

    2010-01-01

    @@ 再生障碍性贫血(aplastic anemia,AA)是一组由于化学、物理、生物因素及不明原因引起的骨髓造血功能衰竭,以造血干细胞损伤、外周血全血细胞减少为特征的异质性疾病,包括先天性和获得性AA.

  2. Effects of Endocrine Hormone Receptors on Patients with Aplastic Anemia%内分泌激素受体对再生障碍性贫血的影响

    Institute of Scientific and Technical Information of China (English)

    陈珍珍; 王博; 武利强; 胡致平; 俞庆宏; 郑智茵; 张宇; 庄海峰; 林圣云

    2013-01-01

    目的:探讨糖皮质激素受体(glucocorticoid receptor,GR)、雄激素受体(androgen receptor,AR)表达对再生障碍性贫血(aplastic anemia,AA)影响.方法:利用酶联免疫吸附试验(ELISA)法检测急性再生障碍性贫血(acute aplastic anemia,AAA)、慢性再生障碍性贫血(chronic aplastic anemia,CAA)以及对照组的外周血单个核细胞胞浆和胞核内AR、GR阳性表达水平.结果:与对照组相比,AAA组、CAA组的AR、GR显著低于对照组(P<0.05);AAA组与CAA组相比,AAA组AR、GR阳性水平显著高于CAA组(P<0.05).结论:AA患者的AR、GR表达水平下降,提示AA的发病与受体水平密切相关.

  3. Detection of CD4+CD25+ T cells in peripheral blood of patients with aplastic anemia%再生障碍性贫血患者外周血CD4+CD25+ T细胞的检测

    Institute of Scientific and Technical Information of China (English)

    王卫国; 马黎丽; 马芳; 李玉云

    2012-01-01

    Objective: To explore the relationship between the level of CD4 + CD25 + T cells and pathogenesis or prognosis of aplastic anemia by detecting CD4 + CD25 + T cells in peripheral blood. Methods: The proportion of CD4 + CD25 + T cells and CD4 + CD25high T cells in peripheral blood of four groups, including 20 cases of healthy individuals as normal controls ,30 cases of patients with first onset of aplastic anemia, 10 cases of patients accepted effective treatment and 10 cases of patients accepted invalid treatment, were determined by flow cytometry. Results: The rate of CD4 + CD25 + T cells in peripheral blood of four groups was not significantly different ( P > 0.05) ; Compared with group of normal controls, the rate of CD4+ CD25high T cells in patients with first onset of aplastic anemia decreased significantly ( P 0. 05 ). Conclusions: The decreasing of CD4 + CD25highT cells is one of the reasons causing breakdown of immune tolerance in patients with aplastic anemia,the rate of CD4 CD25 T cells may not reflect the prognosis of the patients.%目的:通过对外周血CD4+CD25+T细胞的检测,初步探讨CD4+CD25+T细胞水平与再生障碍性贫血(aplastic anemia,AA)发病和预后的关系.方法:流式细胞术分别检测20例正常对照、30例初发AA患者、10例治疗有效AA患者和10例治疗无效AA患者的CD4+CD25+T细胞和CD4+CD25highT细胞水平.结果:4组患者CD4+CD25+T细胞比例差异无统计学意义(P>0.05);初发AA和治疗无效组AA患者CD4+CD25highT细胞比例均低于正常对照组(P0.05).结论:CD4+CD25highT细胞比例下降是造成AA患者免疫耐受破坏的原因之一,CD4+CD25+T细胞比例可能并不能反映AA患者的预后状况.

  4. The immunological assessment of aplastic anemia mice%再生障碍性贫血小鼠模型的免疫学评价

    Institute of Scientific and Technical Information of China (English)

    张恒; 王月英; 吴红英; 李德冠; 王小春; 路璐; 常建辉; 翟志斌; 孟爱民

    2011-01-01

    目的 对再生障碍贫血(aplastic anemia,AA)实验动物模型的免疫学相关指标进行检测和评估,为动物模型提供更全面的评估指标.方法 建模BALB/C小鼠经γ射线照射后尾静脉注射DBA小鼠脾脏细胞,无菌饲养15日检测与再生障碍性贫血症相关的血常规和免疫学指标.结果 (1)模型组小鼠相比正常对照组,外周血常规指标指标与AA患者临床表现基本相符;(2)模型组小鼠外周血免疫学指标变化与临床AA患者基本相符.结论 我们成功构建AA动物模型,进行免疫学评估将有利于利用AA动物模型研究AA治疗手段%Objective Study on establishing aplastic anemia animal model and analyzing traditional and somein munologic exam inations related to pathogenesis,to provide better platform for drugs develop ment. Methods BABL/C mice were used to setup the modelby using whole body γ-ray irradiation and caudal vein injection with spleen cells of DBA mice.After 15 days of experim ental treatment, blood analysis,reticulocyte calculation,myelogram and some inmunologi cexam ination were performed.Results Mice of AA model group has maked reduction in the cells of three system sinperipheral blood ,reticulocyte and maeeow proliferation .The immunologic alteration of the AA model mice coincide with clinical clinical studies.Conclusion We successfully estalished AA animal model.Assessig im munologic alternation of the model based on clinical studies would provide us more issues to develop drugs.

  5. 再生障碍性贫血继发流感病毒感染的危险性研究%Research on danger of aplastic anemia secondary infection with influenza virus

    Institute of Scientific and Technical Information of China (English)

    郭潮潭; 尤金彪; 曹毅; 胡致平; 陈帅帅; 沃恩康; 王怡婷; 杨新燕; 张儒轩

    2015-01-01

    目的 探讨流感病毒感染对再生障碍性(再障)贫血模型小鼠造血功能的影响,以期为临床再障合并流感的研究提供参考.方法 采用环磷酰胺及甲苯法构建小鼠再障模型,观察再障模型小鼠与正常小鼠血红蛋白和血细胞三系的变化,判断造模效果.用微量病毒接种法判断流感病毒对正常小鼠及再障小鼠血细胞及生存的影响.结果 成功建立了小鼠再障模型,再障小鼠的血红蛋白、红细胞、白细胞、血小板计数显著下降,与对照组比较差异均有统计学意义(P<0.05).与未接种病毒的小鼠相比,正常小鼠微量多次接种流感病毒后除发生一过性体重轻度下降外,血细胞三系均无明显变化;而再障小鼠模型接种病毒后的病死率较正常小鼠接种病毒后显著增高.结论 流感病毒感染对正常小鼠的造血功能无明显影响,但可能会导致再障动物病情加重,甚至增加病死率.%Objective To investigate the influence of influenza virus infection on the hematopoietic function of aplastic anemia model mouse,and provide reference for research on clinical aplastic anemia influenza complication.Methods Small mice aplastic anemia model was set up by cyclophosphamide and toluene method.Through observing changes of hemoglobin and blood three series between model mouse and normal mouse to estimate the molding effect.Virus inoculation method was used to estimate the effects of influenza virus on the blood cell and survival of normal and aplastic anemia mouse.Results Aplastic anemia model mouse with chemical method were successfully established.Hemoglobin,red blood cell,white blood cell and blood platelet were reduced obviously,the differences compared with control group had statistical significance (P<0.05).In contrast with uninoculated mouse,normal mouse had no significant change in blood three series after less frequent influenza virus inoculation except for temporary body weight loss

  6. Therapeutic Effect of Shenfu Injection (参附注射液) on Secondary Aplastic Anemia of Tumor Patients after Chemotherapy

    Institute of Scientific and Technical Information of China (English)

    潘敬新; 郭奕斌; 郭熙哲; 郭健欣; 陈云峰; 王志东

    2004-01-01

    Objective: To explore the effect of Shenfu Injection (参附注射液, SFI) on the secondary aplastic anemia (AA) of tumor patients after chemotherapy (CT). Methods: The 15 cases of SFI treated group, 10 cases of control group, 25 cases of SFI + granulocyte-macrophage colony stimulating factor (GMCSF) treated group and the group of 23 GM-CSF-treated tumor cases of secondary AA after CT were compared, with their increasing rate and the rebound speed of neutrophil, platelet, bone marrow nucleated RBC, granulocyte, and megakaryocyte all being investigated. Results. The increasing rate and rebound speed of granulocyte, platelet, and the increasing rate of bone marrow nucleated RBC, granulocyte, megakaryocyte were obviously higher than those of the control, and the clinical manifestations were also obviously improved. The increasing rate of platelet, bone marrow nucleated RBC, megakaryocyte of the SFI -I- GM-CSF group were higher than those of the group which used GM-CSF alone, while the increasing rate of granulocyte in blood and bone marrow in both groups was similar. Conclusion: Significant efficacy was shown in SFI for the treatment of secondary AA of tumor patients after CT.

  7. Quantitation of CD55 and CD59 expression on reticulocytes and mature erythrocytes in paroxysmal nocturnal hemoglobinuria, aplastic anemia, and healthy control subjects.

    Science.gov (United States)

    Kim, Yeongsic; Lim, Jihyang; Kim, Myungshin; Kim, Yonggoo; Lee, Jong-Wook; Han, Kyungja

    2010-01-01

    Since PNH occasionally results in bone marrow failure, it is difficult to differentiate PNH from AA with small numbers of CD(55) (-)CD(59) (-) erythrocytes. We quantified CD55 and CD59 molecules expressed on normal reticulocytes and mature erythrocytes of paroxysmal nocturnal hemoglobinuria (PNH), aplastic anemia (AA), and normal individuals in order to determine their usefulness for differentiation between PNH and AA. A total of 56 patients (AA 39 patients, PNH 17 patients) and 10 healthy volunteers were enrolled. Two-color flow cytometric analyses were conducted using thiazole orange, anti-CD55 and CD59 monoclonal antibodies to identify CD(55) (-)CD(59) (-) reticulocytes and mature erythrocytes. Mean fluorescence level of CD(55) (+) mature erythrocytes was lowest in the PNH patients (13.2 x 10(3) MESF), and was significantly lower than in normal controls (16.7 x 10(3) MESF, p erythrocytes was lowest in the PNH subjects (24.5 x 10(3) MESF), significantly less than in normal controls (39.0 x 10(3) MESF, p erythrocytes may be useful in differentiating PNH from AA patients with small numbers of PNH-phenotype erythrocytes, and the total reticulocyte count may prove useful as a marker for PNH clone size.

  8. Nursing of Severe Aplastic Anemia Patients with Perianal Abscess%重型再生障碍性贫血伴肛周脓肿患者的护理体会

    Institute of Scientific and Technical Information of China (English)

    邓欣雨

    2014-01-01

    Objective To summarize the experience of nursing care of severe aplastic anemia patients with perianal abscess. Methods The implementation of hemostasis, anti infection and anemia cor ection and perianal special care of 1 severe aplastic anemia patients with perianal abscess. Results After the treatment and care of patients with stable vital signs, perianal painbet er, perianal abscess broken skin, smooth drainage, no redness of the skin around the. Conclusion In severe aplastic anemia patients with perianal abscess, take ef ectivesymptomatic and supportive treatment, can reduce the suf ering of patients,reduce the cost for the patient, but also improve the patient's satisfaction.%目的:探讨对重型再生障碍性贫血伴肛周脓肿患者的护理体会。方法对1例重型再生障碍性贫血伴肛周脓肿的患者实施止血、抗感染以及纠正贫血和肛周特殊护理。结果经过治疗和护理,患者生命体征平稳,肛周疼痛好转,肛周脓肿处破皮,引流通畅、周围皮肤未见发红。结论对重型再障伴肛周脓肿患者,采取有效的对症支持治疗,能充分减低患者的痛苦,降低患者的经济费用,也提高了患者的满意度。

  9. Effect observation on red blood cell transfusion for patients with chronic aplastic anemia%基于慢性再生障碍性贫血患者的红细胞输注治疗效果观察

    Institute of Scientific and Technical Information of China (English)

    吴瑞娟

    2014-01-01

    Objective To study the clinical efficacy of red cell transfusion for patients with chronic aplastic anemia (CAA). Methods From January 2012 to December 2013 year, 98 cases of patients with chronic aplastic anemia in our hospital, the red blood cell transfusion therapy, analysis of effect of transfusion of red blood cells. Results After the treatment, patients with obvious effect, correlation infusion treatment effect and red cell preparation, and had a certain relationship with the general data of patients. 98 cases were selected, 7 cases of invalid transfusion, no efficiency was 7.1%. Conclusion Chronic aplastic anemia patients with red blood cells transfusion treatment effect will be influenced by many factors, infusion treatment process should consider all relevant factors, increase the efficacy.%目的:探讨慢性再生障碍性贫血(CAA)患者红细胞输注临床疗效。方法选取本院2012年1月~2013年12月98例慢性再生障碍性贫血患者,对其进行红细胞输注治疗,分析红细胞输注效果。结果经治疗,患者效果明显,输注治疗效果与红细胞制剂存在相关性,且与患者一般资料具有一定关系。选取的98例患者中,7例无效输注,无效率为7.1%。结论慢性再生障碍性贫血患者应用红细胞输注治疗效果会因多种因素而受到影响,输注治疗过程中需综合考虑各相关因素,增加疗效。

  10. 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...

  11. Comparing Outcomes with Bone Marrow or Peripheral Blood Stem Cells as Graft Source for Matched Sibling Transplants in Severe Aplastic Anemia across Different Economic Regions.

    Science.gov (United States)

    Kumar, Rajat; Kimura, Fumihiko; Ahn, Kwang Woo; Hu, Zhen-Huan; Kuwatsuka, Yachiyo; Klein, John P; Pasquini, Marcelo; Miyamura, Koichi; Kato, Koji; Yoshimi, Ayami; Inamoto, Yoshihiro; Ichinohe, Tatsuo; Wood, William Allen; Wirk, Baldeep; Seftel, Matthew; Rowlings, Philip; Marks, David I; Schultz, Kirk R; Gupta, Vikas; Dedeken, Laurence; George, Biju; Cahn, Jean-Yves; Szer, Jeff; Lee, Jong Wook; Ho, Aloysius Y L; Fasth, Anders; Hahn, Theresa; Khera, Nandita; Dalal, Jignesh; Bonfim, Carmem; Aljurf, Mahmoud; Atsuta, Yoshiko; Saber, Wael

    2016-05-01

    Bone marrow (BM) is the preferred graft source for hematopoietic stem cell transplantation (HSCT) in severe aplastic anemia (SAA) compared with mobilized peripheral blood stem cells (PBSCs). We hypothesized that this recommendation may not apply to those regions where patients present later in their disease course, with heavier transfusion load and with higher graft failure rates. Patients with SAA who received HSCT from an HLA-matched sibling donor from 1995 to 2009 and reported to the Center for International Blood and Marrow Transplant Research or the Japan Society for Hematopoietic Cell Transplantation were analyzed. The study population was categorized by gross national income per capita and region/countries into 4 groups. Groups analyzed were high-income countries (HIC), which were further divided into United States-Canada (n = 486) and other HIC (n = 1264); upper middle income (UMIC) (n = 482); and combined lower-middle, low-income countries (LM-LIC) (n = 142). In multivariate analysis, overall survival (OS) was highest with BM as graft source in HIC compared with PBSCs in all countries or BM in UMIC or LM-LIC (P < .001). There was no significant difference in OS between BM and PBSCs in UMIC (P = .32) or LM-LIC (P = .23). In LM-LIC the 28-day neutrophil engraftment was higher with PBSCs compared with BM (97% versus 77%, P = .002). Chronic graft-versus-host disease was significantly higher with PBSCs in all groups. Whereas BM should definitely be the preferred graft source for HLA-matched sibling HSCT in SAA, PBSCs may be an acceptable alternative in countries with limited resources when treating patients at high risk of graft failure and infective complications.

  12. Isolation of a T-cell clone showing HLA-DRB1*0405-restricted cytotoxicity for hematopoietic cells in a patient with aplastic anemia.

    Science.gov (United States)

    Nakao, S; Takami, A; Takamatsu, H; Zeng, W; Sugimori, N; Yamazaki, H; Miura, Y; Ueda, M; Shiobara, S; Yoshioka, T; Kaneshige, T; Yasukawa, M; Matsuda, T

    1997-05-15

    The existence of T cells capable of inhibiting in vitro hematopoiesis has been shown in aplastic anemia (AA), although whether such inhibition is mediated by a specific immune reaction involving an HLA allele remained unknown. We isolated a CD4+ Vbeta21+ T-cell clone that was most dominant among Vbeta21+ T cells in the bone marrow (BM) of an AA patient whose HLA-DRB1 alleles included 1501 and 0405. The T-cell clone named NT4.2 lysed an autologous Epstein-Barr virus-transformed lymphoblastoid cell line (LCL) and phytohemagglutinin-stimulated lymphocytes (PHA-blasts) as well as allogeneic LCLs sharing HLA-DRB1*0405. Cytotoxicity against LCL cells and PHA-blasts by NT4.2 was blocked by anti-HLA-DR monoclonal antibody (MoAb) or anti-CD3 MoAb. NT4.2 also lysed autologous BM mononuclear cells enriched with CD34+ cells that had been cultured for one week in the presence of colony-stimulating factors as well as allogeneic CD34+ cells of a normal individual carrying HLA-DRB1*0405, cultured in the same way. Moreover, NT4.2 strongly inhibited colony formation by hematopoietic progenitor cells derived from cultured CD34+ cells sharing HLA-DRB1*0405. These results indicate that the AA patient has T cells capable of killing hematopoietic cells in an HLA-DRB1*0405-restricted manner and that such cytotoxic T cells may contribute to the pathogenesis of AA.

  13. [Treatment of transfusion-dependent nonsevere aplastic anemia with cyclosporine A plus ATG/ALG versus cyclosporine A plus androgens: a retrospective single center study].

    Science.gov (United States)

    Song, L; Peng, G X; Wu, Z J; Zhang, L; Jing, L P; Zhou, K; Li, Y; Li, Y; Ye, L; Li, J P; Fan, H H; Zhao, X; Yang, W R; Yang, Y; Zhang, F K

    2016-11-14

    Objective: To determine whether cyclosporine A (CsA) plus androgens was as effective as the current standard immunosuppressive therapy (IST) for transfusion-dependent nonsevere aplastic anemia (TD-NSAA). Methods: The records of 125 consecutive TD-NSAA patients who were treated between Aug. 2007 and Sept. 2014, with either CsA plus androgen or ALG/ATG plus CsA regimen were reviewed. The 3-month and 6-month hematologic responses and survival were evaluated. Results: There were 125 TD-NSAA patients (70 were male and 55 female, 1.25∶1). Median age was 27 (6-66) years. There was no significant difference in early mortality between 48 treated by ATG/ALG plus CsA and 77 by CsA plus androgen patients (1/48 vs 0/77, P=0.384). Both the total hematologic response and the better hematological response rates at 3-month (70.8% vs 45.5%, P=0.006 and 27.1% vs 10.4%, P=0.015, respectively) and 6-month (75.0% vs 55.8%, P=0.031 and 41.7% vs 22.1% P =0.020, respectively) after treatment were much higher in the standard IST group than that in CsA plus androgen group. The median time to transfusion independent of 36.5 (0-149) days in the standard IST group was significantly shorter than 98 (14-180) days in CsA plus androgen group (Pplus androgen was inferior to the standard IST of ATG/ALG and CsA regimen in treating TD-NSAA in terms of the hematologic response and the quality of response, despite of comparable short-term survival.

  14. Vascular endothelial growth factor corrected for platelet count and hematocrit is associated with the clinical course of aplastic anemia in children.

    Science.gov (United States)

    Kodama, Yuichi; Okamoto, Yasuhiro; Hashiguchi, Teruto; Shinkoda, Yuichi; Nishikawa, Takuro; Tanabe, Takayuki; Kawano, Yoshifumi

    2012-05-01

    The wide variety of clinical courses that lead to the development of severe aplastic anemia (AA) makes it difficult to speculate whether treatment for AA is required in the early phase. The objective of this study was to identify a method for predicting the clinical course of AA at the onset of the disease. First, in healthy adults, vascular endothelial growth factor (VEGF) released per platelet was measured by the activation of platelet-rich plasma (PRP) and platelet-poor plasma (PPP). Serum concentration of VEGF, serum concentration of VEGF corrected for platelet count, and serum concentration of VEGF corrected for both platelet count and hematocrit (corrected VEGF) were then compared to VEGF released per platelet. Corrected VEGF showed the best correlation with VEGF released per platelet by the activation of PRP in healthy subjects (R (2) = in a single 0.806, p = 0.001). Next, corrected VEGF was assayed in 11 pediatric patients with AA at the time of diagnosis. Corrected VEGF in AA patients was significantly greater than that in age-matched control subjects [1.32 × 10(-6) pg (range 0.36-1.85) vs. 0.18 × 10(-6) pg (range 0.12-0.94)] (p = 0.002). Moreover, corrected VEGF in AA patients who did not require treatment for more than 2 years was significantly greater than that in AA patients who required earlier treatment [1.67 × 10(-6) pg (range 1.32-1.85) vs. 0.87 × 10(-6) pg (0.36-1.34)] (p = 0.011). These data indicate that a compensatory mechanism for increasing VEGF and preventing disease progression might play a role in AA. Corrected VEGF may be useful for predicting the clinical course of AA.

  15. 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.

  16. De-escalation empirical antibiotic therapy improved survival for patients with severe aplastic anemia treated with antithymocyte globulin.

    Science.gov (United States)

    Fu, Rong; Chen, Tong; Song, Jia; Wang, Guojin; Li, Lijuan; Ruan, Erbao; Liu, Hui; Wang, Yihao; Wang, Huaquan; Xing, Limin; Wu, Yuhong; Liu, Hong; Qu, Wen; Shao, Zonghong

    2017-02-01

    We aimed to investigate the efficacy and safety of de-escalation empirical therapy for controlling infection in patients with severe aplastic anaemia (SAA) treated with antithymocyte globulin (ATG). Eighty-seven ATG-treated SAA patients who had microbiological culture-confirmed infections from 2006 to 2015 in our center were retrospectively analyzed. The efficacy of de-escalation and non-de-escalation therapy was compared. Among all 87 patients, 63 patients were treated with de-escalation therapy and 24 patients with non-de-escalation therapy. More patients showed response to anti-infection treatment in de-escalation group than in non-de-escalation group both on day 7 (60.32% vs. 25.00%, P = 0.003) and on day 30 (79.37% vs. 58.33%, P = 0.047) since the initial antimicrobial therapy. On day 30, more patients had increased absolute neutrophil count in de-escalation group compared with non-de-escalation group (76.19% vs. 45.83%, P = 0.007), and de-escalation group had lower morality rate (17.46% vs. 37.50%, P = 0.047) and better survival outcome (P = 0.023) on day 90. Twenty-three patients in de-escalation group and 5 patients in non-escalation group received granulocyte transfusions. Granulocyte transfusions helped to control infections in both de-escalation group (P = 0.027) and non-de-escalation group (P = 0.042) on day 7, but did not improve survival on day 90. We concluded that de-escalation antibiotics improved survival in SAA patients after ATG treatment. Early administration of broad-spectrum antibiotics pending microbiological cultures combined with a commitment to change to narrow-spectrum antibiotics should be recommended for controlling infections in SAA patients treated with ATG. Granulocyte transfusions might be an adjunctive therapy in controlling infections.

  17. Clinical Study on Effect of Astragalus Injection (黄芪注射液) and Its Immuno-regulation Action in Treating Chronic Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    WANG Mao-sheng; LI Jun; DI Hai-xia; LI Zhen-li; YANG Shu-Lian; HOU Wei; YAN Jin-yu; ZHAO Xiao-min

    2007-01-01

    Objective: To observe the clinical effect of Astragalus Injection (黄芪注射液, Al) and its immuno-regulatory action in treating chronic aplastic anemia (CAA). Methods: Sixty patients with CAA were randomly assigned to two groups equally, both were treated with Stanozolol three times a day, 2 mg each time through oral intake, but Al was given additionally to the patients in the treated group once a day via intravenous dripping. All were treated for 15 days as one therapeutic course and the whole medication lasted for more than 4 months totally, with follow-up adopted. The clinical efficacy was estimated and the changes of T-lymphocyte subsets in peripheral blood as well as the serum levels of tumor necrosis factor-α (TNF-α) and interleukin-2 (IL-2) were observed. Results: The total effective rate in the treated group was 83.3% (25/30), which was higher than that in the control group 66.7% (20/30), showing significant difference between them (P<0.05). Levels of hemoglobin,WBC, reticular cell and platelet were elevated in both groups after treatment, but the improvement was significantly better in the treated group than that in the control group with respect to the former three indexes (P<0.05). The level of CD4+ increased and that of CD8+ decreased significantly after treatment in the treated group (P<0.05), which showed significant difference as compared with those in the control group (P<0.05). Levels of serum TNF- α and IL-2 lowered after treatment in both groups, but significance only showed in the treated group (P<0.05). The degree of proliferation in bone marrow got raised significantly and the percentage of non-hemopoietic cells reduced significantly in the treated group after treatment, also showing significant difference to those in the control group (P<0.05).Conclusion: Al could promote the recovery of hemopoietic function, which might be through improving T-lymphocyte subsets and reducing the release of negative regulatory factors such

  18. Incidence of adult aplastic anemia in Shanghai, China%上海市成人再生障碍性贫血发病率调查

    Institute of Scientific and Technical Information of China (English)

    王蔚; 王小钦; 李佩; 林果为

    2011-01-01

    目的 调查上海市成人获得性再生障碍性贫血(AA)发病率,并与20世纪80年代的AA发病率以及国外的AA发病率进行比较,了解其变化趋势.方法 采用回顾性调查的方式对2004至2006年上海市静安区、徐汇区、黄浦区、长宁区、普陀区、杨浦区6个区域的成人新发AA患者进行登记调查,计算这6个区的成人AA发病率情况.结果 上海市6个区内2004年至2006年新诊断成人AA患者共38例,平均年发病率0.33/10万,其中18~34岁、35~59岁和≥60岁人群的年发病率分别为0.40/10万、0.14/10万和0.64/10万,其中重型AA的平均年发病率为0.17/10万.结论 与20世纪80年代相比,上海市成人AA发病率有所下降,但重型AA发病率无明显变化.%Objective To survey the incidence of acquired adult aplastic anemia (AA) in Shanghai, China. Meanwhile, we compared it with the previous data from China in 1986 and other countries in order to explore the trends. Methods Newly diagnosed AA patients were registered in 6 districts (Jingan, Xuhui, Huangpu, Changning, Putuo, Yangpu ) in Shanghai from 2004 to 2006. Then we calculated the crude and age-adjusted incidence of AA according to the population data from Shanghai Statistic Yearbook. Results There were 38 adult patients with acquired AA. The average crude incidence of AA was 0. 33/100 000 from 2004 to 2006. The incidences per 100 000 persons per year were 0. 40, 0. 14and 0. 64 in 18-34, 35-59 and ≥ 60 years, respectively. The rate of severe AA was 0. 17/100 000.Conclusion The incidence of severe AA has no marked change, but the total rate is a little decreased compared with the data from China in 1986.

  19. 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.

  20. Anemia

    Science.gov (United States)

    ... are affected. Low levels of red blood cells leads to anemia. With low levels of white blood cells, the ... foods they eat. Food fads and dieting can lead to anemia. Talk to your doctor about taking iron pills ( ...

  1. B-cell-rich T-cell lymphoma associated with Epstein-Barr virus-reactivation and T-cell suppression following antithymocyte globulin therapy in a patient with severe aplastic anemia

    Directory of Open Access Journals (Sweden)

    Nobuyoshi Hanaoka

    2015-09-01

    Full Text Available B-cell lymphoproliferative disorder (B-LPD is generally characterized by the proliferation of Epstein-Barr virus (EBV-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.

  2. 儿童再生障碍性贫血淋巴细胞亚群及与血常规相关性的研究%Lymphocyte subsets and its correlation with blood count in children with aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    曹萍; 蒋莎义; 谢晓恬; 周妮娜; 李福兴; 覃大卫

    2011-01-01

    Objective To investigate the distribution of lymphocyte subsets in peripheral blood and its correlation with complete blood count in children with aplastic anemia. Methods The lymphocyte subsets CD3+ , CD3 + CD4+ , CD3 + CD8 + , CD3+ CD4 VCD3 + CD8 + , CD3 + CD45RA+, CD3 + CD45RO+ , CD(16 +56) + ,CD19 +from 42 aplastic anemia children and 27 controls were examined by flow cytometry; its correlations with complete blood count were analyzed. Results CD3 + CD4+ and CD3 + CD4 VCD3 + CD8+ decreased while CD3 + CD8+ increased in aplastic anemia patients. CD3 + CD8+ was positively correlated with peripheral HB, WBC counts. CD3+ CD4VCD3+ CD8+ was negatively correlated with peripheral HB, WBC and N counts. Conclusion There are abnormal distributions of lymphocyte subsets in aplastic anemia patients, which may be involved in pathogenesisof the disease.%目的 研究再生障碍性贫血患儿外周血淋巴细胞亚群分布,及其与同期外周血血常规的相关性.方法 采用流式细胞技术对我院42例AA患儿和27例正常儿童外周血淋巴细胞亚群CD3+,CD3+ CD4+,CD3+CD8+,CD3+ CD4+/CD3+ CD8+,CD3+ CD45 RA+,CD3+ CD45RO+,CD( 16 +56)+,CD19+的表达进行检测,同时检测血常规.结果 再障组CD3+ CD4+,CD3+ CD8+,CD3+ CD4+/CD3+ CD8+的平均值分别为(26.94±11.93)%,(33.33±10.3)%,0.918±0.53,对照组分别为(33.53±9.97)%,(27.08±9.23)%,1.44±0.80,差异有显著性(P<0.05);CD3+ CD8+与HB,WBC呈一定程度的正相关,CD3+ CD4+/CD3+ CD8+比值与HB,WBC,N呈一定程度的负相关.结论 再障患儿存在淋巴细胞亚群的失调,其中由T淋巴细胞介导的免疫异常对造血功能的抑制在再障的发生中起重要作用,再障患儿Th格局向Th1偏移.

  3. Clinical features of aplastic anemia patients with hepatitis B virus infection: a case report of 12 patients%乙型肝炎病毒相关再生障碍性贫血12例临床特征分析

    Institute of Scientific and Technical Information of China (English)

    王亚东; 樊翠翠; 赵彩彦; 张莉; 于伟燕; 甄真; 米立军

    2012-01-01

    本研究回顾了12例HBV相关再生障碍性贫血(hepatitisassociated aplastic anemia,HAAA)患者临床资料,探讨本病发病机制、临床特征、诊治方案以及预后转归等特点,现报道如下. 一、资料与方法 1.研究对象:收集1996年1月至2011年6月河北医科大学第三医院感染科、肝病科以及血液科收治获得性再生障碍性贫血(aplastic anemia,AA)患者中筛查HAAA患者资料.

  4. Clinical study on viral hepatitis combined with aplastic anemia%病毒性肝炎合并再生障碍性贫血的临床研究

    Institute of Scientific and Technical Information of China (English)

    路遥; 张艳丽; 申戈; 张璐; 王琳; 邱国华; 吴云忠; 杨民; 李明慧

    2011-01-01

    Objective To study the clinical features, outcomes and treatments of viral hepatitis combined with aplastic anemia. Methods25 cases diagnosed as viral hepatitis combined with aplastic anemia in Beijng Ditan Hospital between April 2004 and September 2009 were retrospectively analyzed. In this group of patients aplastic anemia was finally diagnosed by bone marrow aspiration. We collected clinical data of these patients, including a history of liver disease, drug allergies, hospital medication history, laboratory data, and then performed descriptive analysis. Results25 patients with viral hepatitis were diagnosed as complicated with aplastic anemia by histopathological data. Among these patients, 17 were male and 8 were women. Viral hepatitis included: chronic hepatitis B ( 12 cases), chronic hepatitis C (4 cases), acute hepatitis E ( 1 case), hepatitis caused by CMV infection ( 1 case), and unclassified hepatitis (7 cases). Among these patients, 7 were diagnosed as severe hepatitis. Considering previous history, only 3patients had history of short-term interferon therapy before hospitalization, and the remaining patients did not use drug that affects blood system. Treatments were as followings: using colony stimulating factor in 6patients, gamma globulin in 9 patients, glucocorticoids in 3 patients, erythropoietin in 1 patient, only oral drug to raise erythrocytes in 2 patients, red blood cells transfusion in 6 patients, platelets transfusion in 2patients. As for clinical outcomes, 20 patients acquired improved condition and were discharged, 3 patients were discharged voluntarily and 2 patients died of severe hepatitis combined with other complications.Conclusion Main treatments of viral hepatitis combined with aplastic anemia were to treat primary hepatopathy and nucleoside analogue-based antiviral therapy, to provide symptomatic and supportive treatment for blood diseases. Blood diseases would recover simultaneously while liver disease was improved, and the

  5. 氯吡格雷相关非重型再生障碍性贫血%Non-severe aplastic anemia associated with clopidogrel

    Institute of Scientific and Technical Information of China (English)

    李杰; 李扬

    2011-01-01

    A 66-year-old man with myocardial infarction was hospitalized and underwent percutaneous transluminal coronary angioplasty and stenting immediately. The day after surgery, he received clopidogrel 75 mg once daily, intial dose of aspirin 300 mg followed by 100 mg per day, fluvastatin 20 mg once daily, and fosinopril sodium 10 mg once daily. One month later, the paTient developed chills and high fever. Routine hlood tests revealed the following values : white blood cell count 1.1 × 109/L with neutrophils 0. 034, absolute value of neukophils 0. 037 × 109/L, red blood cell count 3.6 × 1012/L, hemoglobin 113 g/L, and platelet count 119 ×109/L. Bone marrow aspiration and biopsy showed non-severe aplastic anemia. He received symptomatic treatment, his routineblood tests showed marked decreases in his complete blood count. On day 44 of hospitalization, routine blood tests revealed the following values: white blood cell count 2. 6 × 109/L with neutrophils 0. 367, absolute value of neutrophils 0. 954 × 109/L, red blood cell count 3. 6 × 1012/L, hemoglobin 113 g/L, and platelet count 84 × 109/L. Clopidogrel and aspirin were withdrawn immediately and switched to warfarin 2.5 mg once daily and cilostazol 100 mg twice daily,other medicines continued also. Subsequently, the routine blood tests were performed, and complete blood count gradually increased after reaching the lowest values. Bone marrow aspiration was repeated two times and results showed that bone marrow picture gradually retumed to normal. On day 102 of hospitalization, routine blood tests revealed the following values: white blood cell count 5. 8 × 109/L with neutrophils 0. 552, absolute value of neutrophils 3. 202 × 109/L, red blood cell count 4. 2 × 1012/L, hemoglobin 140 g/L, and platelet count 170 × 109/L. He was prescribed clopidogrel 50 mg once daily, aspirin 100 mg once daily. One week later, his routine blood tests showed marked decreases in complete blood caunt and , two weeks later, routine

  6. 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) Last Updated: February 2014 This ...

  7. Effect of treatment of perianal abscess in patients with severe aplastic anemia%重型再生障碍性贫血患者肛周脓肿治疗效果分析

    Institute of Scientific and Technical Information of China (English)

    杨舟; 杨琳; 肖朝晖; 刘文华; 郝敬全; 赵复锦; 江劲波; 王跃

    2015-01-01

    OBJECTIVE To enhance the clinicians'cognition of severe aplastic anemia complicated with perianal ab-scess so as to raise the cure rate .METHODS A retrospective survey was conducted for 1 case of severe aplastic ane-mia complicated with perianal abscess ,including the clinical data ,clinical manifestations ,B-ultrasound ,results of etiological culture ,and treatment process ,with the review of literature accompanied .RESULTS The Escherichia coli strains were cultured from this patient after the admission ,the symptom was improved and the scope of peri-anal abscess was reduced after the treatment with cefoperazone-tazobactam and etimicin ,the patient was automati-cally discharged after the treatment for 7 days and was readmitted due to the perianal pain ,the previous anti-infec-tion treatment was ineffective ,the perianal bacterial culture was carried out for the second time ,the Citrobacter freundii and Enterococcus faecium were isolated ,meropenem and teicoplanin were used for anti-infection treatment based on the results of the drug susceptibility testing ,accompanied by the hip bath of TCM ,elevation of white blood cells ,and blood transfusion ,and the perianal abscess was completely cured after the treatment for two weeks .CONCLUSION The reasonable use of antibiotics accompanied by the hip bath of TCM and active supportive treatment can raise the cure rate of the perianal abscess in the patient with severe aplastic anemia .%目的 加强临床医师对重型再生障碍性贫血合并肛周脓肿的认识 ,提高治愈率.方法 回顾性分析1例重型再生障碍性贫血合并肛周脓肿患者的临床资料、临床表现、B超、病原学培养结果及治疗经过 ,并结合文献复习.结果 该例患者入院后肛周细菌培养为大肠埃希菌 ,给予头孢哌酮/他唑巴坦和依替米星抗感染 ,患者症状好转、肛周脓肿范围缩小 ,治疗7d后自动出院 ;两周后患者因肛周疼痛再次入院 ,应用

  8. 妊娠合并再生障碍性贫血的分娩方式与临床监测%Childbirth way choice and clinical monitoring of merger aplastic anemia during pregnancy

    Institute of Scientific and Technical Information of China (English)

    李庆梅

    2015-01-01

    Objective To explore merger in patients with aplastic anemia during pregnancy childbirth way, to strengthen clinical monitoring, reduce complications.Methods For our hospital maternity merger of 32 cases of pregnancy with aplastic anemia patients childbirth way and clinical monitoring were analyzed retrospectively.Results 32casesofaplasticanemiapregnantmetaphaseinducedlabor14cases,18casesof pregnancy to late, the 13 cases of vaginal delivery, on average, postpartum blood loss was 360 ml; Cesarean delivery in 5 cases, average blood loss was 520 ml;Different childbirth way with postpartum haemorrhage amount had significant difference (P<0.05).18 cases of late pregnancy to maternal death in 1 case, neonatal death in 2 cases, the rest didn't have a case of a merger complications such as bleeding, infections, maternal and child were safe.Conclusions To strengthen perinatal health care during pregnancy and after birth to strengthen disease surveillance, and choose the appropriate childbirth way, effective treatment measures, is to ensure that aplastic anemia pregnant women mother baby safe and reduce the complications.%目的:探讨妊娠合并再生障碍性贫血患者的分娩方式,加强临床监测,减少并发症发生。方法对本院产科32例妊娠合并再生障碍性贫血患者的分娩方式及各阶段的临床监测进行了回顾性的分析。结果32例再障孕妇中期引产14例,18例妊娠至晚期,经阴道分娩13例,平均产后出血量为360 ml;剖宫产5例,平均出血量为520 ml;不同分娩方式与产后出血量有显著差异(P<0.05)。18例妊娠至晚期的产妇中死亡1例,新生儿死亡2例,余无1例合并大出血、感染等并发症,母婴均平安出院。结论孕期加强围产期保健,临产后加强病情监护,选择合适的分娩方式,采取有效的治疗措施,是保证再障孕妇母婴平安及减少并发症的关键。

  9. 辅助性T细胞与再生障碍性贫血的关系及研究展望%Relationship and Research Prospects of T Helper Cells and Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    沈英英; 林圣云

    2014-01-01

    Objective To review the pathogenesis and research prospects in AA.[Methods] In this paper, we summarize the imbalance mechanism of Th1/Th2, and the relationship of fol icular helper T cells(Tfh),Thl7, Th9 ,Th22 with aplastic anemia. [Results]The imbalance of Th1/Th2 cells leads to bone marrow failure. Immunosuppressive therapy can inhibit Th1 cell, restore the balance. The pathogenesis of Tfh, Thl7, Th9 and Th22 is closely correlated with AA. [Conclusion] AA pathogenesis is complex, CD4+cellsubsets is related to the occurrence and development of AA. Detect the levels of immune cells in the serum of patients is beneficial for diagnosis and treatment of AA.%[目的]综述了Th细胞各亚群与再生障碍性贫血(aplastic anemia, AA)相关的发病机制,并针对其近来的研究进行展望。[方法]查阅近10年国内相关文献资料并总结Th1/Th2失衡机制、滤泡辅助性T细胞(Tfh),Thl7,Th9和Th22与AA的关系。[结果]Th1/Th2细胞比例失衡,可引起骨髓造血功能衰竭。免疫抑制治疗可抑制Th1细胞,恢复Th1/Th2比例平衡,从而使造血功能恢复。Tfh、Thl7、Th9和Th22与AA的发病机制密切相关。[结论]AA的免疫发病机制复杂,CD4+的细胞亚群及其多种相关的细胞因子与AA发生发展相关,全面检测AA患者血清中各种免疫细胞的比例变化和相关免疫功能分子的水平变化有利于AA的诊治。

  10. In patients with chronic aplastic anemia, bone marrow–derived MSCs regulate the Treg/Th17 balance by influencing the Notch/RBP-J/FOXP3/RORγt pathway

    Science.gov (United States)

    Li, Hongbo; Wang, Lin; Pang, Yan; Jiang, Zujun; Liu, Zenghui; Xiao, Haowen; Chen, Haijia; Ge, Xiaohu; Lan, Hai; Xiao, Yang

    2017-01-01

    The standard treatment for aplastic anemia (AA) in young patients is a matched sibling hematopoietic stem cell transplant. Transfusion of a chronic AA patient with allogeneic bone marrow–derived mesenchymal stromal cells (BMMSCs) is currently being developed as a cell-based therapy, and the safety and efficacy of such transfusions are being continuously improved. Nevertheless, the mechanisms by which BMMSCs exert their therapeutic effects remain to be elucidated. In this study, mesenchymal stromal cells (MSCs) obtained from bone marrow donors were concentrated and intravenously injected into 15 chronic AA patients who had been refractory to prior immunosuppressive therapy. We showed that BMMSCs modulate the levels of Th1, Th2, Th17 and Treg cells, as well as their related cytokines in chronic AA patients. Furthermore, the percentages of Th1 and Th17 cells among the H-MSCs decreased significantly, while the percentage Treg cells increased. The Notch/RBP-J/FOXP3/RORγt pathway was involved in modulating the Treg/Th17 balance after MSCs were transfused in vitro. Additionally, the role played by transfused MSCs in regulating the Treg/Th17 balance via the Notch/RBP-J/FOXP3/RORγt pathway was further confirmed in an AA mouse model. In summary, in humans with chronic AA, BMMSCs regulate the Treg/Th17 balance by affecting the Notch/RBP-J/FOXP3/RORγt pathway. PMID:28195151

  11. Effects of Sheng-Mai injection on the PRPP synthetase activity in BFU-es and CFU-es from bone marrows of mice with benzene-induced aplastic anemia.

    Science.gov (United States)

    Liu, L P; Liu, J F; Lu, Y Q

    2001-08-10

    160 Kunming mice were divided at random into 3 groups. Group 1: normal control (40 mice). Group 2: aplastic anemia (AA) control (60 mice); benzene inhalation was carried out for 2.5 months and sterilized normal saline was injected i.p. for another 6 weeks. Group 3: treated AA (60 mice); benzene was administered by inhalation in a similar manner, Sheng-Mai Injection (SMI) was administered i.p. for 6 weeks after the AA models were established. SMI is a famous Chinese traditional prescription of Panax ginseng C.A. Meyer (0.1 g/ml), Ophiopogon japonicus (Thunb.) Ker-Gawl (0.312 g/ml) and Fructus Schisandrae (0.158 g/ml). Activities of phosphoribosylpyrophosphate (PRPP) synthetase in BFU-Es and CFU-Es were estimated by ion pair reversed phase HPLC (IPrHPLC). Accompanying the sharp drop in counts of erythroid progenitor cells, the PRPP synthetase activity in CFU-Es of AA mice was reduced significantly (PCFU-Es returned nearly to normal levels following treatment with SMI of mice in Group 3 (PCFU-Es from their bone marrow. The impairment of PRPP formation would explain ATP depletion and disorders of energy metabolism in AA erythrocytes. SMI can distinctly increase the reduced quantity of erythroid progenitor cells and promote rapid restoration of PRPP synthetase activity in CFU-Es of AA mice.

  12. Retrospective study of alemtuzumab vs ATG-based conditioning without irradiation for unrelated and matched sibling donor transplants in acquired severe aplastic anemia: a study from the British Society for Blood and Marrow Transplantation.

    Science.gov (United States)

    Marsh, J C; Pearce, R M; Koh, M B C; Lim, Z; Pagliuca, A; Mufti, G J; Perry, J; Snowden, J A; Vora, A J; Wynn, R T; Russell, N; Gibson, B; Gilleece, M; Milligan, D; Veys, P; Samarasinghe, S; McMullin, M; Kirkland, K; Cook, G

    2014-01-01

    This retrospective national study compared the use of alemtuzumab-based conditioning regimens for hematopoietic SCT (HSCT) in acquired severe aplastic anemia with antithymocyte globulin (ATG)-based regimens. One hundred patients received alemtuzumab and 55 ATG-based regimens. A matched sibling donor (MSD) was used in 87 (56%), matched unrelated donor (MUD) in 60 (39%) and other related or mismatched unrelated donor (UD) in 8 (5%) patients. Engraftment failure occurred in 9% of the alemtuzumab group and 11% of the ATG group. Five-year OS was 90% for the alemtuzumab and 79% for the ATG groups, P=0.11. For UD HSCT, OS of patients was better when using alemtuzumab (88%) compared with ATG (57%), P=0.026, although smaller numbers of patients received ATG. Similar outcomes for MSD HSCT using alemtuzumab or ATG were seen (91% vs 85%, respectively, P=0.562). A lower risk of chronic GVHD (cGVHD) was observed in the alemtuzumab group (11% vs 26%, P=0.031). On multivariate analysis, use of BM as stem cell source was associated with better OS and EFS, and less acute and cGVHD; young age was associated with better EFS and lower risk of graft failure. This large study confirms successful avoidance of irradiation in the conditioning regimens for MUD HSCT patients.

  13. 获得性再生障碍性贫血发病机制研究最新进展%Recent progeress of pathogenesis in acquired aplastic anemia He Wei,Xie Xiaotian

    Institute of Scientific and Technical Information of China (English)

    何薇; 谢晓恬

    2016-01-01

    再生障碍性贫血(再障)是儿童期严重的血液病,尤其是重型再障治疗难度大,病死率高。因为95%左右的再障病例属于获得性再障,所以深入研究获得性再障的发病机制,对于临床诊断和提高疗效均具有重大意义。现对近5年关于儿童获得性再障发病机制的国际最新进展进行综述。%Aplastic anemia (AA)is a serious disease of hematological malignant disease in children.Severe AA is difficult to treat and may lead to high motality.Acquired AA accounts for 95% of all cases,so it′s significant to lucubrate the pathogenesis of acquired AA for clinic diagnosis and therapy.The documents in recent 5 years are collected,and a reviews about the progress of pathogenesis in acquired AA children is provided.

  14. Mouse models of Fanconi anemia

    OpenAIRE

    Parmar, Kalindi; D'Andrea, Alan; Niedernhofer, Laura J.

    2009-01-01

    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 ...

  15. 再生障碍性贫血患者PIG-A基因外显子2、4、5突变及粒细胞CD 55、CD 59的表达%Mutations of PIG-A gene Exons 2,4,5 and Granulocytic Expressions of CD55 and CD59 in Patients with Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    李玉云; 昝丽娜; 王卫国

    2009-01-01

    背景与目的:探讨再生障碍性贫血(aplastic anemia,AA)患者PIG-A基因外显子2、4、5的突变及粒细胞表面CD55、CD59锚蛋白表达的情况. 材料与方法:从30例AA患者及20例正常对照组人群外周血提取基因组DNA,采用PCR扩增PIC-A基因外显子2、4、5,再将纯化的PCR产物双向测序检测基因序列;并用流式细胞术检测两组人群外周血粒细胞中CD55、CD59的表达. 结果:30例从患者中11例发现PIG-A基因外显子2突变,包括碱基替代、缺失、插入;PIG-A基因外显子4和外显子5突变各5例,仅为碱基替代;共5例患者有2个或2个以上外显子存在突变,正常对照组未发现突变.粒细胞CD55和CD59的表达率在AA患者PIG-A基因突变者中分别为(86.57 4±5.90)%、(88.174±5.90)%,在PIG-A基因未突变者中分别为(91.874±4.79)%、(94.24±3.76)%.均较正常对照组(97.864±1.52)%、(98.824±1.42)%显著降低(P均<0.05).结论:再生障碍性贫血患者存在PIG-A基因突变和粒细胞CD55、CD59表达缺失的现象,提示再障患者可能存在造血的克隆源性异常.%BACKGROUND AND AIM: To explore mutations of PIG-A gene exon2, exon4, exon5 and expression of CD55 and CD59 in granulocytes of patients with aplastic anemia. MATERIALS AND METHODS: Genomic DNA from peripheral blood of 30 aplastic anemia patients and 20 normal controls were extracted, and PIG-A gene exon2, exon4, exon5 were then examined with polymerase chain reaction (PCR) , nucleotide sequences were analyzed by bidirectional sequencing after PCR products were purified. The expressions of CD55 and CD59 in granulocytes from peripheral blood of the two groups above were detected by flow cytometry. RESULTS: The mutations of PIG-A gene exon2 including base substitution, deletion, insertion occurred in 11 of 30 aplastic anemia patients , the mutations of exon4, exon5 were also found in five aplastic anemia patients, with only base substitution. Two or more exon mutations were found in 5

  16. 儿童肝炎相关再生障碍性贫血3例临床分析%Clinical analysis of childhood hepatitis associated aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    邵越霞; 谢晓恬; 石苇; 周晓迅

    2012-01-01

    目的 探讨儿童肝炎相关再生障碍性贫血(HAAA)的病原学、临床特点及免疫抑制治疗(IST)疗效.方法 归纳与分析2007年1月-2011年12月收治的HAAA患儿的临床资料,包括临床表现,再障相关血液与免疫学指标(外周血象、骨髓涂片与活检、T细胞亚群),肝炎相关检测(肝功能、病毒血清学、自身免疫肝炎抗体)等,以及治疗与疗效结果.结果 3例HAAA,占同期获得性再障患儿4.2%.其中1例经血清学与病毒DNA检测确诊为乙型肝炎,经抗病毒和保肝治疗后,接受抗胸腺细胞免疫球蛋白(ATG)和环孢菌素A(CSA)治疗获得痊愈.另2例检测各类血清型肝炎病毒,以及巨细胞病毒(CMV),EB病毒和单纯疱疹病毒血清学结果均呈阴性,但肝炎自身抗体抗核抗体(ANA)为1:100,血免疫球蛋白升高,符合自身免疫性肝炎后HAAA.其中1例经CSA治疗后好转,脱离成分输血依赖,获得明显进步;另1例死于重症感染和颅内出血.结论 儿童HAAA可发生于自身免疫性肝炎或血清型肝炎,起病急,病情重.因发病机制仍属于T细胞免疫介导,故IST有效.%Objective To study the pathogenesis, clinical characteristics and the therapeutic effects of immunosuppression therapy on childhood hepatitis associated aplastic anemia (HAAA). Methods Clinical data of children with HAAA from January of 2007 to December of 2011 were collected and analyzed, including clinical manifestations, aplastic anemia ( AA) related hematological and immunological parameters (peripheral blood smear, bone marrow smear and biopsy, T-cell subpopulation) , hepatitis related tests ( liver functions, viral serology, autoimmune hepatitis antibodies) , treatment regimens and the therapeutic effect as well. Results Three cases of HAAA, account for4. 2% of all acquired AA patients, were collected. Among them, one case was diagnosed as type B hepatitis after serological and viral DNA assays. This patient received anti-thymocyte globulin and

  17. Mixed T Cell Chimerism After Allogeneic Hematopoietic Stem Cell Transplantation for Severe Aplastic Anemia Using an Alemtuzumab-Containing Regimen Is Shaped by Persistence of Recipient CD8 T Cells.

    Science.gov (United States)

    Grimaldi, Francesco; Potter, Victoria; Perez-Abellan, Pilar; Veluchamy, John P; Atif, Muhammad; Grain, Rosemary; Sen, Monica; Best, Steven; Lea, Nicholas; Rice, Carmel; Pagliuca, Antonio; Mufti, Ghulam J; Marsh, Judith C W; Barber, Linda D

    2017-02-01

    Prevention of graft-versus-host disease (GVHD) is paramount for allogeneic hematopoietic stem cell transplantation (HSCT) to treat nonmalignant diseases. We previously reported that allogeneic HSCT for severe aplastic anemia (SAA) using the fludarabine, cyclophosphamide, and alemtuzumab (Campath-1H) (FCC) regimen is associated with a very low risk of GVHD and excellent clinical outcomes. We now report a single-center study of 45 patients with longer follow-up and investigation of lymphocyte recovery. Overall survival (OS) was 93%, and event-free survival (EFS) was 90.7%. Acute and chronic GVHD each occurred in 6 patients (13.3%), and only 1 case was severe. Mixed T cell chimerism was frequent and persisted after cessation of immunosuppression. T cells were extensively depleted, representing only 11.3% of lymphocytes at day 30 and rising to 43.8% by 1 year, but still significantly below normal levels (67.2%; P = .018), and deficiency persisted after immunosuppressive therapy (IST) withdrawal. Depletion of CD4 T cells was particularly profound, causing inversion of the normal CD4:CD8 T cell ratio. T cell subset composition was also abnormal, with memory and effector T cells predominating for at least 6 months after FCC HSCT. Analysis of T cell subset chimerism showed that CD4 T cells were predominantly donor-derived at 1 year, whereas recipient-derived CD8 T cells shaped mixed chimerism with a notable contribution of recipient effector CD8 T cells. The prolonged mixed T cell chimerism after IST withdrawal and low incidence of GVHD indicates the establishment of mutual tolerance, but the low incidence of viral disease suggests maintenance of antiviral immunity. Our study shows that despite the abnormal T cell profile after allogeneic HSCT for SAA using the FCC regimen, this regimen is conducive to an excellent clinical outcome.

  18. Development of a disease-specific quality of life questionnaire for patients with aplastic anemia and/or paroxysmal nocturnal hemoglobinuria (QLQ-AA/PNH)-report on phases I and II.

    Science.gov (United States)

    Groth, Martha; Singer, Susanne; Niedeggen, Cathrin; Petermann-Meyer, Andrea; Röth, Alexander; Schrezenmeier, Hubert; Höchsmann, Britta; Brümmendorf, Tim H; Panse, Jens

    2017-02-01

    Acquired aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) are interrelated ultra-rare diseases. Quality of life (QoL) evaluation tools used in studies for AA and PNH are unspecific and designed for cancer patients (e.g., the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire, EORTC QLQ-C30). Given the complexity of AA and PNH, variation in symptoms and treatments, younger age of many patients, and the fact that AA and PNH are not classified as malignant diseases, it is likely that cancer-specific questionnaires are inappropriate. We generate an AA/PNH-specific QoL questionnaire (QLQ-AA/PNH), performed according to EORTC guidelines. QoL issues were obtained from the literature and interviews with patients and physicians (phase I), then ranked by patients and physicians. In phase II, items were created. Patients in more than 25 German and Swiss cities were interviewed face to face. In phase I, interviews of 19 patients and 8 physicians specialized in AA/PNH treatment resulted in 649 QoL issues; these were condensed to 175 and graded according to their importance by 30 patients and 14 physicians (phase II). Five physicians took part in phases I and II. Altogether, 97 issues were rated important. Twelve EORTC QLQ-C30 items were not rated important, while several new QoL aspects were brought up. Modifications in wording and phrasing led to two questionnaires with 77 items regarding general QoL aspects and 20 items regarding medical care. Important QoL aspects of PNH/AA patients are inappropriately captured with available QoL tools. Developing a new QoL questionnaire specific for this patient group is warranted.

  19. Clinical research of ATG + CsA regimen in the treatment of severe aplastic anemia%ATG加CsA方案治疗重型再生障碍性贫血临床研究

    Institute of Scientific and Technical Information of China (English)

    梁欣荃; 符丽梅; 祝平安; 禹环; 谷敏; 黄斌; 廖欣

    2011-01-01

    目的:探讨ATG加CsA方案治疗重型再生障碍性贫血(再障)的疗效、疗程和治疗相关并发症.方法:对56例重型再障给予ATG加CsA方案治疗,每月至少观察一次血常规、肝、肾功能等项目.结果:56例中完全反应45例(80%),部分反应5例(9%),无效6例(11%).复发10例(20%).结论:ATG加CsA治疗重型再障疗效好、并发症可防可治,主要不足是疗程长.%Objective: To investigate therapeutic effectiveness, courses and complications of the ATG + CsA regimen in the treatment of severe aplastic anemia (SAA) patients. Method: Fifty-six patients with SAA received ATG + CsA treatment. Every patient received the examinations of blood routine, liver function and kidney function and other items at least once a month. Result: Of 56 patients, complete response was received in 45 patients (80%), partial response in 5 cases (9%), no response in 6 cases (11%) and relapse in 10 cases (20%). Conclusion: ATG + CsA regimen has good effect on the treatment of SAA. Its complication was preventable and curable. The main deficiency of this regimen is long course.

  20. Varied clinical course of aplastic crisis in hereditary spherocytosis.

    Science.gov (United States)

    Kataoka, Akihiro; Doi, Shoichi; Suemori, Shinichiro; Nakanishi, Hidekazu; Jonen, Daisuke; Mori, Mioko; Mizushima, Yasuhiro; Wakazono, Yoshihiro

    2014-02-01

    This study is the first to report a familial case involving differing clinical courses of aplastic crisis triggered by parvovirus B19 in two patients with HS, although similar eosin-5-maleimide-binding test and sodium dodecylsulfate-polyacrylamide gel electrophoresis results had been obtained for both. One patient had short-term mild symptoms, whereas the other patient developed severe anemia that required blood transfusion, experienced fever for 13 days, and did not have any rash. The severity of aplastic crisis is reported to be correlated with the severity of the underlying hemolytic anemia; the present findings show that the severity of infection should also be considered as an important predictive factor of the severity of aplastic crisis.

  1. 重型再生障碍性贫血抗胸腺球蛋白治疗后伴垂体功能减退治疗研究%Case Report and Review: Hypopituitarism after the ATG Treatment for the Patient of Severe Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    陈智; 戴铁颖; 林圣云

    2013-01-01

    OBJECTIVE To find out the cause of hypopituitarism induced by ATG in the process of intensive immunosuppressive therapy (IST) for severe aplastic anemia,and to prevent related complications.METHODS A case of hypopituitarism induced by ATG was chosen,which clinical physician participated in the whole treatment and literatures were reviewed.RESULTS ATG can induce hypopituitarism,which should bring enough attentions in clinical.CONCLUSION In the clinical diagnosis and treatment of severe aplastic anemia,we should pay more attention to the potential complications,so as to reduce the risk of treatment,improve the outcome of treatment.%目的 了解在使用ATG行强化免疫抑制治疗重型再障的过程中引起垂体功能减退的原因及相关并发症的防治.方法 临床医师总结1例应用ATG引起垂体功能减退的诊治过程及对国内外文献进行查阅.结果 ATG可以引起垂体功能减退,应引起临床注意.结论 在临床诊治重型再生障碍性贫血时,要特别注意潜在的并发症,从而降低治疗风险,提高疗效.

  2. 68例儿童重型再生障碍性贫血强化免疫抑制治疗的疗效分析%Clinical Analysis of the Efficacy of Intensive Immunosuppressive Therapy in 68 Children With Severe Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    陈纯; 李迎飞; 方建培; 薛红漫; 周敦华; 黄科; 徐宏贵; 郭海霞; 黄绍良

    2009-01-01

    IST亚组及其无反应IST亚组患儿治疗6个月后,有效率分别为76.9%(10/13)和45.6%(5/11),两亚组比较,差异有显著意义(P<0 05).G-CSF反应IST亚组患儿总有效率(92.3%,12 /13)较无反应IST亚组(54.5%,6/11)高,且差异有显著意义(P<0.05).结论 强化免疫抑制治疗重型再生障碍性贫血的疗效明显优于单用环孢菌素A. 强化免疫抑制治疗前,对粒细胞集落刺激因子有反应的患儿,强化免疫抑制治疗的预后相对较好.%children with severe aplastic anemia is better than that of cyclosporine A therapy only. Children with severe aplastic anemia who responded well to granulocyte colony stimulating factor had a better prognosis in the intensive immunosuppressive therapy.

  3. Aplastic anaemia: a review

    NARCIS (Netherlands)

    S. Sleijfer (Stefan); P.J. Lugtenburg (Pieternella)

    2003-01-01

    textabstractAplastic anaemia is featured by bone marrow hypocellularity and peripheral pancytopenia and is a potentially fatal disease. In recent years, insight in it pathogenesis has increased. It appears that activated autoreactive T lymphocytes induce apoptosis of haematopoietic

  4. Relationship between antigen presenting cells and pathogenesis of aplastic anemia%抗原提呈细胞与再生障碍性贫血发病机制的关系

    Institute of Scientific and Technical Information of China (English)

    李仲龙

    2015-01-01

    Aplastic anemia(AA) is defined as an immune-mediated bone marrow failure syndrome,characterized by pancytopenia and hypocellular bone marrow.At present,the pathogenesis of AA is concerned chiefly with abnormal hematopoietic stem cells,hematopoietic microenvironment abnormality and disorder of immune system.Antigen presenting cell (APC) is the main initiator of adaptive immunity.It plays a role in processing and presenting antigens to T lymphocyte,and leading to an immune response or immune tolerance.Activation of APC may lead to the imbalance and dysfunction of T lymphocyte subsets,such as the polarization of helper T lymphocyte (Th)1,activation of CD8+T cells and secretion of negative regulators of hematopoiesis.All of these compose a cytokine network to destruct stem/progenitor cells as well as hematopoietic stem cells,mesenchymal stem cells and angioblasts/endothelial progenitor cells.Thus,this article reviews literatures on the relationship between APC including dendritic cells (DC),monocytes,B lymphocytes and the pathogenesis of AA.%再生障碍性贫血(AA)为以全血细胞数量减少,骨髓细胞数量减少及骨髓造血功能降低或衰竭为特征的疾病.目前,AA的发病机制主要涉及造血干细胞异常、造血微环境异常及免疫系统异常.抗原提呈细胞(APC)则是适应性免疫的主要启动者,具有摄取、处理抗原并将其提呈给T淋巴细胞,并引起免疫应答或耐受的功能.APC的激活可能会引起T淋巴细胞亚群比例失衡及功能异常,如辅助性T淋巴细胞(Th)1细胞极化、CD8+T淋巴细胞激活、造血负性调控因子分泌,进一步破坏造血干细胞、间充质干细胞、血管干/祖细胞,最终导致骨髓造血功能衰竭.笔者拟就近年来,树突状细胞(DC)、单核细胞、部分B淋巴细胞等APC与AA发病机制的关系进行综述.

  5. Análise da rejeição nos pacientes transplantados por anemia aplástica severa condicionados com ciclofosfamida ou a associação desta ao bussulfano Analysis of rejection in transplanted patients suffering severe aplastic anemia conditioned with cyclophosphamide alone or associated with busulfan

    Directory of Open Access Journals (Sweden)

    Lídice C. Lenz e Silva

    2005-03-01

    Full Text Available O transplante de medula óssea é um tratamento eficaz para pacientes com anemia aplástica severa (AAS e é a modalidade terapêutica de escolha para pacientes jovens com doador aparentado HLA idêntico. A rejeição é uma importante complicação do transplante de medula, que, independentemente do tipo de tratamento imunossupressor pré e pós-transplante, ocorre em 55% a 60% dos pacientes. O serviço de TMO da Universidade Federal do Paraná (UFPR acumula a experiência de 178 casos de AAS transplantados no período de 1993 a 2001, usando como condicionamento tanto a ciclofosfamida (CFA como a combinação desta ao bussulfano (CFA + BU. Dentre eles, 39 apresentaram rejeição ou falha de pega. Dos pacientes condicionados com ciclofosfamida, 24 (46% apresentaram rejeição, sendo 3 (6% com falha primária de pega (FPP e 21 (40% com pega transitória (PT. Entre os pacientes condicionados com BU+CFA, 15 (12% apresentaram rejeição, sendo 4 (3% com FPP e 11 (9% com pega transitória. Os pacientes condicionados com ciclofosfamida (200 mg/kg que apresentaram rejeição tiveram uma sobrevida global alta (aproximadamente 80%, pois conseguiram ser resgatados por um novo transplante ou pelo tratamento imunossupressor com ciclosporina. A sobrevida dos pacientes politransfundidos condicionados com a associação de ciclofosfamida e bussulfano foi de aproximadamente 35%.Bone marrow transplantation is an effective therapy for severe aplastic anemia and is generally considered the preferable treatment for young patients who have an HLA (Human Leukocyte Antigen identical sibling donor. Recent studies report 55% to 80% extended survival. Graft failure owing to rejection or others causes remains an important life-threatening complication following allogeneic bone marrow transplantation for aplastic anemia. It occurs in 55% to 60% of patients receiving HLA identical transplants, using different immunosuppressive therapies before and after transplant. The BMT

  6. 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.

  7. 异基因BMSC对重型再障患者T细胞免疫功能影响的研究%Effects of allogeneic bone marrow mesenchymal stem cells on T lymphocytes in patients with severe aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    余卫; 李萡; 罗英; 谭雪芳; 陈玲珍

    2014-01-01

    目的:探索异基因骨髓间充质干细胞( allo-BMSC)对重型再生障碍性贫血( SAA)患者T细胞免疫功能的影响。方法:将allo-BMSC与SAA患者( n=15)外周血T细胞按不同比例( allo-BMSC:T cell分别为0∶1,1∶100,1∶50,1∶25)及进行共培养。72 h后,检测T cell数及allo-BMSC:T cell分别为0∶1(对照组)和1∶25(allo-BMSC组)上清中IL-10、IFN-γ、TNFα的含量。结果:(1).不同比例allo-BMSC(0∶1,1∶100,1∶50,1∶25)对SAA患者T淋巴细胞增殖抑制率分别为3%±2%,51%±17%,64%±21%,73%±18%,抑制作用与allo-BMSC的数量呈正相关(r=0.684,P<0.001);(2).allo-BMSC组IL-10浓度显著增加[(300±175)ng/mL vs (25±14)pg/mL,P<0.05],allo-BMSC组IFN-γ浓度显著降低[(2.1±1.5)ng/mL vs(3.5±1.9) ng/mL,P<0.05];allo-BMSC组TNFα浓度显著降低[(8±5)pg/mL vs (17±11)pg/mL,P<0.05]。结论:allo-BMSC可以抑制SAA患者T细胞增殖,并减低Th1细胞炎性因子的表达,而增加Th2细胞因子的表达。%Objective:To explore the effects of allogeneic bone marrow mesenchymal stem cells ( allo-BMSCs) on T lymphocytes in patients with severe aplastic anemia. Methods: The allo-BMSCs were cocultured with T cells derived from 15 patients with severe aplastic anemia ( allo-BMSC/T cell ratio, 0∶1, 1∶100, 1∶50 and 1∶25 ) . T cells count and cytokine levels ( IL-10, IFN-γ and TNF-α) in culture supernatants were determined in control group ( allo-BMSC/T cell ratio, 0:1) and allo-BMSC group ( allo-BMSC/T cell ratio, 0:25) after 72 hours of cultivation. Results: The allo-BMSCs of different ratios ( 0∶1, 1∶100, 1∶50, 1∶25) resulted in the suppression rate of proliferation of 3%± 2%, 51%± 17%,64%± 21% and 73%± 18%,respectively,suggesting a positive correlation between the inhibitory effects and allo-BMSC count ( r=0.684,P<0.001). The allo-BMSCs group was associated with markedly higher levels of IL-10 [(300±175)ng/mL vs (25

  8. Efficacy of Allogeneic Hematopoietic Stem Cell Transplantation in Treatment of Severe Aplastic Anemia%异基因造血干细胞移植治疗重型再生障碍性贫血的疗效研究

    Institute of Scientific and Technical Information of China (English)

    朱玲; 薛梅; 王志东; 闫洪敏; 刘静; 丁丽; 王恒湘

    2011-01-01

    Objective To explore the effectiveness of Allogeneic hematopoietic stem cell transplantation ( allo -HSCT ) in treating severe aplastic anemia ( SAA ).Methods Among the 8 patients, 1 patient received allogeneic peripheral stem cell transplantation from an HLA matched sibling, 4 patients received allogeneic bone marrow and peripheral stem cell transplantation from haploidentical donors ( parents ), 3 patients received unrelated allogeneic peripheral stem cell transplantation.Conditioning regimens included: fludarabine, Cyeclophosphamide, anti - themocyte globulin ( for unrelated and HLA matched sibling donors ); fludarabine, Cyeclophosphamide, busulphan and anti - lymphocyte globulin ( ALG ) /anti - themocyte globulin ( ATG ) ( for haploidentical donors ).For prevention of graft versus host disease ( GVHD ) the patient with HLA matched sibling donor was administered with a combination of immunosuppressive drugs including CSA, short - course MTX while for the patients with haploidentical or unrelated donors, MMF, anti - CD25 monoclonal antibody and ATG were, also employed.Results All the 8 patients achieved hematopoietic reconstitution after transplantation.It took 10 ~ 17 days ( : median: 12.5 days ) for the level of neutrophils to reach 0.5 × 10 /L and 9-25 days ( median: 13.8 days ) for platelets to reach 20 × 10 /L.All the 8 patients became donor chimerism.As for the complications, CMV - related sepsis was found in 5 cases, hemorrhagic cystitis in 3 cases, Grade Ⅰ ~ Ⅲ graf - versus - host disease ( GVHD ) and chronic local GVHD in 2 patients, and central nervous system infection accompanied with pure red aplastic anemia in 1 case.All the patients survived during the follow - up ( range: 9 ~ 38 months; median: 20 months).Conclusion Allo - HSCT is an effective approach for treating patients with SAA.It may be helpful to prolong the survivals of these patients.%目的 探讨异基因造血干细胞移植(allo-HSCT)治疗重型再生障

  9. Study on the therapeutic effects of 2SZAPXRO on aplastic anemia in a mouse model induced by busulfan%2SZA PXRO治疗小鼠经白消安诱导的再生障碍性贫血模型的效果评价

    Institute of Scientific and Technical Information of China (English)

    易翠林; 郝苗; 韩丽丽; 王玉林

    2015-01-01

    2SZAPXRO is an elaborate extraction of sea cucumber ,oyster and Chinese wolfberry by the technique of “BalanCell” ,with various enriched biologically active components ,including mucopoly-saccharides ,polypeptide ,amino acid ,taurine and diverse trace elements and vitamins .From the view of TCM theory ,2SZAPXRO should be of kidney tonifying and blood replenishing . In this study ,aplastic anemia was established in mice by administration of busulfan for the in vivo effect e-valuation of 2SZAPXRO on aplastic anemia ,which was administered to mice orally .Meanwhile , normal control group and non-therapeutic group were set .The results indicated that 2SZAPXRO ex-hibits excellent in v ivo effects on aplastic anemia in a mouse model .%2SZAPXRO是一种以海参、牡蛎及枸杞为原料,应用细胞平衡液(BalanCell)技术制备的复合提取液,富含海参黏多糖、海参皂苷、多肽、氨基酸、牛磺酸、多种微量元素和维生素等多种生物活性成分。从中医学角度,该提取液具有补肾益精、滋阴养血的功效。用白消安诱导小鼠,建立再生障碍性贫血动物模型。对再生障碍性贫血小鼠进行口服2SZAPXRO给药治疗,同时设立正常小鼠对照组(未经白消安造模)和无药物治疗组(对白消安造模小鼠不进行2SZAPXRO给药治疗)。结果表明:2SZAPXRO复合提取液对小鼠再生障碍性贫血具有显著的治疗效果。

  10. Expression of T-bet/GATA-3 mRNA in peripheral blood in patients with aplastic anemia%转录因子T-bet和GATA-3对再生障碍性贫血免疫失衡的作用

    Institute of Scientific and Technical Information of China (English)

    杨晓红; 唐旭东; 许勇钢; 张姗姗; 麻柔; 刘锋

    2010-01-01

    目的 探讨外周血中 T-bet和GATA-3基因的表达在再生障碍性贫血(aplastic anemia,AA)中发病机制及意义.方法 选择23例AA患者为再障组,其中重型再障(severe aplastic anemia,SAA)13例,轻型再障(mediate aplastic anemia,mAA)10例,再选择22例健康体检为对照组,对纳入对象采用流式细胞术检测其外周血Th1和Th2,同时运用RT-PCR技术检测外周血单个核细胞(PBMCs)中转录因子T-bet和GATA-3 mRNA基因表达.结果 与对照组相比,再障组的lg(T-bet/β-actin)和Th1(%)升高,差异有统计学意义(P<0.05、P<0.01),lg(GATA-3/β-actin)和Th2(%)降低,差异有统计学意义(P<0.05、P<0.05);其中SAA组的lg(T-bet/β-actin)、lg(GATA-3/β-actin)和Th1(%)、mAA组的lg(T-bet/β-actin)、Th1(%)相比对照组明显升高(P<0.05、P<0.01、P<0.01、P<0.05、P<0.05);SAA组的Th2(%)、mAA组的lg(GATA-3/β-actin)和Th2(%)相比对照组显著降低(P<0.05、P<0.01、P<0.05).结论 T-bet和GATA-3表达异常在AA发生、发展过程中发挥重要作用,可能机制为增强Th1细胞功能,抑制Th2细胞功能,导致患者免疫功能异常,最终引起再障发生.

  11. Hemoglobinopatia H adquirida em um paciente com anemia aplástica e evolução para leucemia mielóide aguda

    OpenAIRE

    2004-01-01

    CONTEXT: The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia. CASE REPORT: We report the first case of a Brazilian patient with aplastic anemia who developed myelodys...

  12. 慢性再生障碍性贫血患者CD28共刺激分子及Fas的表达%Expressions of co-stimulatory molecule CD28 and Fas in chronic aplastic anemia patients

    Institute of Scientific and Technical Information of China (English)

    刘敏; 王运律; 胡琦; 孙思明; 刘元霞; 张晓峰

    2008-01-01

    再生障碍性贫血(aplastic anemia,AA)简称再障,是免疫介导的造血抑制的综合病症,其中又以慢性再生障碍性贫血(chronic aplastic anemia,CAA)患者多见。本文旨在观测CAA患者外周血共刺激分子CD28及Fas(CD95)的表达水平。

  13. Association of Mitochondrial DNA Mutation with Telomere Length in Patients with Aplastic Anemia%再生障碍性贫血线粒体突变与端粒长度相关性的研究

    Institute of Scientific and Technical Information of China (English)

    曹慧琴; 韦玮; 崔兴

    2016-01-01

    Objective To examine the association of mitochondrial DNA ( mtDNA) mutations with telomere length in patients with aplastic anemia ( AA) . Methods Bone marrow and oral epi-thelial samples were collected from 45 patients diagnosed as having AA between 2010-2014 for de-tection of mtDNA mutation and telomere length. Complete sequence analysis of mtDNA revealed 151 mutations in 18 genes, including 40 silent mutations and 28 frameshift mutations. Moreover, the relative length of telomere ( relative T/S value) was detected in AA patients and healthy volunteers, with HBG as internal control gene. Results Non-silent mutation of mtDNA was negatively correla-ted with the WBC count, hemoglobin level and platelet count. Relative T/S value was positively correlated with the WBC count, hemoglobin level and platelet count, and a negative correlation was found between the non-silent mutation of mtDNA and relative T/S value. Conclusion Mutation-caused functional impairment of the mitochondrial respiratory chain and telomere length shortening may play important roles in the process of hematopoietic failure in AA patients. Additionally, mtD-NA mutations and telomere length shortening influence each other.%目的:研究再生障碍性贫血患者线粒体突变及端粒长度情况,了解两者间的相关性。方法选择2010~2014年确诊为再生障碍性贫血的患者45例,留取骨髓及口腔黏膜上皮标本以进行线粒体DNA ( mitochondrial DNA, mtDNA)突变和端粒长度的检测。线粒体全测序检测到了151个突变,分布在18个基因中,其中包括了40个沉默突变及28个框移突变。同时使用HBG作为内参基因,检测了再障患者和健康志愿者端粒的相对长度( relative T/S value,端粒长度)。结果分析发现非沉默突变的mtDNA突变与白细胞数、血红蛋白水平及血小板数成负相关。端粒长度与白细胞数、血红蛋白水平及血小板数成正相关性,而且非沉默突变的mtDNA突变与端粒长

  14. Effectiveness and Safety of Astragalus Injection for Aplastic Anemia:A Systematic Review%黄芪注射液治疗再生障碍性贫血疗效和安全性的系统评价

    Institute of Scientific and Technical Information of China (English)

    晁荣; 席亚明; 靳蕊蕊

    2011-01-01

    目的 系统评价黄芪注射液+雄激素与雄激素单用比较治疗再生障碍性贫血(再障)的疗效和安全性.方法 计算机检索Cochrane Library(2011年第3期)、PubMed (1966 ~ 2011.3)、EMbase (1974 ~ 2011.3)、CNKI (1994 ~ 2011.3)、VIP (1989~2011.3)和万方(1997 ~ 2011.3)数据库等,按照纳入排除标准选择文献、提取资料和进行质量评价后,采用RevMan 5.0.24软件进行Meta分析.结果 共纳入7个随机对照试验,合计518例患者.Meta分析结果显示:黄芪注射液+雄激素治疗组的总有效率优于单用雄激素组,其差异有统计学意义[OR=3.12,95%CI (2.09,4.66),P<0.000 01];治疗组与对照组的骨髓增生升级程度相当,其差异无统计学意义[OR= 1.93,95%CI (0.85,4.38),P=0.11];治疗组的不良反应明显少于对照组,其差异亦有统计学意义[OR=0.30,95%CI (0.12,0.76),P=0.01].结论 黄芪注射液+雄激素治疗再障的有效率高于单用雄激素,且不良反应少.但由于纳入研究样本量小且质量较低,上述结论尚需更多高质量的研究来加以证实.%Objective To assess the clinical effectiveness and safety of astragalus injection plus androgen versus androgen alone for patients with aplastic anemia (AA). Methods Such databases as The Cochrane Library (Issue 3, 2011), PubMed (1966 to March 2011), Embase (1974 to March 2011), CNKI (1994 to March 2011), VIP (1989 to March 2011) and Wanfang Data (1997 to March 2011) were searched to include the randomized controlled trails (RCTs) according to the inclusive and exclusive criteria. The data were extracted, the quality was assessed, and meta-analysis was conducted by using Revman5.0.24 software. Results Seven RCTs involving 518 patients with AA were included. The meta-analysis showed that the astragalus plus androgen treatment group was superior to the androgen alone group in the total effective rate with significant difference (OR=3.12, 95%CI 2.09 to 4.66, P<0.000 01); the adverse events

  15. Comparison of two types of antithymocyte globulin in the treatment of children with aplastic anemia%抗人体T淋巴细胞球蛋白和兔抗人胸腺细胞免疫球蛋白治疗儿童再生障碍性贫血的效应对比研究

    Institute of Scientific and Technical Information of China (English)

    谢晓恬; 何薇; 石苇; 周晓迅; 乔晓红

    2016-01-01

    Objective This study was designed to compare the effects of the anti-human T lymphocyte globulin (Fresenius,ATG-F)and rabbit anti-human thymocyte immunoglobulin (Genzyme,R-ATG) in the treatment of childhood aplastic anemia (AA) and their effects.Method A total of 59 children with aplastic anemia were analyzed in the present study,including 34 cases of severe aplastic anemia (SAA),12 cases of very severe aplastic anemia (VSAA) and 13 cases of transfusion-dependent non-severe aplastic anemia (NSAA).While receiving immunosuppressive therapy (IST),30 and 29 patients,with long-term oral supplement with cyclosporin A (CSA),androgen and Chinese traditional medicines,were treated with ATG-F and R-ATG,respectively.When it was necessary,some supportive cares such as component transfusion and infection control were also employed.Absolute counts of peripheral blood lymphocyte (ALC) at various time points were dynamically detected after ATG therapy.Result According to the International Aplastic Anemia Treatment and Effect standards.There were no statistically significant differences in the overall response rate (67% (20/30) vs.69% (20/29),x2 =0.036,P =0.676) and the survival rate (87% (26/30)vs.83% (24/29),x2 =0.173,P =0.676) between the ATG-F and R-ATG groups.There were significant and long-term ALC decrease after ATG therapy,the rate of ALC decrease in ATG-F and R-ATG group,the ALC only recovered to 47.8% (ATG-F group) and 47.4% (RATG group) of the pre-treatment level respectively.Conclusion ATG-F 5 mag/(kg · d) and R-ATG 3.75mg/(kg · d) could achieve similar effects in the treatment of childhood AA,through similar significant clearance of T cells.Therefore,all of these suggest that ATG-F and R-ATG might serve as the drugs of frontline choice for IST in childhood AA patients who do not have an available human leukocyte antigen identical related donor.%目的 比较抗人体T淋巴细胞球蛋白(ATG-F)和兔抗人胸腺细胞免疫球蛋白(R-ATG)治疗儿童

  16. 单倍体造血干细胞移植治疗儿童重型再生障碍性贫血%Haploidentical Hematopoietic Stem Cell Transplantation for the Treatment of Severe Aplastic Anemia in Children

    Institute of Scientific and Technical Information of China (English)

    刘英; 唐锁勤; 黄文荣; 李红华; 赵瑜; 薄剑; 张宁; 王芳; 于力

    2013-01-01

    本研究分析非体外去T细胞亲缘单倍体造血干细胞移植(hi-HSCT)治疗儿童重型再生障碍性贫血(SAA)的疗效.我院于2010年10月-2013年3月对2例SAA/极重型再生障碍性贫血(VSAA)患儿进行了非体外去T淋巴细胞的父亲2个HLA位点不合的HSCT.2例在病程4个月内用环孢素A(CsA)+粒细胞集落刺激因子(G-CSF)治疗无效,有活动或重症感染,输血依赖,无HLA全合的同胞供者或非血缘供者.移植前预处理用福达拉滨(Flu)+环磷酰胺(Cy)+抗胸腺细胞球蛋白(ATG).移植物为G-CSF动员的外周血造血干细胞(PBHSC)和骨髓(BM).移植物抗宿主病(GVHD)预防用CsA+骁悉(MMF)+短程甲氨蝶呤(MTX).结果2例患儿均达到100%供者植入,粒细胞植入时间分别为移植后12 d、18 d,血小板植入时间分别为移植后17 d、26 d.2例均出现I度急性GVHD (aGVHD),l例发展为可控制的局限性慢性GVHD(cGVHD).2年随访期间,2例患儿保持稳定的100%供者植入并有免疫功能的重建.结论:小数量临床研究结果提示在没有同胞HLA全合供者或非血缘HLA全合供者时,亲缘单倍体造血干细胞移植对儿童SAA是适宜的选择,总体生存率(OS)的提高还有待大规模前瞻性临床研究的开展.%This study was purposed to assess the effectiveness of haploidentical hematopoietic stem cell transplantation(HSCT) without in vitro T cell depletion for the treatment of severe aplastic anemia(SAA) in children.Two children with SAA/very SAA(VSAA) received T cell-depleted HSCT from their fathers with 2 loci mismatched in our center between October 2010 and March 2013.During 4 months after onset,both failed in treatment of cyelosporine(CsA) + granulocyte colony stimulating factor (G-CSF),had active or serious infections,were transfusion dependent and lacked HLA-identical sibling donors and unrelated donors.The conditioning regimen before HSCT included fludarabine,cyclophosphamide and thymoglobulin.The source of grafts was a

  17. Development of acute leukemia in a known case of fanconi anaemia ( aplastic anaemai

    Directory of Open Access Journals (Sweden)

    Preeti Jhaveri

    2013-01-01

    Full Text Available Fanconi anemia is an autosomal recessive disease associated with an abnormal DNA damage. Although Fanconi anemia is well known for its association of Aplastic anemia and characteristic birth defects, leukemia and solid tumors also occur at a high rate in this group of patients. A patient male / 20yrs, known case of Fanconi anemia presented with ulcer over left lower limb. On further evaluation, the patient was found to have pancytopenia and his peripheral smear revealed many atypical blast like cells. So bone marrow study was done which revealed it to be Acute leukemia probably Acute Myeloid leukemia.

  18. Allogeneic bone marrow transplantation for severe aplastic anemia patients with risk factors for poor prognosis: is fludarabine a requirement? Transplante alogênico de medula óssea em portadores de anemia aplásica severa com fatores de mau prognóstico: é necessário fludarabina?

    Directory of Open Access Journals (Sweden)

    Carlos R. de Medeiros

    2008-08-01

    Full Text Available Hematopoietic progenitor cell transplantation from HLA-identical sibling donors cures 70-90% of Severe Aplastic Anemia (sAA patients. Older age, heavy exposure to transfusions, immunosuppression treatment (IST with a long interval from diagnosis to transplant and infection at procedure are associated with poor outcomes. We transplanted 18 patients with sAA and at least one risk factor (RF for poor prognosis (age >35 years, >50 transfusions prior to transplant, unresponsiveness to previous IST and bacterial or fungal infection at transplant from 2001 to 2005, using cyclophosphamide (CY - 5 patients or busulfan plus CY (13 patients. Sixteen patients engrafted, two died with no engraftment, three patients had evidence of graft failure at days +67, +524 and +638 (two died and one was rescued with IST. Grade III/IV mucositis occurred in 39% but neither aGVHD nor cGVHD were observed. The Kaplan-Meier probability of survival was 75% at 2.14 years, with a trend favoring survival by number of RF (1 versus =2 RF (P = 0.06. These results are comparable to recent data reported with fludarabine-based conditioning in patients with poor prognosis sAA. Due to the small sample size, prospective clinical trials with larger cohorts of patients are needed to confirm the real benefits of fludarabine-based conditioning, and also to define the best agent(s to be associated with Fludarabine as preparative regimen for sAA patients with poor prognosis.Transplante de medula óssea de doador irmão HLA-idêntico pode curar 70%-90% dos portadores de anemia aplásica severa (AAs. Pacientes mais idosos, muito transfundidos, longamente tratados com imunossupressão (IS e com infecções ao tempo do transplante têm pior evolução. Nós transplantamos 18 pacientes com AAs e pelo menos um dos fatores associados a pior prognóstico (idade >35 anos, >50 transfusões antes do transplante, falta de resposta à imunossupressão prévia e infecção bacteriana ou fúngica ao

  19. Aloimunidade contra antígenos HLA de classe I em pacientes com síndromes mielodisplásicas e anemia aplástica Aloimmunity against HLA class I antigens in patients with myelodisplastic syndrome and aplastic anemia

    Directory of Open Access Journals (Sweden)

    Daisy M. M. Arruda

    2008-02-01

    Full Text Available As síndromes mielodisplásicas (SMD e a anemia aplástica (AA apresentam citopenias periféricas necessitando, com freqüência, de reposições transfusionais contínuas de concentrados de hemácias e/ou de concentrados de plaquetas. O objetivo do presente estudo foi verificar a ocorrência de anticorpos anti-HLA de classe I em pacientes portadores das SMD e AA atendidos no ambulatório de Hematologia do Hemoce/UFC. Foram analisados 110 pacientes, sendo 70 com SMD e 40 com AA. A pesquisa de anticorpos anti-HLA de classe I foi realizada frente a um painel (PRA, utilizando-se a técnica de microlinfocitotoxicidade dependente do complemento. Vinte (28,6% dos 70 pacientes com as SMD e 18 (45% dos 40 pacientes com AA desenvolveram anticorpos anti-HLA contra o PRA. Esses pacientes que receberam uma carga de antígenos estranhos advindos de múltiplas transfusões de vários doadores de CH e/ou CP, geralmente desenvolvem aloanticorpos contra os antígenos HLA presentes na superfície das plaquetas e dos leucócitos que contaminam esses concentrados. A produção desses anticorpos pode trazer sérias complicações para o tratamento dos pacientes com SMD e AA. As avaliações sistemáticas para detecção de anticorpos anti-HLA após a reposição transfusional podem ser valiosas para adoção de estratégias transfusionais mais adequadas para esta população de pacientes.Patients with myelodysplastic syndromes (MDS or aplastic anemia (AA present peripheral cytopenias and require continuous transfusions of red cell and/or platelet concentrates. The objective of this study is to verify the existence of anti-HLA class 1 antibodies in patients with MDS and AA treated at the hematology Out patient Clinic of Hemoce/UFC. A total of 110 patients were analyzed, 70 with MDS and 40 with AA. Anti-HLA class 1 antibody detection was achieved with an antibody reactivity panel using the complement-dependent microlymphocytotoxicity technique. A total of 20 (28.6% of

  20. Aplastic crisis and leg ulceration: two rare complications of hereditary sideroblastic anaemia.

    Science.gov (United States)

    Mehta, J; Singhal, S; Mehta, B C

    1992-07-01

    Aplastic crisis as a result of parvovirus infection is seen in a number of haematologic disorders characterized by decreased red cell survival, and leg ulceration due to unknown causes is seen in a number of haemolytic anaemias. Neither of the two has been reported in a case of sideroblastic anemia. We report one case with each of these complications in association with sideroblastic anaemia.

  1. 造血干细胞移植治疗重型再生障碍性贫血进展%Progression of haemopoietic stem cell transplantation on severe aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    胡依民; 储榆林

    2006-01-01

    再生障碍性贫血(aplastic anemia,AA)是一组异质性疾病,部分患者的病情较重,诊断为重型再生障碍性贫血(severeaplastic anemia,SAA),具有以下特点:骨髓活检中的细胞成分少于25%,并且具有以下3项中的2项:中性粒细胞少于0.5×109L-1,血小板少于20×109L-1,网织红细胞的绝对数少于20×109L-1,当中性粒细胞少于0.2×109L-1时称为极重型再生障碍性贫血(verysevere aplastic anemia,VSAA)。

  2. 表现为大细胞性贫血的再生障碍性贫血患者长期随访研究%Aplastic anemia with macrocytic anemia: a study based on long-term follow-up

    Institute of Scientific and Technical Information of China (English)

    李英梅; 郑以州; 邹典斌; 李星鑫; 孙慧; 孙玲; 万鼎铭; 刘林湘; 陈胜梅; 陈绍倩; 刘少君

    2013-01-01

    目的 探讨表现为大细胞性贫血的再生障碍性贫血(AA)患者的临床特征.方法 回顾性分析2000年1月至2011年10月568例大细胞性贫血AA(mAA)患者的临床资料.结果 ①568例初诊AA患者中153例(26.9%)为mAA,其中非重型AA(NSAA)114例(74.5%)、重型AA (SAA) 39例(25.5%)、超重型AA(VSAA)0例;而376例正细胞性贫血AA(nAA)患者中NSAA、SAA、VSAA比例分别为16.2%、45.2%、38.6%(χ2=181.390;P=0.000).mAA患者中位年龄明显高于nAA组[30(4~70)岁对19(3~68)岁,P=0.001].②NSAA、SAA组mAA与nAA患者的HGB、中性粒细胞绝对值、血小板计数、6个月疗效、总体生存(OS)率差异均无统计学意义.但SAA组mAA患者的外周血中位网织红细胞计数(Ret)显著高于nAA患者[23.90(2.99~61.00)×109/L对13.10(0~70.60)×109/L,P=0.000],骨髓红系比例中位值亦高于nAA[0.235(0~0.580)对0.145(0~0.650),P=0.043];而NSAA组mAA患者外周血Ret、骨髓红系比例比较差异均无统计学意义.③mAA、nAA组患者初诊时PNH克隆阳性、染色体异常患者的比例比较,差异均无统计学意义.随访153例mRNA、376例nAA患者中分别有7、9例转化为PNH(χ2=1.099,P=0.294),3、13例转化为骨髓增生异常综合征/急性髓系白血病(χ2=0.399,P=0.528),差异均无统计学意义.结论 与nAA患者比较,mAA患者发病年龄偏大,NSAA比例、外周血Ret水平及骨髓红系比例较高,但治疗反应、OS率及克隆性疾病转化率差异无统计学意义.%Objective To elucidate the clinical features, response rate, prognosis and clonal evolution of aplastic anemia (AA) with macrocytic anemia(mAA). Methods The clinical features at initial diagnosis and datas in follow up of mAA hospitalized from January 2000 to October 2011 were analyzed retrospectively. Results ① Of 153/568 (26.9%) cases of mAA at initial diagnosis, 114(74.5%)were non-severe AA (NSAA), 39(25.5%)severe AA (SAA) and 0 very severe AA (VSAA), while the proportion was 16.2%, 45.2%, and 38

  3. Red cell indices for distinguishing macrocytosis of aplastic anaemia and megaloblastic anaemia.

    Science.gov (United States)

    Gupta, P K; Saxena, Renu; Karan, A S; Choudhry, V P

    2003-07-01

    Megaloblastic anaemia and aplastic anaemia are important causes of pancytopenia in India. Since both may have presence of macrocytes, peripheral smear examination alone may pose a difficulty in distinction between the two in the absence of macro-ovalocytes and hypersegmented neutrophils. The present study was conducted to evaluate the role of red cell indices in differentiation between macrocytosis of aplastic anaemia and megaloblastic anaemia. Haemogram from 25 cases each of biopsy proven megaloblastic anaemia and aplastic anaemia were reviewed. It was observed that MCV was greater than 97 fl in 15 cases of aplastic anemia (mean MCV 109.7 fl), and 25 cases of megaloblastic anaemia (mean MCV 113.2 fl). Hb, MCV & MCHC were comparable in the two groups. However, mean RDW in megaloblastic anaemia (mean 87.7 fl) was significantly higher than those in aplastic anaemia (mean 71.4 fl). The difference in RDW of patients with megaloblastic anaemia and aplastic anaemia was statistically significant. We conclude that RDW can be of help to differentiate between the two conditions.

  4. 再生障碍性贫血中医辨证分型的物质基础研究进展%Current Reseach of Basic Substances by TCM Syndrome Differentiation on Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    刘宝山; 戴锡孟

    2005-01-01

    再生障碍性贫血(Aplastic Anemia,AA,简称再障)是一组由化学物质、生物因素、放射线或不明原因引起的骨髓造血功能衰竭,以造血干细胞损伤、骨髓脂肪化、外周血全血细胞减少为特征的疾病。中医学虽无再障这一病名,但根据其临床表现,可属于“急劳”、“热劳”、“血证”、“虚劳”、“血虚”、“血证”之范畴。

  5. Unexpected Anemia and Reticulocytopenia in an Adolescent With Sickle Cell Anemia Receiving Chronic Transfusion Therapy.

    Science.gov (United States)

    Blauel, Emily R; Grossmann, Lily T; Vissa, Madhav; Miller, Scott T

    2015-10-01

    In a patient with sickle cell disease receiving chronic transfusion, exacerbation of anemia with reticulocytopenia must prompt consideration of a delayed hemolytic transfusion reaction with hyperhemolysis, as further transfusion may worsen this condition; definitive diagnosis is sometimes difficult. Anemia evolving during parvovirus B19-induced erythroid hypoplasia (transient aplastic crisis) should be attenuated in chronic transfusion patients due to superior survival of transfused over endogenous red blood cells. A 16-year-old with sickle cell disease receiving chronic transfusion of modified intensity (goal to maintain hemoglobin Sanemia with reticulocytopenia was later shown to have had transient aplastic crisis.

  6. 父母供者外周血单倍体移植治疗儿童难治性重型再生障碍性贫血%Parental haploidentical peripheral blood stem cell transplantation for treatment of children with refractory severe aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    万鼎铭; 陈晓娜; 曹伟杰; 邢海洲; 何海燕; 柳飞; 陈诗彧; 庞雨晴

    2015-01-01

    BACKGROUND:For pediatric patients with aplastic anemia in China, it is difficult to find human leucocyte antigen-matched sibling donors that are mostly replaced by parental donors. OBJECTIVE:To retrospectively analyze the clinical efficacy and safety of parental haploidentical peripheral blood hematopoietic stem cel transplantation in children with relapsed and refractory severe aplastic anemia. METHODS:Seventeen children with relapsed and refractory severe aplastic anemia who had no matched sibling or unrelated donor and failed to respond to immunosuppressive therapy were subjected to parental haploidentical peripheral blood hematopoietic stem cel transplantation. A conditioning regimen of fludarabine+cyclophosphamide+rabbit anti-human thymocyte immunoglobulin antibody and the triple therapy of methotrexate, cyclosporine A and mycophenolate mofetil were applied to prevent graft-versus-host disease. RESULTS AND CONCLUSION: (1) Of the 17 children, 16 cases (94%) reached hematopoietic reconstitution, and the median time of neutrophils≥ 0.5×109/L and platelets≥ 20×109/L was 13 (11-15) days and 17 (12-28) days, respectively. (2) Incidence of acute graft-versus-host disease was 47% (8 of 17 cases), including 29% (5/17) of grades I-II and 18% (3/17) of grades III-IV. Incidence of chronic graft-versus-host disease was 41% (7/17). (3) With a median folow-up duration of 268 (43-753) days, the overal survival rate was 70.6% (12/17). Five dead cases (29%) belonged to transplantation-related death, including one case of fungal skin infections, one case of graft-versus-host disease, three cases of severe lung infection. No relapse case was reported. These findings indicate that if there are no matched sibling or unrelated donors and the immunosuppression effect is poor, parental haploidentical peripheral blood hematopoietic stem cel transplantation is a safe and effective salvage treatment for children with relapsed and refractory severe aplastic anemia.%移

  7. A new method of inducing immune tolerance for haplotype hematopoietic stem cell transplantation in the treatment of severe aplastic anemia%免疫耐受新方法单倍型造血干细胞移植治疗重型再生障碍性贫血

    Institute of Scientific and Technical Information of China (English)

    郭智; 陈惠仁; 杨凯; 刘晓东; 楼金星; 何学鹏

    2015-01-01

    BACKGROUND:Alogeneic hematopoietic stem cel transplantation (alo-HSCT) is an effective mean to cure severe aplastic anemia, and especialy haplotype transplantation is regarded as a transplantation system with Chinese characteristics, and rank at the international leading level. OBJECTIVE:To explore the patterns of haplotype alo-HSCT as a new immune tolerance method for severe aplastic anemia and to solve the transplantation rejection and graft-versus-host disease. METHODS:Twelve patients with severe aplastic anemia who underwent haplotype alo-HSCT at the Department of Hematology, General Hospital of Beijing Military Area, China from April 2013 to May 2014 were enroled. Al these patients received the new regimen of inducing immune tolerance through the application of high-dose cyclophosphamide (400 mg/m2, consecutively 3 days before transplantation; 50 mg/kg, consecutively 3 days after haplotype transplantation). RESULTS AND CONCLUSION:The median time of neutrophil recovery was 17 (13-21) days, and the median time of platelet recovery was 21 (15-31) days. After transplantation, there were one case of degree II acute graft-versus-host disease and one case of chronic graft-versus-host disease, both of which were controled. The folow-up time was 6 months at least, and the median time was 11 months. During the folow-up, one case died of rejection reaction and one case died of severe lung infection. These findings indicate that the new method of inducing immune tolerance with high-dose cyclophosphamide after transplantation for severe aplastic anemia has significant effects in reducing graft-versus-host disease and transplantation-related mortality rate.%背景:异基因造血干细胞移植是根治重型再生障碍性贫血的有效手段,尤其单倍型造血干细胞移植是具有中国特色的移植体系,在国际上处于领先水平。目的:探索免疫耐受新方法单倍型异基因造血干细胞移植治疗重型再生障碍性贫血的模式

  8. Pernicious anemia

    Science.gov (United States)

    ... malabsorption); Anemia - intrinsic factor; Anemia - IF; Anemia - atrophic gastritis ... of pernicious anemia include: Weakened stomach lining (atrophic gastritis) An autoimmune condition in which the body's immune ...

  9. 黄世林教授治疗再生障碍性贫血的经验%Professor Shi-lin Huang's experience in treatment of aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    陈楠楠

    2008-01-01

    再生障碍性贫血(aplastic anemia,AA)是血液系统较常见的疾病。解放军第210医院中医血液科黄世林教授采用中医药主治、中西医结合、辨证分型、分期论治的方法治疗AA30余年,积累了丰富的经验。在统计的149例AA患者中,急、重型AA46例,治愈缓解率为63.04%,总有效率76.08%;慢性AA103例,治愈缓解率57.28%,总有效率74.47%,疗效显著。吾跟师学习数年,耳濡日染,感受颇深,现将师之宝贵经验总结如下,望可发扬之。

  10. 含盐酸二甲双胍联合方案治疗再生障碍性贫血的短期疗效观察%Observations on the short-term therapeutic effects of combined therapy with metformin hydrochloride for aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    卢学春; 李松威; 脱帅; 张峰; 脱朝伟; 姚善谦; 范利; 杨波; 迟小华; 蔡力力; 于睿莉; 刘洋; 刘丽宏; 李炳军; 吴晓雄

    2012-01-01

    目的 采用临床生物信息学方法筛选治疗再生障碍性贫血(AA)的新型药物并评估其临床疗效.方法 首先建立AA的人类全基因组表达谱,与药物基因组学数据库进行相似性分析,筛选可能有效的药物.纳入难治性AA患者[接受过免疫抑制剂和(或)雄激素治疗时间超过6个月无效]作为研究对象评估疗效,6个月后评估临床疗效和不良反应.结果 临床生物信息学筛选结果显示盐酸二甲双胍可能对AA有治疗作用.纳入43例难治性AA患者(其中15例为重型AA),采用包含盐酸二甲双胍、环孢素A(CsA)和司坦唑醇的联合方案进行治疗,结果显示:27例输血依赖患者在治疗6个月后全部(100%)停止输血;40例贫血患者中,37例(92.5%)血红蛋白完全恢复正常;30例血小板低于20×109/L的患者中,28例(93.3%)升至50×109/L以上;35例白细胞低于2.5×109/L的患者中,31例(88.6%)升至3.5×109/L以上.40例贫血患者中,1例出现肾功能异常,停用环孢素A后恢复正常;18例出现转氨酶升高,加用保肝药物并减少司坦唑醇用量后恢复正常.所有患者均未出现低血糖反应.结论 盐酸二甲双胍、环孢素A和司坦唑醇联合方案治疗难治性AA有效.%Objective This paper aims to screen and select new drugs with potential in the treatment of aplastic anemia, and to evaluate their clinical efficacy through clinical bioinformatics methods. Methods First, we established genome expression profiles of aplastic anemia (AA) patients, and conducted similarity analyses with the pharmacogenomics database to screen and select drugs with possible efficacy. Intractable AA patients who received immunosuppressors and/or androgen for more than six months showing no clinical efficacy were included for the study to evaluate therapeutic effects of the therapeutic regime for thestudy. Clinical efficacy and adverse effects were evaluated after six months. Results The clinical bioinformatics

  11. The clinical efficacy of hemopoietic growth factor on adult aplastic anemia patients with severe infection%造血生长因子治疗成人再生障碍性贫血伴严重感染的疗效观察

    Institute of Scientific and Technical Information of China (English)

    陆亚岚; 高清平

    2014-01-01

    目的:观察造血生长因子对成人再生障碍性贫血伴严重感染患者的疗效,对造血生长因子的安全性及有效性作出评价。方法选取2010年3月至2014年3月治疗的再生障碍性贫血伴严重感染患者80例,采用随机数表法随机分为观察组与对照组,对照组接受免疫抑制治疗( IST),观察组在对照组基础上给予粒细胞集落刺激因子( G -CSF)治疗,统计并分析两组治疗的有效率以及治疗1月后感染相关死亡率、克隆性演变发生率,对造血生长因子安全性及有效性作出评价。结果观察组治疗有效率为95.0%(38/40),对照组治疗有效率为80.0%(32/40),观察组治疗有效率明显高于对照组( P ﹤0.05)。观察组死于肺部感染1例,死于败血症1例,感染相关死亡率为5.0%(2/40);对照组死于肺部感染2例,死于胃肠道感染1例,死于败血症3例,感染相关死亡率为15.0%(6/40),观察组感染相关死亡率低于对照组,但差异无统计学意义( P ﹥0.05)。观察组出现骨髓增生异常综合征1例,急性髓系白血病1例,夜间阵发性血红蛋白尿2例,克隆性演变发生率10.0%(4/40);对照组出现夜间阵发性血红蛋白尿1例,克隆性演变发生率为2.5%(1/40),观察组克隆性演变发生率低于对照组,但差异无统计学意义( P ﹥0.05)。结论造血因子对于控制成人再生障碍性贫血并发感染具有良好效果,且不明显增高克隆性疾病的发生率,是一种安全有效的治疗方式,值得临床推广使用。%Objective To observe the clinical efficacy of hemopoietic growth factor treatment on adult aplastic anemia patients with severe infection,and to evaluate the safety of the treatment. Methods From March 2010 to March 2014,80 aplastic anemia patients complicated with severe infection were randomly divided into observation group and control group,the control

  12. A clinical analysis of myelodysplastic syndrome and aplastic anemia combined with autoimmune disease%骨髓增生异常综合征与再生障碍性贫血并发自身免痰性疾病的临床分析

    Institute of Scientific and Technical Information of China (English)

    许晓倩; 王健民; 吕书晴; 杨建民; 陈莉; 宋献民; 章卫平; 侯军

    2009-01-01

    Objective To investigate the characteristics of pathogenesis and therapeutics of myelodysplastic syndrome(MDS)and aplastic anemia(AA)combined with autoimmune disease(AID).Methods Retrospective analysis and follow-up visit of 111 patients with MDS and 56 patients with AA in our hospital were studied.Results There were 9(8.1%)of 111 patients with MDS and 2 (3.6%)of 55 patients with AA coexistent with AID.Autoimmune hemolytic anemia(36.4%)and Behcet Disease(18.2%)were common among those combined with AID.5 patients had AID proceeding to the occurrence of MDS/AA.4 patients had simultaneous occurrence of AID and MDS/AA.2 patients developed AID three years later after the diagnosis of MDS.Conclusion There was a certain intrinsic relationship between MDS/AA and AID.%目的 探讨骨髓增生异常综合征(MDS)与再生障碍性贫血(AA)并发自身免疫性疾病(AID)的发病特点及治疗学特点.方法 对该院111例MDS患者与56例AA患者的临床资料进行回顾性分析与随访观察.结果 111例MDS患者中有9例并发AID(8.1%),56例AA患者中有2例并发AID(3.6%),并发的AID以自身免疫性溶血性贫血(36.4%)与白塞病(18.2%)多见.5例患者AID发病在前,4例患者MDS和(或)AA与AID同时发病,2例患者MDS发病后3年并发AID.结论 MDS和(或)AA与AID具有一定的内在相关性.

  13. In Vitro Study of Shengxue Mixture Interfering the Expressions of T-bet GATA-3 Relevant Signal Transduction MolecuLar and Cytokine of Peripheral Blood Mononuclear Cell from Patients of Chronic Aplastic Anemia%生血合剂影响慢性再障患者外周血单个核细胞T-bet/GATA-3及相关信号分子、细胞因子表达的体外研究

    Institute of Scientific and Technical Information of China (English)

    李峻; 周永明; 胡明辉; 薛志忠

    2013-01-01

    Objective: Transcription factors T-bet ( T-box expressed in T cell ), GATA-3 ( GATA binding protein 3 ) and relevant signal transduction pathways play important roles in the balance, differentiation of Th1/Th2 cells and regulation of immunological responses. This study aims to explore the effects of Shengxue mixture ( SXM ) on the expressions of T-bet, GATA-3, relevant signal transduction Molecular and Cytokine of peripheral blood mononuclear cell ( PBMNC ) from patients of chronic aplastic anemia ( CAA ) in vitro. Methods: 10 patients with CAA ( 5 male,5 female, age range from 42 to 73 ) were included. They all didn't take Chinese materia medica or cyclosporin A ( CsA ) within 3 days before drawing peripheral blood. SXM was made into aseptic parenteral solution containing 2 grams of crude drug per milliliter. CsA was diluted into solution ( 1 mg/mL of concentration ) with aseptic cell c μ, Lture fluid ( RPMI1640 ) . PBMNC from CAA patients was isolated and cultured in 72 h with or without drugs. They were divided into four groups, i.e. group of aplastic anemia PBMNC without adding drugs ( group Blank ), group of aplastic anemia PBMNC with adding high-dose Shengxue Mixture ( group HDSXM ), group of aplastic anemia PBMNC with adding low-dose Snengxue mixture ( group LDSXM ) and group of aplastic anemia PBMNC with adding CsA ( group CsA ). Finally, mRNA expressions of PBMNC transcriptional factors and signaling molecule were determined with methods of realtime fluorescent quantitation polymerase chain reaction ( Realtime FQ-PCR ), and levels of cytokine in the supernatant were detected by applying enzyme linked immunosorbent assay ( ELISA ) . Results: After culturing in vitro, expressions of T-bet, STAT4, IFN-γ and IL-12 in blank group were significantly higher than those in LDSXM group, HDSXM group, and CsA group ( P0.05 ) . In the expressions of GATA-3, STAT6 and IL-4, no significant difference existed between these groups ( P>0.05 ) . Conclusion: Shengxue

  14. DIAGNOSIS AND THERAPY IN CHILDREN WITH SEVERE APLASTIC ANEMIA COMPLICATING CAPILLARY LEAK SYNDROME: REPORT OF TWO CASES%儿童重型再生障碍性贫血并发毛细血管渗漏综合征诊治探讨(附2例报告)

    Institute of Scientific and Technical Information of China (English)

    马素亭; 孙立荣

    2012-01-01

    目的 探讨联合免疫抑制治疗重型再生障碍性贫血(SAA)并发毛细血管渗漏综合征(CLS)儿童的临床特征、发病机制及诊治方法.方法 总结2011年我科行联合免疫抑制治疗SAA并发CLS病儿2例的临床特点及诊治过程,并结合相关文献进行分析.结果 2例病儿分别于连续5d应用抗胸腺细胞免疫球蛋白(ATG)结束后38 d和1d发生CLS,临床特征有体质量增加、腹围增加、双下肢轻度凹陷性水肿、胸腔积液、心包积液、腹水,非感染性腹泻、低清蛋白血症,单纯输注清蛋白无效,以及无明显低血容量性低血压.经糖皮质激素+羟乙基淀粉+呋塞米+清蛋白治疗后,症状及体征均逐渐消失.结论 CLS是联合免疫抑制治疗SAA过程中的一种罕见并发症,早期诊断和治疗可治愈.%Objective To explore the clinical features and pathogeneay in children with severe aplastic anemia (SAA) complicating capillary leak syndrome (CLS) treated with combined immunosuppressive (CIS) therapy. Methods A summary of clinical features and the course of therapy in two children with SAA complicating CLS treated with CIS was done and literature concerned revieved. Results The two sick kids received continuous therapy for five days with antithymocyte immunoglobin (AT1G). One day and 38 days after completion of the treatment, the patients developed CLS. The clinical manifestations were weight gain, increase of abdomen circumference, mild pitting edema of lower extremity, pleural effusion, pericardial effusion, asri-tes, non-infectious diarrhea, hypoalbuminemia and non-hypovolemic hypotension. Their symptoms and signs dribbled away after corticosteroids + hetastarch+furosemide+albumin therapy. Conclusion CLS is a rare complication in patients with SAA treated with immunosuppressive therapy, which can be cured if diagnosed and treated early.

  15. Pathogenesis of chronic aplastic anemia suffering from kidney yin deficiency through the maternal genetics%从母系遗传研究肾阴虚型慢性再生障碍性贫血的发病机制

    Institute of Scientific and Technical Information of China (English)

    崔兴; 张文静; 蔡治国; 徐瑞荣; 刘菲; 王敬毅; 刘奎

    2012-01-01

    BACKGROUND: Several laboratories have reported unexpectedly large number of mitochondrial mutations in leukemia. But the direct relations hip between mitochondrial mutations and chronic a plastic anemia (CAA) has not studied yet. OBJECTIVE: To study the mitochondrial mutations of CAAsuffered from kidneyyin deficiency and kidney yang deficiency.andCAA suffered from kidney yin deficiency in order to further study the pathogenesis of CAA.METHODS: The bone marrow and the oral epithelium were obtained from 10 patients with CAA suffered from kidney yindeficiency and 5 patients with CAA suffered from kidney yang deficiency. DNA was extracted and underwent the entireRESULT SAND CONCLUSION: The entire sequencing of mitochondrial DNA in CAA suffer from kidney yin deficiency showed that the mutations were occurrence in the areas that closely related with mitochondrial oxidative respiratory chain, it included themutations in CAA suffered from kidney yang deficiency were not obvious. We are led to conclude that mitochondrial gene mutation can change the expression of respiratory chain enzyme complex in CAA patients, which results in energy metabolismimpairment may participate in the physiological and athology processes of hem atopoietic failure,Functional impairment of mitochondrial respiration chain induced by gene mutation may be an important reason of hematopoietic failure in CAA.And this change is closely related to maternal inheritance and kidney yin deficiency.%背景:多项研究表明恶性血液病可以出现线粒体的突变,但尚未有关于慢性再生障碍性贫血中线粒体变化的研究.目的:研究肾阴虚和肾阳虚型慢性再生障碍性贫血患者线粒体突变情况,探讨母系遗传的本质--线粒体与肾阴虚型慢性再障发生、发展的关系,以期进一步研究慢性再障的发病机制.方法:收集10例诊断明确的肾阴虚型5例肾阳虚型慢性再生障碍性贫血患者骨髓和口腔黏膜上皮,提取DNA,进行线粒

  16. 大剂量环磷酰胺治疗β地中海贫血合并重型再生障碍性贫血一例报告附文献复习%High-dose cyclophosphamide for severe aplastic anemia associated with β-thalassemia: a case report and literatures review

    Institute of Scientific and Technical Information of China (English)

    赵馨; 李园; 周康; 张莉; 井丽萍; 叶蕾; 彭广新; 李洋; 李建平

    2013-01-01

    Objective To investigate the clinical features and therapeutic method for severe aplastic anemia (SAA) associated with β-thaiassemia,and to improve the recognition of the disease.Methods One patient hospitalized for pancytopenia was reported and the related literatures were reviewed.Results A 14-years old girl who presented with anemia from her childhood was hospitalized for acute onset of pancytopenia.Routine blood test showed that WBC count was 1.28 × 109/L,hemoglobin 65 g/L,platelet count 18 ×109/L,reticulocyte count 2 × 109/L,neutrophil count 0.03 × 109/L and mean corpuscular volume 59.6 fl,respectively.Both bone marrow aspiration and biopsy showed hypoplasia.Her red blood cells presented as microcytic hypochromic and target erythrocytes were common on peripheral blood smear.DNA analysis of the patient and her mother showed exon 17 heterozygous β-thalassemia (c.52 A > T).A diagnosis of SAA associated with β-thalassemia was clarified and high-dose cyclophosphamide (HD-CTX,1.2 g/d × 4 d) plus cyclosporine were offeved,which eventually led to a complete hematologic remission 12 months later.Conclusion This was the first report of SAA associated with β-thalassemia,and the regimen of HD-CTX led to a complete hematologic remission.%目的 分析1例β地中海贫血合并重型再生障碍性贫血(SAA)患者的临床特征及治疗方法,以提高对该类疾病诊治方法的认识.方法 分析1例因全血细胞减少就诊患者的临床资料,并进行相关文献复习.结果 患者,女,14岁,自幼贫血,此次急性起病,血常规检查示WBC 1.28×109/L,HGB 65 g/L,PLT 18×109/L,网织红细胞计数2×109/L,中性粒细胞0.03 × 109/L,红细胞平均体积59.6 fl.骨髓液涂片检查示增生减低,粒系占0.010,红系占0.170,成熟红细胞胞体小,中心淡染区扩大,全片未见巨核细胞,外周血涂片易见靶形红细胞.DNA杂交方法检测β地中海贫血基因显示为17号杂合子(c.52 A>T).确诊为β地中海贫血合

  17. 荧光原位杂交在诊断骨髓形态学拟诊的再生障碍性贫血中的应用%Application of fluorescent in situ hybridization in primary diagnosis of aplastic anemia by morphology

    Institute of Scientific and Technical Information of China (English)

    林晓岚; 陈万紫; 傅蔷; 陈加弟; 黄慧芳

    2016-01-01

    Objective To investigate the clinical application of fluorescent in situ hybridization (FISH) for the differential diagnosis of myelodysplastic syndromes (MDS) and a plastic anemia (AA).Methods A FISH kit capable of detecting the chromosomal abnormalities related to MDS was used to analyze 94 patients who were suspected to have AA by bone marrow morphology.Results Cytogenetic abnormalities were detected in 11 of the 94 patients,which included trisomy 8 (5 cases),20q-(1 case) and -Y (1 case).There were 4 cases related to MDS,which included 3 cases of 5q-,in which 1 case carry 20q-at the same time,and 7q-(1 case).No significant difference was found between the MDS and AA groups in terms of age,sex or routine blood examination including absolute neutrophil count,hemoglobin content and platelet count.Conclusion FISH can detect certain cytogenetic abnormalities related to MDS in patients morphologically diagnosed as AA.%目的 探讨采用骨髓增生异常综合征(myelodysplastic syndromes,MDS)组套探针的荧光原位杂交(fluorescent in situ hybridization,FISH)检测在鉴别诊断再生障碍性贫血(aplastic anemia,AA)与MDS中的临床价值.方法 应用针对MDS常见异常的组套探针,对94例形态学拟诊为AA患者的骨髓标本进行FISH检测,检出具有MDS诊断价值的遗传学异常,并比较MDS与AA两组患者临床特征的差异.结果 (1)94例AA患者中11例存在遗传学异常,其中8号三体5例,20q缺失和Y缺失各1例;检出具有MDS诊断价值的遗传学异常4例:包括5q缺失3例,其中1例伴有20q缺失;7q缺失1例.(2)遗传学MDS与AA两组在临床特征,如年龄、性别、入院时血常规的中性粒细胞绝对值、血红蛋白与血小板计数等方面的差别均无统计学意义.结论 通过FISH检测发现部分形态学拟诊AA的患者存在具有MDS诊断价值的细胞遗传学异常.

  18. 非血缘HLA相合供者造血干细胞移植治疗重型再生障碍性贫血的临床分析%Clinical Analysis of Haploidentical or Unrelated Donor Hematopoietic Stem Cell Transplantation for Patients with Severe Aplastic Anemia

    Institute of Scientific and Technical Information of China (English)

    陈惠仁; 刘丹; 楼金星; 张媛; 刘晓东; 杨凯; 陈鹏; 刘兵; 何学鹏; 郭智

    2012-01-01

    本研究旨在评价HLA不全相合的亲属供者或HLA相合非血缘供者造血干细胞移植在治疗重型再生障碍性贫血(SAA)的疗效和安全性.在2005年11月至2011年5月期间采用非血缘供者或者单倍体相合供者造血干细胞移植治疗SAA患者20例,其中亲缘HLA不合单倍体相合供者14例,非血缘HLA相合供者6例.预处理采用氟达拉滨(FLU)、环磷酰胺(CTX)和抗胸腺细胞球蛋白(ATG)方案,移植物抗宿主病(GVHD)预防方案为经典的环孢素A(CsA)联合短程甲氨蝶呤(MTX)及霉酚酸酯(MMF).对单倍体相合供者采集经G-CSF动员的骨髓及外周血干细胞联合应用;非血缘供者单纯采集外周血干细胞.结果表明:所有患者均获供者型造血重建,粒细胞植活中位时间14(11-20)d,血小板植活中位时间17(13-31)d,2例取得完全供者植入后2个月发生排斥,其中1例进行母亲单倍体相合供者二次移植,达到完全供者持久植入;移植后发生Ⅱ度急性GVHD 4例,慢性GVHD发生7例,其中1例为慢性广泛性GVHD;14例无病生存,所有存活患者最少随访时间在8个月以上,中位随访时间为48个月,血象完全恢复,Kaplan-Meier计算的累积无病生存率为68.9%.结论:采用FLU、CTX和抗淋巴细胞免疫球蛋白进行预处理,用HLA不全相合的亲属供者或HLA相合非血缘供者造血干细胞移植治疗SAA,植入率高,感染发生率降低,获得良好的长期生存疗效.%Objective of this study was to evaluate the efficacy and satety of haploidentical or unrelated donor hematopoietic stem cell transplantation (HSCT) for patients with severe aplastic anemia (SAA). Twenty patients with SAA received allogeneic HSCT from haploidentical or unrelated donors ( 14 from haploidentical donors and 6 from unrelated donors) from November 2005 to May 2011. Conditioning regimen consisted of fludarabine ( FLU ) , cyclophosphamide ( Cy) and anti-thymocyte immunoglobulin (ATG). The patients were

  19. Imbalance of Treg/Th17 cells in patients with aplastic anemia and regulative role of umbilical cord-derived mesenchymal stem cells%再生障碍性贫血患者Treg/Th17细胞失衡及脐带MSCs对其的调节作用

    Institute of Scientific and Technical Information of China (English)

    陈莹; 陈柯材; 刘林

    2013-01-01

    目的 研究Treg/Th17细胞的平衡状态在再生障碍性贫血(aplastic anemia,AA)发病机制中的作用及意义,探讨脐带间充质干细胞(umbilical cord-derived mesenchymal stem cells,UC-MSCs)对AA患者外周血Treg/Th17比率的调节作用.方法 体外分离、培养和鉴定UC-MSCs.流式细胞术(flow cytometry,FCM)检测10例健康对照者、15例AA患者外周血Treg细胞和Th17细胞分别占外周血单个核细胞(peripheral blood mononuclear cells,PBMCs)的百分比,比较Treg/Th17细胞的比率.将AA患者的PBMCs与UC-MSCs共培养72 h,检测AA患者单独培养组和与MSCs共培养组Treg细胞、Th17细胞分别占PBMCs的百分比,比较Treg/Th17比率的变化.结果 经FCM鉴定MSCs表面标记CD90、CD105阳性率≥98%,CD34、CD45阳性率≤1%.AA患者组外周血Treg细胞百分率明显低于健康对照组(P<0.05),Th17细胞百分率明显高于健康对照组(P<0.05),Treg/Th17细胞比率明显低于健康对照组(P<0.05).AA患者的PBMC与UC-MSCs共培养后,Treg细胞百分率明显高于单独培养组(P<0.05),Th17细胞百分率明显低于单独培养组(P<0.05),Treg/Th17细胞比率较单独培养组明显升高(P<0.05).结论 AA患者外周血存在Treg/Th17分化失衡;UC-MSCs可能通过抑制Th17细胞分化,诱导Treg细胞生成/聚集,使得其Treg/Th17的失衡在一定程度上得到恢复.%Objective To determine the role and significance of the balance of Treg/Th17 cells in pathogenesis of aplastic anemia (AA),and to investigate the regulatory effect of umbilical cord-derived mesenchymal stem cells (UC-MSCs) on the ratio of Treg/Th17 cells in patients with AA.Methods UC-MSCs were isolated,cultured and identified in vitro.Flow cytometry (FCM) was used to detect the percentage of Treg cells and Th17 cells in the peripheral blood mononuclear cells (PBMCs) from 10 healthy volunteers and 15 AA patients,respectively by FCM,and the ratio of Treg/Th17 cells was compared.After PBMCs of the

  20. Las anemias, sin anemia

    OpenAIRE

    Villamarin V., A.; Villamarin C., Maria José

    2011-01-01

    Estudiando las anemias idiopáticas, encontramos dos tipos: la anemia hipócroma megalocítica u enfermedad de Biermer y la anemia hipócroma microcítica o clorosis; en la mayoría de los casos estos dos tipos, se oponen por su figura hematológica, sus manifestaciones clínicas, su evolución y su terapéutica. La anemia de Biermer, tiene por remedio heroico el hígado a altas dosis; la segunda, el hierro en forma química. Pero debemos reconocer que ciertos síntomas son comunes a las anemias idiopátic...

  1. Hemolytic anemia

    Science.gov (United States)

    Anemia - hemolytic ... bones that helps form all blood cells. Hemolytic anemia occurs when the bone marrow isn't making ... destroyed. There are several possible causes of hemolytic anemia. Red blood cells may be destroyed due to: ...

  2. 兔抗人胸腺细胞免疫球蛋白治疗重型再生障碍性贫血25例疗效分析%The therapeutic effects of Antithymocyte globulin to treat children with severe aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    李玉玲; 薛惠良

    2011-01-01

    Objective To evaluate the effects and side effects of Antithymocyte globulin (ATG)to treat children with severe aplastic anemia (SAA). Methods The therapeutic effects of 25 children with SAA treated by Antithymocyte globulin were analyzed retrospectively from 2004 to 2009. Peripheral hemogram, long-term clone change and infection were observed after treatment for more than 6 months.Results ATG were effective in 17 children (68%). Among them, five (20%) cases were nearly cured, three (12%) got remission and nine (36%) got improved. Eight (32%) cases had no response to ATG treatment. Among them, two (8%) cases were cured after receiving allogeneic hematopoietic stem cell transplantation and two (8%) died because of infection after 6 months. One (4%) case had a clone change into MDS. One case (4%) got rash, fever and joint pain and two cases (8%) got laryngeal edema during ATG infusion. Some children had serum reaction in two weeks after ATG infusion. Conclusion The effective rate of ATG was 68%. The side effects of ATG included rash, fever and joint pain. Laryngeal edema was rare. Corticosteroid hormone should be considered to prevent allergic and serum reactions during ATG treatment.%目的 探讨使用兔抗人胸腺细胞免疫球蛋白(ATG)治疗重型再生障碍性贫血(SAA)的疗效及毒副反应.方法 回顾性分析2004-2009年共25例SAA患儿应用ATG+环孢素A+雄激素治疗后随访半年以上的疗效.观察患儿血象变化、远期克隆性改变、感染.结果 ATG治疗半年后25例患儿中17例(68%)有效,其中5例(20%)基本治愈,3例(12%)缓解,9例(36%)明显进步,8例(32%)无效中2例(8%)行异基因造血干细胞移植后治愈,2例(8%)半年后因感染死亡.随访期间1例SAA-II型发生远期克隆性改变转变为MDS.1例患儿在输注ATG过程中出现皮疹、发烧、关节痛;2例出现喉头水肿;输注后2周内患儿出现血清病反应,表现为皮疹、发热、关节痛.第1周7例,第2

  3. The impact of immunosuppressive therapy on genetic instabilities of bone marrow hematopoietic cells in patients with aplastic anemia%免疫抑制治疗对再生障碍性贫血患者骨髓细胞遗传不稳定性的影响

    Institute of Scientific and Technical Information of China (English)

    张丽红; 刘强; 储榆林; 张凤奎; 王慧君; 张莉; 周康; 杨栋林; 阎璋松; 李洪强; 刘庆国; 齐军元

    2008-01-01

    Objective To investigate the impact of immunosuppressive therapy(IST) on genetic in-stabilities of bone marrow hematopoietic cells (BMHCs) in patients with aplastic anemia(AA). Methods Comet assay as used to detect genetic instabilities of hematopoietic ceils from patients, and the percent of DNA in comet tail (TDNA), tail length(TL), tail moment(TM), olive tail moment(OTM) and the rate of comet cells were measured. BMHCs from AA patients were examined with comet assay before and after IST, and the results were compared with those from controls. Results Comet parameters from 91 AA patients in-cluding TDNA, TL, TM, OTM comet cell percentage were (5.0 ± 4.0) %, 11.3 ± 7.2, 1.7 ± 2.0, 1.5 ± 1.4, (16.8 ± 13.7)%, respectively, which were significandy higher than those from control group (P 0.05). The TDNA, TL, TM, OTM and comet cells percentage were (4.4±3.6)%, 10.4 ± 7.5, 1.4 ± 1.6, 1.3 ± 1.4 and (20.2 ± 21.2) %, respectively at 3 months after [ST in 53 SAA pa-tients and were (3.7 ± 3.3) %, 10.0 ± 7.2, 1.2 ± 1.8, 1.1 ± 1.3 and (18.5 ± 19.0) % respectively at 6 months after IST in 30 SAA patients, being no statistical difference from those of 58 SAA patients before IST (P values were all > 0. 05). Conclusion B MHCs of AA had inherent genetic instabilities which were not increased by recent IST. It indicated that there was no correlation between LST and the development of clonal hematologic disorders in AA.%目的 研究强烈免疫抑制治疗(IST)对再生障碍性贫血(AA)患者骨髓细胞遗传不稳定性的影响.方法 采用彗星试验检测患者骨髓细胞遗传不稳定性,以彗星尾部DNA(TDNA)百分比、尾长(TL)、尾矩(TM)、Olive尾矩(OTM)和彗星细胞率作为分析参数,分别检测IST前后AA患者骨髓细胞,并与对照组结果比较.结果 91例初治从患者TDNA百分比、TL、TM、OTM、彗星细胞率分别为(5.0±4.0)%、11.3±7.2、1.7±2.0、1.5±1.4、(16.8±13.7)%,明显高于对照组(P值均0.05),二组患

  4. 重型再生障碍性贫血免疫抑制治疗后恶性克隆造血临床分析%Clinical analysis on malignant clonal hematopoiesis in severe aplastic anemia patients with immunosuppressive therapy

    Institute of Scientific and Technical Information of China (English)

    崔宁博; 付蓉; 瞿文; 阮二宝; 王晓明; 王国锦; 吴玉红; 刘鸿; 关晶

    2015-01-01

    目的 探讨重型再生障碍性贫血(SAA)患者联合免疫抑制治疗(IST)后出现7号染色体缺失(-7)恶性克隆造血的临床特征和危险因素.方法 回顾性分析天津医科大学总医院血液科2004年10月至2012年1月IST后出现-7染色体异常的SAA患者的临床资料.结果 SAA患者IST后出现-7染色体异常的比例为4.2% (6/143),出现异常的中位时间是IST后36(12~ 75)个月.此6例患者均诊断为骨髓增生异常综合征(MDS),其中3例进展为急性髓系白血病(AML),时间分别为IST后24、45和51个月.中位随访时间42(17~84)个月,4例患者随访期间死亡,从确诊MDS到死亡中位时间是9(5 ~17)个月,3例患者死于感染,1例患者死于脑出血.6例患者临床特点包括IST后6个月治疗无效、IST联合重组人粒细胞集落刺激因子(rHu-GCSF)治疗后1个月单核细胞百分比升高、IST联合rHu-GCSF后3个月处于粒细胞缺乏状态.结论 SAA患者经IST后粒系反应不良提示恶性克隆性造血存在,且预后差.%Objective To investigate the clinical characteristics and risk factors of monosomy 7 malignant clonal evolution in patients with severe aplastic anemia (SAA) treated with combined immunosuppressive therapy(IST).Methods The clinical data of SAA patients treated with IST who had monosomy 7 malignant clonal evolution from October 2004 to January 2012 were analyzed respectively.Results Six patients (4.2%) had monosomy 7 clonal evolutions.The median time to monosomy 7 was 36 (12-75) months after IST.All 6 patients were diagnosed myelodysplastic syndromes (MDS).Among them,3 patients transformed to acute myeloid leukemia following MDS.The time was 24,45 and 51 months after IST.The median following time was 42 (17-84) months.Four patients died during the following time.The median time from MDS to death was 9 (5-17) months.Among them,three patients died with infection,one died with cerebral hemorrhage.Six patients had the clinical characteristics

  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. Bone Marrow Transplantation for Severe Aplastic Anemia Secondary to Temozolomide

    OpenAIRE

    Morris, E. Brannon; Kasow, Kimberly; Reiss, Ulrike; Ellison, David; Broniscer, Alberto

    2008-01-01

    Radiotherapy (RT) and concomitant/adjuvant therapy with temozolomide (Temodar) is a common treatment regimen for children and adults with glioma. Although temozolomide is generally well tolerated with temporary myelosuppression as the primary dose-limiting toxicity, irreversible bone-marrow aplasia after treatment with temozolomide has been reported. We report the case of an adolescent patient with a high-grade glioma who, after > 2 years of event-free survival, underwent successful bone marr...

  7. Hereditary Spherocytosis Unmasked by Human Parvovirus B19 Induced Aplastic Crisis in a Family.

    Science.gov (United States)

    Alavi, Samin; Arabi, Nahid; Yazdi, Mohammad Kaji; Arzanian, Mohammad Taghi; Zohrehbandian, Farahnaz

    2015-09-01

    Human parvovirus (HPV) B19 induced aplastic crisis in a family leading to the diagnosis of hereditary spherocytosis (HS) is a very rare condition being barely reported in the literature. We herein report a 4-year-old girl, her brother, and their mother who all presented with progressive pallor and jaundice after a febrile illness. The HPV B19 was diagnosed using polymerase chain reaction (PCR) and positive serology for specific anti-HPV B19 IgM. They were further diagnosed with having HS. The clinical importance of this report is that in the case of an abrupt onset of unexplained severe anemia and jaundice, one should consider underlying hemolytic anemias mostly hereditary spherocytosis complicated by HPV B19 aplastic crisis. Herein, we report the occurrence of this condition, simultaneously in three members of a family. The distinguished feature of this report is that all affected family members developed some degrees of transient pancytopenia, not only anemia, all simultaneously in the course of their disease.

  8. Hereditary Spherocytosis Unmasked by Human Parvovirus B19 Induced Aplastic Crisis in a Family

    Directory of Open Access Journals (Sweden)

    Samin Alavi

    2015-09-01

    Full Text Available Human parvovirus (HPV B19 induced aplastic crisis in a family leading to the diagnosis of hereditary spherocytosis (HS is a very rare condition being barely reported in the literature. We herein report a 4-year-old girl, her brother, and their mother who all presented with progressive pallor and jaundice after a febrile illness. The HPV B19 was diagnosed using polymerase chain reaction (PCR and positive serology for specific anti-HPV B19 IgM. They were further diagnosed with having HS. The clinical importance of this report is that in the case of an abrupt onset of unexplained severe anemia and jaundice, one should consider underlying hemolytic anemias mostly hereditary spherocytosis complicated by HPV B19 aplastic crisis. Herein, we report the occurrence of this condition, simultaneously in three members of a family. The distinguished feature of this report is that all affected family members developed some degrees of transient pancytopenia, not only anemia, all simultaneously in the course of their disease.

  9. Aplastic

    Science.gov (United States)

    Stedman's Online Medical Dictionary. Wolters Kluwer Health, Inc. 2009. Lippincott Williams & Wilkins. Accessed May 29, 2014. Dorland's Online Medical Dictionary. Elsevier. 2013. Accessed May 29, 2014.

  10. Treatment for intractable anemia with the traditional Chinese medicines Hominis Placenta and Cervi Cornus Colla (deer antler glue

    Directory of Open Access Journals (Sweden)

    Yasuyo Hijikata

    2009-05-01

    Full Text Available Yasuyo Hijikata1, Takashi Kano2, Lu Xi31Toyodo Hijikata Clinic, Osaka, Japan; 2Kano Clinic, Osaka city, Osaka, Japan; 3Traditional Chinese Medicine Institute, Si-chuan Province, ChinaObjective: Intractable anemia, such as aplastic anemia or that presumably associated with chronic herpes virus infections, sometimes require bone marrow transplant. We investigated the use of traditional Chinese medicine (TCM for the treatment of intractable anemia. Method: Placenta Hominis (PH, steam boiled and roasted, and Cervi Cornus Colla (deer antler glue has been used in China for hundreds of years to treat anemia. After consent was obtained, we prescribed these two materials for a 74-year-old female with aplastic anemia and a 26-year-old male with presumably a virus-induced anemia. Concomitant conventional therapy was continued in both patients as prescribed by their respective attending physicians. Conclusion: Conventional therapy with steroid hormones, immunosuppressive drugs, platelet and erythrocyte transfusions were not effective in these patients. In addition, both patients suffered from serious side effects. In two patients, ingestion of Placenta Hominis and Cervi Cornus Colla with TCM prescriptions increased the platelet and enhanced the hemoglobin concentration in several months of therapy accompanied by a dramatic improvement in quality of life. The addition to conventional therapy of PH and Cervi Cornus Colla, the latter of which is very easy to obtain, may be one of the potentially advantageous choices in case of otherwise intractable anemia.Keywords: placenta, antler glue, Cervi Cornus Colla, anemia, aplastic anemia

  11. Avian anemia's

    Directory of Open Access Journals (Sweden)

    Raukar Jelena

    2005-01-01

    Full Text Available This paper deals with avian anemia's classified by MCHC/MCV and with types of anemia's. Father hematological and immunological research is needed to secure information on hematological parameters in different avian species at their earliest age. Anemia is a common clinical finding in birds because the avian erythrocyte half - life is much shorter than the mammalian. Therefore anemia should be determined as soon as possible. Researchers should standardize hematological parameters for every single avian species.

  12. 不同中性粒细胞计数阈值下极重型再生障碍性贫血免疫抑制治疗的血液学反应及生存研究%Outcomes of very severe aplastic anemia patients with different absolute neutrophil counts after frontline immnunosuppressive therapy

    Institute of Scientific and Technical Information of China (English)

    李洋; 武志洁; 赵馨; 张莉; 井丽萍; 周康; 彭广新; 叶蕾; 李园

    2016-01-01

    Objective To analyze early hematopoietic response and long-term survival of very severe aplastic anemia (VSAA) patients with different absolute neutrophil counts (ANC) after frontline immnunosuppressive therapy (IST).Methods Clinical data and outcome of 145 VSAA patients treated with rabbit antithymocyte globulin combined with cyclosporine were retrospectively analyzed.Hematopoietic responses to IST and long-term survival were statistically analyzed for VSAA patients in different ANC subgroups.Results Pre-IST ANC=0.05 × 109/L acted as the best cutoff level to predict IST response at 3,6 months.For 145 VSAA patients,early death rate was 13.4% (11/82) vs 1.6% (1/63),respectively,in the ANC≤0.05 × 109/L group and ANC>0.05 × 109/L group (P<0.05).Hematopoietic response rates to IST was 22.0% vs 54.0%(P=0.000) at 3 months,34.1% vs 63.5% (P=0.000) at 6 months;the overall five-year survival rate was only (62.5±5.4)% vs (91.4±3.7)%(P=0.000) and five-year eventfree survival rate was (42.3 ± 5.5)% vs (63.1 ± 6.5)% (P=0.003),respectively,in the ANC≤0.05 × 109/L group and ANC>0.05 × 109/L group.Conclusion VSAA patients with extremely low ANC (≤0.05 × 109/L) had high early death rate and with very low response rate to frontline IST and poor survival,so it is urgent to seek for the alternative frontline therapy that will bring faster and better outcome for these patients.%目的 分析不同中性粒细胞绝对计数(ANC)阈值下极重型再生障碍性贫血(VSAA)患者接受一线免疫抑制治疗(IST)的早期血液学反应和长期生存情况.方法 回顾性分析145例接受一线IST的VSAA患者临床资料,比较不同ANC阈值下VSAA患者的早期血液学反应、5年总生存(OS)及无病生存(EFS)率.结果 以ANC 0.05× 109/L为界观察VSAA患者IST后3、6个月的血液学反应,145例患者中ANC≤0.05×109/L者为82例,ANC>0.05×109/L者为63例,早期病死率分别为13.4%(11/82)、1.6%(1/63)(P<0

  13. The relationship of telomere and telomerase activity with outcome of aplastic anemia after immunesu pysessive therapy%端粒及端粒酶与再生障碍性贫血免疫抑制治疗疗效的关系

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    宋佳音; 邝丽萍; 王洋; 李勇华; 吴九龙; 张航; 李力; 王耀春; 蒋祖军

    2013-01-01

    Objective To observe the changes of telomere length and telomerase activity in patients with aplastic anemia (AA),and relationship with immunosuppressive therapy (IST) efficacy,to explore the pathogenesis of AA and the role of telomere length in evaluating immunosuppressive therapy efficacy.Method 71 cases of AA patients between September 2010 and March 2013 were enrolled into this study.3 ml peripheral blood specimens from this cohort of patients were collected to test the telomere length in peripheral blood mononuclear cell (PBMNC) with flow-FISH and detect telomerase activity with TRAP-PCR-ELISA method.Results Telomere length and age showed negative correlation (b=-0.387,P=0.001) in normal control,NSAA and SAA + VSAA groups,telomere length became shorter with the growth of age,and normal control group telomere length decreased along with the age growth slightly greater than the other two groups (NSAA,SAA+VSAA).Besides the effect of age on telomere length,(30.957±4.502) (29.510±5.911) no significant difference was observed between NSAA and SAA+VSAA groups (P=0.573),and NSAA,SAA+VSAA groups were significantly shorter than normal control group (51.086± 10.844) (P<0.01).Telomere length in NR group (25.357±4.848) was significantly lower than normal control group (51.086± 10.844) (P=0.005),telomere length in CR (32.808±4.685)/PR groups (30.334±4.464) compared with normal control group had no significant difference (P=0.517,P=0.254).Telomere length below 29.21% obviously decreased outcomes of IST.Telomerase activity had significant difference (x2=20.385,P<0.01).The telomerase activity had no significant difference interms of age and gender in three groups,multiple comparison found that telomerase activities in SAA + VSAA (0.324± 0.178) (P<0.01),and NSAA (0.234± 0.175)groups (P=0.002) were significantly higher than normal control group (0.107±0.083).Conclusion Telomere length of PBMNC in AA patients was significantly shortened than normal control group

  14. 人参二醇组皂苷提取物对再生障碍性贫血小鼠免疫调节作用的研究%Effects of PDS-C on immunoregulation in mice with aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    郑智茵; 尹利明; 庄海峰; 成志; 赵燕娜; 余潇苓; 高瑞兰

    2015-01-01

    Aim To observe the effects of panaxdiol saponins component ( PDS-C) extracted and isolated from Chinese ginseng herb as new Chinese patent med-icine on the promotion of hematopoiesis and the regula-tion of the immune system in treating mice models with aplastic anemia ( AA ) . Methods For preparation of immune mediated AA models, BALB/c mice were ex-posed to sublethal doses of 5. 0 Gy γ radiation, fol-lowed by transplanted lymphocytes from DBA/2 donor mice. The mice models were divided into six groups in-cluding normal control, AA model, PDS-C treated groups with lower, medium and higher dosages, cy-closporine ( CsA) as positive drug control. Both PDS-C and CsA were administered by gastrogavage for 15 days. The peripheral blood cells counts and bone mar-row pathological examination were tested, the percenta-ges of Th1/Th2/Treg cells from spleen were measured, the protein expression levels of T-bet, GATA-3 and FOXP3 transcription factors in spleen cells were detec-ted. Results Curative effect of PDS-C on treating AA mice was satisfactory. The peripheral hemoglobin, white blood cells and platelet counts in PDS-C groups with medium and higher doses were significantly higher than those in model control. Meanwhile, PDS-C ele-vated the percentages of Th2 cells and Treg cells, but decreased the percentage of Th1 cells, as well as up-regulated the GATA-3 , FOXP3 and down-regulated the T-bet protein levels. Conclusion PDS-C possesses the activities of promoting hematopoiesis obviously. It can improve marrow myelosuppression, enhance the re-covery of hematopoiestic function, and elevate the pe-ripheral blood cells counts. PDS-C also pays its immu-noregulatory efficacy though recovering from unbal-anced Th1/Th2/Treg cells in treating immune media-ted AA mice.%目的:观察自行提取分离的中药新药人参二醇组皂苷提取物( PDS-C)对再生障碍性贫血(再障)小鼠促进造血和调节免疫的作用。方法 BALB/c小鼠经亚致死量照射后输入DBA/2

  15. 比较不同重组人TPO方案联合免疫抑制治疗对重型再生障碍性贫血近期疗效的影响%Comparison of efficacy and safety of two different dose of recombinant human thrombopoietin regimens in severe aplastic anemia patients with immunosuppressive therapy

    Institute of Scientific and Technical Information of China (English)

    周康; 李洋; 李建平; 樊慧慧; 张莉; 井丽萍; 彭广新; 叶蕾; 李园

    2016-01-01

    Objective To compare the short-term therapeutic effect of different regimens combining recombinant human thrombopoietin (rhTPO) with immunosuppressive therapy (IST) applied in severe aplastic anemia (SAA).Methods The clinical data of newly diagnosed adult SAA patients treated with first-line IST,including 18 patients with rhTPO daily and 43 patients with rhTPO every other day,was analyzed retrospectively.Results There was no significant difference between the basic clinical characteristics of the patients classified in different groups.The therapeutic effect was assessed 3 months and 6 months after IST.The statistical data revealed that the overall responses (OR) were not significantly different in daily group and every other day group (3 months:50.0% vs 51.2%,P=0.934;6 months:77.8% vs 69.8%,P=0.525),while the good hematological response (CR+GPR) in SAA treated with rhTPO daily was significantly higher than that of patients treated with rhTPO every other day at 3 month after IST (38.9% vs 9.3%,P=0.011).RBC transfusion independence were not significantly different between the two groups after 4 weeks as well as 8 weeks treatment(4 weeks:22.2% vs 18.6%,P=0.736;8 weeks:55.6% vs 46.5%,P=0.519),while platelet transfusion independence in rhTPO daily treated group was significantly higher than that in every other day group (88.9% vs 48.8%,P=0.003).In addition,there were no more adverse events observed in rhTPO daily group.Conclusions It' s more effective to promote hematopoietic recovery and reduce platelet transfusion dependence when rhTPO was daily used other than used every other day.%目的 比较不同重组人TPO(rhTPO)方案联合免疫抑制剂治疗重型再生障碍性贫血(SAA)的近期疗效.方法 回顾性分析接受一线免疫抑制治疗(IST)成人SAA初诊患者资料,对比分析18例IST联合rhTPO每日1次(连续组)与43例IST联合rhTPO隔日1次(间日组)患者的疗效差异.结果 两组患者在IST前基础临床特

  16. Impact of recombinant human thrombopoietin (rhTPO) on short-term response of immunosuppressive therapy in patients with newly diagnosed acquired severe aplastic anemia%重组人血小板生成素对重型再生障碍性贫血免疫抑制治疗近期疗效的影响

    Institute of Scientific and Technical Information of China (English)

    张莉; 杨文睿; 叶蕾; 周康; 井丽萍; 李洋; 李园; 李建平; 彭广新

    2015-01-01

    Objective To evaluate the impact of recombinant human thrombopoietin (rhTPO) on short-term response of immunosuppressive therapy (IST) in patients with newly diagnosed acquired severe aplastic anemia (SAA).Methods The clinical data of forty adult acquired SAA patients,who treated with IST combined with rhTPO,were retrospective analyzed and the hematologic recovery were compared with patients by the IST alone during the same period.The factors affecting the short-term response were also analyzed.Results At 3 months after IST,both the total response rate and CR+GPR rate in rhTPO group were much higher than those in control group (75.0% vs 50.0%,P=0.022; and 17.5% vs 2.5%,P=0.025).At 6 months after IST,there was no difference of total hematologic response rate in rhTPO group and control group (77.5% vs 57.5%,P=0.058),while the CR+GPR rate was still higher in rhTPO group (45.0% vs 22.5%,P=0.033).The median time of platelet transfusion independence was much shorter in rhTPO group [33 (0-90) vs 53 (0-75) d,P=0.019].Patients in rhTPO group needed less platelets transfusion support.The median platelet count in rhTPO group was 29(4-95)× 109/L at 3 months after IST,which was much higher than that in control group [29 (4-95) × 109/L,P=0.006].There was no significant difference regarding overall survival between the two groups (100.0% vs 91.0%,P=0.276).Conclusion rhTPO is effective in promoting platelet recovery and improving the hematopoietic response for SAA patients with IST.%目的 评价重组人血小板生成素(rhTPO)对重型再生障碍性贫血(SAA)免疫抑制治疗(IST)近期疗效的影响.方法 回顾性分析IST联合rhTPO治疗40例成人SAA患者,以同期单用标准IST方案的患者为对照组,比较两组患者血液学反应及血小板恢复情况,并分析影响近期疗效的相关因素.结果 IST后3个月,rhTPO组患者血液学反应率及良好血液学反应率均明显高于对照组(75.0%对50.0%,P=0.025;17.5%对2

  17. High-dose cyclophosphamide-induced immunogenic tolerance following haploidentical allogeneic hematopoietic stem cell transplantation in severe aplastic anemia children%单倍型异基因造血干细胞移植后大剂量环磷酰胺诱导免疫耐受治疗儿童重型再生障碍性贫血

    Institute of Scientific and Technical Information of China (English)

    郭智; 童春; 刘晓东; 杨凯; 何学鹏; 张媛; 陈鹏; 楼金星; 陈惠仁

    2016-01-01

    BACKGROUND:High-dose cyclophosphamide (CTX)-induced immunogenic tolerance folowing haploidentical alogeneic hematopoietic stem cel transplantation (alo-HSCT) is developed to optimize the treatment of childhood severe aplastic anemia (SAA) using haplotype alo-HSCT, providing a theoretical basis for the clinical application. OBJECTIVE:To investigate the clinical efficacy and safety of the use of high-dose CTX folowing haploidentical alo-HSCT in SAA children. METHODS:Clinical data from 10 children with SAA undergoing haploidentical alo-HSCT at the Department of Hematology, General Hospital of Beijing Military Area from January 2013 to January 2015 were retrospectively analyzed. Pretreatment was CTX, fludarabine, Busulfex combined with anti-human lymphocyte immune globulin used for 2 consecutive days, and then 3 days after transplantation, CTX (50 mg/kg per day) was used to induce immunogenic tolerance. Combined use of cyclosporin A, methotrexate and tacrolimus functioned as a prophylaxis for graft-versus-host disease. Another 10 SAA children who underwent synchronous HLA-identical sibling HSCT served as controls. Complications and survival in children were statisticaly analyzed in the two groups. RESULTS AND CONCLUSION:In the treatment group, children were folowed up until May 2015, and the median folow-up period was 18.1 months (5-28 months). Hematopoietic reconstruction was successful in al cases, and there were three cases of graft-versus-host disease, three cases of pulmonary infection and two cases dying of pulmonary infection. In the control group, the median folow-up period was 20.7 months (6-27 months), and al the children received hematopoietic reconstruction. Additionaly, there were two cases of graft-versus-host disease, four cases of pulmonary infection, one case dying of graft-versus-host disease and one case dying of pulmonary infection in the control group. The total survival rate in each group was 80%. In summary, high-dose CTX-induced immunogenic

  18. Study on clonal evolution of monosomy 7 in patients with aplastic anemia by interphase-fluorescence in situ hybridization%应用间期荧光原位杂交研究再生障碍性贫血单体7克隆性演变

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    李英梅; 刘旭平; 李承文; 徐方运; 贡金英; 于成龙; 王建祥; 郑以州

    2010-01-01

    目的 探讨再生障碍性贫血(AA)单体7(-7)克隆的演变.方法 应用间期荧光原位杂交(FISH)技术分析81例核型正常的初诊AA患者及46例免疫抑制联合重组人粒细胞集落刺激因子(rhuG-CSF,疗程大于6个月)治疗后AA患者的-7克隆.结果 81例初诊AA患者中,11例(13.6%)-7克隆阳性,阳性细胞比例5.4%~7.6%,-7克隆阳性患者疗效及生存率与-7克隆阴性者相比差异无统计学意义(P值分别为0.481和0.865),11例阳性患者(包括5例rhuG-CSF疗程大 于6个月者)治疗后-7比例均下降至正常,中位随访时间44个月,未发现转化为骨髓增生异常综合征(MDS)或急性髓系白血病(AML)的证据;追踪随访46例AA患者的-7克隆,治疗后3~6个月均为阴性,治疗后12~15个月5例阳性,中位随访时间48个月,FISH共检测到6例-7克隆阳性,均进展为MDS或AML,5例为-7核型.FISH检出阳性克隆的时间较常规核型分析提前3~18个月;应用HSH技术回顾性分析了4例转化为MDS或AML的AA患者初诊时标本,-7克隆均为阴性.结论 部分初诊AA患者具有潜在低比例的-7克隆,但与治疗反应及最终进展为克隆性疾病无关;rhuG-CSF可能促进-7克隆扩增,长期应用rhuG-CSF治疗的AA患者需用间期FISH技术密切监测异常克隆.%Objective To explore the clonal evolution of monosomy 7 in patients with aplastic anemia (AA).Methods Monosomy 7(-7)in 81 AA patients with normal karyotype at diagnosis and 46 AA treated with innnunosuppressive therapy(IST)and more than 6 months of recombinant human granulocyte colonystimulating factor(rhuG-CSF)were detected by interphase-fluorescence in situ hybridization(FISH)retrospectively.Results There were 5.4%-7.6% of-7 cells in 11(13.6%)of 81 patients at diagnosis,the survival and response rate to IST in-7 positive patients did not differ significantly from that in-7 negative patients(P = 0.481,0.865);-7 cells disappeared after IST in all of the 11 patients including 5

  19. Long-term follow-up of malignant clonal evolution in patients with acquired aplastic anemia%获得性再生障碍性贫血恶性克隆性演变的长期随访研究

    Institute of Scientific and Technical Information of China (English)

    李星鑫; 葛美丽; 施均; 冯祥艳; 邵英起; 钱林生; 郑以州

    2011-01-01

    目的 观察获得性再生障碍性贫血(AA)治疗后演变为骨髓增生异常综合征(MDS)和(或)急性髓系白血病(AML)的发生率并分析其危险因素.方法 长期随访1991至2009年收治的1003例从患者,观察疾病演变,并分析其进展为MDS/AML的可能危险因素,包括患者性别、年龄、病因、治疗前后染色体核型变迁、疾病严重程度、治疗方案及治疗反应等.结果 1003例患者中位随访时间为62(2~423)个月,其5年总生存率为(78.0±1.0)%,共计27例转化为MDS/AML[非重型AA(NSAA)11例,重型AA(SAA)6例,超重型AA(VSAA)10例].Kaplan-Meier估计法分析1003例AA患者10年MDS/AML转化率为(4.5 ±1.0)%,VSAA组10年MDS/AML转化率[(12.8±3.5)%]显著高于NSAA组[(4.1±1.9)%,P<0.01]和SAA组[(3.5±1.4)%,P<0.01],而后二组间差异无统计学意义(P=0.616).单因素及多因素分析均显示患者年龄>40岁[RR=3.527(95%CI:1.598~7.784),P<0.01]、VSAA[RR=5.122(95%CI:2.214~11.853),P<0.01]、发病前射线、毒物、化学制剂等接触史[RR=3.401(95%CI:1.535~7.534),P<0.01]及重组人粒细胞集落刺激因子(rhuGCSF)疗程大于300 d[RR=10.782(95%CI:4.600~25.269),P<0.01]为AA转化为MDS/AML的危险因素.结论 长期随访对于评估AA患者治疗后进展为MDS/AML至关重要,随访期间应制定规范监测策略,及时发现转化的MDS/AML并尽早采取相应措施阻断其进展.%Objective To assess the incidence and risk factors for evolution of acquired aplastic anemia (AA) into myelodysplastic syndrome/acute myeloid leukemia (MDS/AML).Method A total of 1003 AA patients hospitalized in our institute hospital between January 1991 and December 2009 enrolled into this study.The incidence and risk factors for AA developing MDS/AML by the Kaplan-Meier method and Cox proportional hazards models, respectively.Results The median follow-up was 62(2 -423) months and the projected 5-year survival rate was (78.0 ± 1.0) %.Twenty-seven patients evolved to

  20. Clinical Observation of Treating 62 Patients with Severe Aplastic Anemia Failing in Immunosuppressive Therapy by Integrative Medicine%中西医结合治疗62例免疫抑制治疗无效的重型再生障碍性贫血临床观察

    Institute of Scientific and Technical Information of China (English)

    苏尔云; 方玉华; 陈辉树

    2012-01-01

    目的 探讨对免疫抑制治疗(IST)无效的重型再生障碍性贫血(SAA)的治疗方法.方法 用中西医结合治疗IST无效的SAA 62例.将治疗过程分3期:危重期用羚羊乙癸汤(羚羊角粉1.2 g 黄连6 g焦栀子12 g 生地30 g 白茅根50 g 黄柏9 g 丹皮12 g 阿胶9 g 红枣30 g 炙甘草6 g等)与二至补髓汤(女贞子120 g 旱莲草100 g 制首乌24 g 枸杞子12 g 生地90 g 黄芪60 g 当归9 g 阿胶9 g金银花30 g 莲子芯12 g等)交替服用,每日1剂,煎2次分服,同时给予丙酸睾酮50 mg隔日肌肉注射,治至好转期.发热者在羚羊乙癸汤中加生石膏60 g,知母60 g,蒲公英30 g,白英30 g,白薇30 g,三叶青15 g等.好转期用鸡血藤复方治疗(肾阳虚给予鸡血藤拯阳汤:鸡血藤100 g 黄芪60 g 红参3 g 补骨脂12 g菟丝子18 g 当归12 g 仙灵脾18 g 葫芦巴6 g 制首乌24 g 女贞子30 g 旱莲草30 g 紫河车6 g等.同时给予丙酸睾酮50 mg隔日肌肉注射.肾阴虚给予鸡血藤益精汤:女贞子100 g 旱莲草100 g 生地90 g 鸡血藤30 g 菟丝子12 g 补骨脂6 g 制首乌30 g 阿胶9 g 枸杞子9 g 丹参24 g 黄芪30 g当归6 g等)每日1剂,煎2次服.治至恢复期.恢复期逐渐减药至停药随访.结果 62例患者经6~57个月的治疗,基本治愈12例(19.4%),缓解14例(22.6%),明显进步8例(12.9%),无效28例(45.2%),总有效率54.8%.初诊时体温37.6~38.5 ℃者23例,退热(38.5 ℃者20例,退热所需时间4 h~ 5天.初诊时因IST而引起的肝、肾功能异常(ALT、AST、BUN、Cr)者26例,经中西医结合治疗2个月后,25例恢复正常.结论 IST无效的SAA的治疗有其特殊性,中西医结合分期论治符合IST无效的SAA的病理生理,改善和保护骨髓造血微环境可能是治疗IST无效的SAA的关键所在.而中药的抗菌消炎作用有阻断病情恶化的趋势.%Objective To explore treatment methods for patients with severe aplastic anemia (SAA) failing in immunosuppressive therapy (IST). Methods Totally 62 SAA

  1. Fanconi anemia and radiation

    Energy Technology Data Exchange (ETDEWEB)

    Nakamura, Asako; Komatsu, Kenshi [Hiroshima Univ. (Japan). Research Inst. for Nuclear Medicine and Biology

    1999-09-01

    Aplastic Fanconi anemia (FA) accompanying malformation was firstly reported in 1927. This review concerns the recent findings on FA. FA belongs to the chromosomal instability syndrome and its detailed molecular mechanism is still unknown. The disease has been defined to be highly sensitive to radiation, however, which is quite an important problem since irradiation with a large dose of radiation is required before its radical treatment (bone marrow transplantation). FA cells are also mitomycin C-sensitive and FA patients are said to be the mosaic of the sensitive and normal cells. This enables to classify FA into 8 types of A-H groups, whose genotypes (FAA-FAH, FANCA-FANCH) are becoming clear. However, the intracellular function of the FANC-expressed protein, although known to form a big complex, is not elucidated yet. There is an abnormality in DNA processing such as re-linkage of the double strand-broken DNA in FA cells. FA causal gene FANCG is found identical to XRCC9 which is associated to high sensitivity to radiation. Analysis of FANC genes will provide useful findings on molecular mechanism of DNA-repair. (K.H.)

  2. 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 ...

  3. Analysis of risk factors for preeclampsia in pregnancies complicated with chronic aplastic anemia%妊娠合并慢性再生障碍性贫血患者并发子痫前期的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    张超; 尹璐瑶; 梁梅英; 王山米; 张晓红; 王建六

    2012-01-01

    Objective To investigate the risk factors for preeclampsia (PE) in pregnancies complicated with chronic aplastic anemia ( CAA ) by analyzing the obstetric management and pregnancy outcome.Methods Retrospectively review the clinical data including the obstetric management,the laboratory findings and the pregnancy outcome of 41 pregnant women complicated with CAA,all of whom were hospitalized in Peking University People's Hospital from May 2002 to February 2011.Multiple logistic regression was used to explore the risk factors associated with PE.Results ( 1 ) Twenty-eight patients were diagnosed before conception while 13 were diagnosed during gestation.Eleven patients including all the 7 who were categorized as severe CAA presented with mild bleeding in the third trimester.( 2 ) The medians of white blood cell counts,hemoglobin concentrations and platelet counts were 5.0 × 109/L,66.0 g/L and 12.0 × 109/L respectively.(3) The obstetric management consisted of strict assessment, intensive surveillance and follow-up,appropriate supportive measures,timely recognition of complications,and delivery when necessary.Twenty-one patients received supportive transfusions.Ten patients developed PE,all of whom were diagnosed as severe PE( SPE).Twelve patients suffered postpartum hemorrhage,and 3 of them had blood loss more than 1000 mL All were conservatively treated in success.(4) The median gestational age of delivery was 37 weeks.Sixteen cases delivered before 37 weeks and 5 delivered before 34 weeks.Two patients developed SPE at 29 weeks and 30 weeks respectively,and both of the neonates died for severe asphyxia.The birth weight of the live neonates ranged from 1500 to 3660 g.(5) The postpartum follow-up period ranged from 6 months to 7 years.Thirty-three patients got improvement without dependence on transfusions.Four achieved no remission and still needed intermittent transfusions.Four were lost in followup.(6) Significant differences were found in the bleeding tendency

  4. Cytogenetic profile of aplastic anaemia in Indian children

    Directory of Open Access Journals (Sweden)

    Vineeta Gupta

    2013-01-01

    Interpretation & conclusions: Five (11.9% patients with acquired aplastic anaemia had chromosomal abnormalities. Trisomy was found to be the commonest abnormality. Cytogenetic abnormalities may be significant in acquired aplastic anaemia although further studies on a large sample are required to confirm the findings.

  5. Immune hemolytic anemia

    Science.gov (United States)

    Anemia - immune hemolytic; Autoimmune hemolytic anemia (AIHA) ... for no reason, the condition is called idiopathic autoimmune hemolytic anemia . The antibodies may also be caused by: Complication ...

  6. Pernicious Anemia

    Science.gov (United States)

    ... well, and live normal lives. Without treatment, pernicious anemia can lead to serious problems with the heart, nerves, and other parts of the body. Some of ... INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  7. HLA相合无关供者造血干细胞移植联合脐带间充质干细胞输注治疗儿童重型再生障碍性贫血19例疗效及安全性研究%Efficacy and security of matched unrelated donor hematopoietic stem cell transplant with transfusion of multipotent mesenchymal cells in pediatric severe aplastic anemia

    Institute of Scientific and Technical Information of China (English)

    王玲; 王恒湘; 朱玲; 郑晓丽; 王志东; 闫洪敏; 丁丽; 韩冬梅

    2016-01-01

    目的 探讨HLA相合无关供者造血干细胞移植(MUD-HSCT)联合脐带间充质干细胞(MSC)输注治疗儿童重型再生障碍性贫血(SAA)的疗效及安全性.方法 回顾性分析19例行MUD-HSCT联合MSC治疗的儿童SAA患者临床资料,观察移植后造血重建及移植相关并发症.结果 19例患儿移植后均获得迅速造血重建,粒细胞中位植入时间为12(9~21)d,血小板中位植入时间为14(8~24)d,1例患儿于移植后4个月出现继发植入失败.9例患儿发生Ⅰ度急性移植物抗宿主病(aGVHD),1例发生Ⅲ度aGVHD并于移植后6个月发生广泛型慢性移植物抗宿主病.CMV阳性15例,出血性膀胱炎2例.10例患儿移植后出现不同程度、不同部位的感染.中位随访时间为27(8~70)个月,19例患儿截至随访终点均无病存活,其中l例患儿于移植后4个月发生淋巴细胞增殖性疾病,接受利妥昔单抗联合化疗治疗后发生继发性植入失败,后接受以父亲为供者的单倍体造血干细胞移植成功植入.结论 MUD-HSCT联合MSC输注治疗儿童SAA是安全有效的.%Objective To observe the efficacy of matched unrelated donor hematopoietic stem cell transplant (HSCT) with transfusion of multipotent mesenchymal cells (MSC) in pediatric severe aplastic anemia (SAA).Methods 19 children with SAA received matched unrelated donor HSCT with MSC,and the hematopoietic recovery and transplant-associated complications of these children were monitored.Results All patients achieved rapid hematopoietic reconstruction after HSCT,and the median durations to neutrophil and platelet recovery were 12 (9-21) days and 14 (8-24) days respectively,but delayed rejection occurred in one case four months after HSCT.9 cases developed grade Ⅰ acute graft-versus-host (aGVHD),and one case grade Ⅲ aGVHD and diffuse chronic graft-versus-host.Cytomegalovirus viremias were observed in 15 patients.2 cases developed hemorrhagic cystitis,10 children experienced infections

  8. 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 ...

  9. Anemia (For Teens)

    Science.gov (United States)

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Anemia KidsHealth > For Teens > Anemia Print A A A ... Getting Enough Iron en español Anemia What Is Anemia? Lots of teens are tired. With all the ...

  10. Anemia (For Teens)

    Science.gov (United States)

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Anemia KidsHealth > For Teens > Anemia A A A What's ... Getting Enough Iron en español Anemia What Is Anemia? Lots of teens are tired. With all the ...

  11. Anemia (For Parents)

    Science.gov (United States)

    ... Old Feeding Your 1- to 2-Year-Old Anemia KidsHealth > For Parents > Anemia Print A A A ... Preventing Iron-Deficiency Anemia en español Anemia About Anemia Anemia happens when the level of healthy red ...

  12. Orofacial manifestations of hematological disorders: Anemia and hemostatic disorders

    Directory of Open Access Journals (Sweden)

    Titilope A Adeyemo

    2011-01-01

    Full Text Available The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of hemostasis. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial manifestations AND disorders of haemostasis. The Boolean operator "AND" was used to combine and narrow the searches. Anemic disorders associated with orofacial signs and symptoms include iron deficiency anemia, Plummer-Vinson syndrome, megaloblastic anemia, sickle cell anemia, thalassaemia and aplastic anemia. The manifestations include conjunctiva and facial pallor, atrophic glossitis, angular stomatitis, dysphagia, magenta tongue, midfacial overgrowth, osteoclerosis, osteomyelitis and paraesthesia/anesthesia of the mental nerve. Orofacial petechiae, conjunctivae hemorrhage, nose-bleeding, spontaneous and post-traumatic gingival hemorrhage and prolonged post-extraction bleeding are common orofacial manifestations of inherited hemostatic disorders such as von Willebrand′s disease and hemophilia. A wide array of anemic and hemostatic disorders encountered in internal medicine has manifestations in the oral cavity and the facial region. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself.

  13. Hemolytic Anemia

    Science.gov (United States)

    ... may require ongoing treatment. Acquired forms of hemolytic anemia may go away if the cause of the condition is found and corrected. Rate This Content: NEXT >> Updated: March 21, 2014 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  14. Anemia in Pregnancy

    OpenAIRE

    Umran Kucukgoz Gulec; Fatma Tuncay Ozgunen; Ismail Cuneyt Evruke; Suleyman Cansun Demir

    2013-01-01

    Iron deficiency anemia (IDA) is the most frequent form of anemia in pregnant women. Folic acid, vitamin B12 deficiency, and hemoglobinopathies are other causes of anemia in pregnancy. Finding the underlying cause are crucial to the management of the anemia. Anemia is defined as hemoglobin of

  15. 地拉罗司对伴有铁过载的再生障碍性贫血患者的祛铁疗效及安全性--一项单臂、多中心、前瞻性临床研究%Efficacy and safety of deferasirox in aplastic anemia patients with iron overload:a single arm, multi-center, prospective study in China

    Institute of Scientific and Technical Information of China (English)

    施均; 全日成; 郑春梅; 肖海燕; 胡明辉; 胡令彦; 刘锋; 周永明; 郑以州; 张凤奎; 常红; 张莉; 邵英起; 聂能; 张静; 黄金波; 张丽; 唐旭东

    2016-01-01

    Objective To explore the efficacy and safety of deferasirox in aplastic anemia(AA) patients with iron overload. Methods A single arm, multi-center, prospective, open-label study was conducted to evaluate absolute change in serum ferritin(SF)from baseline to 12 months of deferasirox administration, initially at a dose of 20 mg · kg-1 · d-1, and the safety in 64 AA patients with iron overload. Results All patients started their deferasirox treatment with a daily dose of 20 mg · kg-1 · d-1. The mean actual dose was(18.6 ± 3.60)mg · kg-1 · d-1. The median SF decreased from 4 924(2 718-6 765)μg/L at baseline (n=64) to 3 036(1 474-5 551)μg/L at 12 months (n=23) with the percentage change from baseline as 38%. A median SF decrease of 651(126-2 125)μg/L was observed at the end of study in 23 patients who completed 12 months’treatment, the median SF level decreased by 1 167(580-4 806)μg/L[5 271(3 420-8 278)μg/L at baseline;3 036(1 474-5 551)μg/L after 12 months’treatment;the percentage change from baseline as 42%]after 12 months of deferasirox treatment. The most common adverse events (AEs)were increased serum creatinine levels(40.98%), gastrointestinal discomfort(40.98%), elevated liver transaminase(ALT:21.31%;AST:13.11%)and proteinuria(24.59%). The increased serum creatinine levels were reversible and non-progressive. Of 38 patients with concomitant cyclosporine use, 12(31.8%) patients had two consecutive values>ULN, 10(26.3%)patients had two consecutive values>1.33 baseline values, but only 1(2.6%)patient’s serum creatinine increased more than 1.33 baseline values and exceeded ULN. For both AST and ALT, no patients experienced two post-baseline values >5 × ULN or >10 × ULN during the whole study. In AA patients with low baseline PLT count(less than 50 × 109/L), there was no decrease for median PLT level during 12 months’treatment period. Conclusions AA patients with iron overload could achieve satisfactory efficacy of iron chelation by deferasirox

  16. Treatment of severe aplastic anemia with intensified immunosuppressive therapy and two different regimens with recombinant human granulocyte colony-stimulating factor: a retrospective study based on long-term follow-up%强化免疫抑制疗法联合不同方案G-CSF治疗重型再生障碍性贫血患者的长期随访研究

    Institute of Scientific and Technical Information of China (English)

    李英梅; 李星鑫; 葛美丽; 施均; 钱林生; 王建祥; 郑以州

    2010-01-01

    目的 比较强化免疫抑制疗法(IIST)联合两种不同方案重组人粒细胞集落刺激因子(rhG-CSF)治疗重型再生障碍性贫血(SAA)患者的有效性和安全性.方法 回顾性分析1994年3月至2007年12月lIST联合rhG-CSF治疗的176例SAA患者资料.方案A:96例患者IIST后1个月始用rhG-CSF,300μg/次,每周3次、2次、1次各用1个月;方案B:80例患者IIST前始用rhG-CSF 5μg·kg-1·d-1,直至造血功能出现恢复.比较两组的治疗反应、感染相关死亡率和克隆性演变发生率.结果 ①B组患者早期治疗反应率(67.5%)显著高于A组(37.5%)(P<0.01),且其获早期治疗反应时间明显提前.B组患者IIST后4个月内感染相关死亡率(6.3%)显著低于A组(16.7%)(P=0.034).B组患者4年总体生存率[(77.7±4.9)%]显著高于A组[(57.2±5.1)%](P=0.006).②IIST后4个月无效的难治性SAA患者,A组者停用rhG-CSF,B组者继续应用,两组12个月疗效、感染相关死亡率及总体生存率差异均无统计学意义(P值分别为0.066、0.296、0.288),但B组患者转化为骨髓增生异常综合征/急性髓系白血病(MDS/AML)风险显著增高.③多因素分析发现疾病严重程度(RR=1.922,P=0.010)、是否有早期治疗反应(RR=5.749,P<0.01)为独立预后影响因素,且SAA转化为MDS/AML仅与rhG-CSF疗程相关(RR=1.004,P=0.017).结论 早期足量应用rhG-CSF联合IIST有助于造血功能恢复,并降低其感染相关死亡率,但延长rhG-CSF疗程并不能进一步提高难治性SAA患者的远期疗效,且增加其转化为MDS/AML的危险性.%Objective To compare the efficacy and safety of two different regimens with recombinant human granulocyte colony-stimulating factor (rhG-CSF) combined with intensified immunosuppressive therapy (IIST) in severe aplastic anemia (SAA). Methods Retrospectively analyzed 176 SAA treated with IIST and rhG-CSF in our hospital from March 1994 to December 2007. Regimen A (Group A, n =96) , rhG-CSF 300 (μg/d was initiated on day 31

  17. [Sideroblastic anemias].

    Science.gov (United States)

    Matthes, T

    2006-01-01

    Sideroblastic anemias are a heterogenous group of disorders characterized by the presence of sideroblasts in the bone marrow aspirate. Current classification schemes distinguish between diseases of the heme synthesis pathway and diseases of other mitochondrial pathways which can either be of primary origin (defects in mitochondrial DNA) or of secondary origin (defects in nuclear DNA). Although several distinct hereditary forms exist, sideroblastic anemias are most frequently acquired diseases and belong to the group of myelodysplastic syndromes with the propensity to develop into overt leukemia. Treatment is mainly supportive (vitamins, blood transfusions, cytokines) and only rarely are bone marrow transplantations performed. The molecular defects of a few hereditary forms have already been elucidated, but the genes involved in the acquired forms are still largely unknown.

  18. [A regenerative anemia in infants: 2 cases of Pearson´s syndrome].

    Science.gov (United States)

    Martínez de Zabarte Fernández, José M; Rodríguez-Vigil Iturrate, Carmen; Martínez Faci, Cristina; García Jiménez, Inmaculada; Murillo Sanjuan, Laura; Muñoz Mellado, Ascensión

    2017-02-01

    Anemia is very common in infants. Although its causes are usually not severe and treatable, proper etiologic diagnosis should be established. When anemia is non-regenerative, it can be caused by aplastic anemia, myelodysplastic syndrome, bone marrow infiltration or hematopoietic factors deficiencies. Another possible cause is Pearson's syndrome, a rare mitochondrial disease that causes non-regenerative anemia associated with other cytopenias, pancreatic insufficiency, lactic acidosis and great variability in clinical presentation conditioned by heteroplasmy. It is characteristic to find in bone marrow studies variable vacuolization in erythroblastic progenitors and ring sideroblasts. The diagnosis is established by genetic study of mitochondrial deoxyribonucleic acid performed by Southern blot analysis (complete mitochondrial deoxyribonucleic acid amplification by polymerase chain reaction -long), obtaining 70-80% deletion of 4977 bp (NMD 8343-13459). There is no curative therapy and support treatment is the only available nowadays. Death is frequent in early years of life.

  19. Clinical Evaluation of Anemia in Geriatric Patients - A Cross Sectional Study Conducted At Tertiary Care Hospital

    Directory of Open Access Journals (Sweden)

    Sfurti Mann, Amit Kumar, Shiv Kumar Singh, Surbhi Katyal, Gaurav Chopra, Sanjeev Kumar Varma

    2014-01-01

    Methods: After taking informed written consent, patients were subjected to a detailed history, thorough clinical examination and various relevant investigations including bone marrow examination and radiological means. Result: 42 patients (70% were male and 18 patients (30% were female. Maximum number of patients, 24 (40% were in the age group 65-69 years. Out of which 15 (62.5% were male and 9 (37.5% were female. Conclusion: Among all the patients (irrespective of age groups and types of anemia, ACD was found to be most common (41.67%, followed by IDA (35%, MDS (5%, Megaloblastic anemia (3.34%, myelofibrosis and haemolytic anemia (3.34% each and aplastic anemia (1.67%.

  20. 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) ...

  1. 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 ...

  2. Anemia and Pregnancy

    Science.gov (United States)

    ... Advocacy Toolkit Home For Patients Blood Disorders Anemia 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 ...

  3. Cooley's Anemia Foundation

    Science.gov (United States)

    ... role in their lives. Welcome to the Cooley's Anemia Foundation Website The Cooley's Anemia Foundation is dedicated to serving people afflicted with ... major form of this genetic blood disease, Cooley's anemia/thalassemia major. Our mission is advancing the treatment ...

  4. Anemia - B12 deficiency

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000574.htm Vitamin B12 deficiency anemia To use the sharing features on ... tissues. There are many types of anemia. Vitamin B12 deficiency anemia is a low red blood cell ...

  5. Vitamin Deficiency Anemia

    Science.gov (United States)

    Vitamin deficiency anemia Overview By Mayo Clinic Staff Vitamin deficiency anemia is a lack of healthy red ... you have lower than normal amounts of certain vitamins. Vitamins linked to vitamin deficiency anemia include folate, ...

  6. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Events Spokespeople Email Alerts E-Newsletters About NHLBI Organization NHLBI Director Budget, Planning, & Legislative Advisory Committees Jobs ... food. Overview Iron-deficiency anemia is a common type of anemia . The term "anemia" usually refers to ...

  7. Clinical heterogeneity and chromosome breakage in Iranian patients suspicious of Fanconi anemia

    Directory of Open Access Journals (Sweden)

    Ghasemi Firoozabadi S

    2007-10-01

    Full Text Available Background: Fanconi anemia (FA is a rare autosomal recessive disorder characterized by short stature, skeletal anomalies, increased incidence of solid tumors and leukemia, and bone marrow failure (aplastic anemia. FA has been reported in all races and ethnic groups and affects men and women in an equal proportion. The frequency of FA has been estimated at approximately 1 per 360,000 live births. In some populations, including Ashkenazi Jews, Turks, Saudi Arabians and Iranians, this frequency appears to be higher, probably as a result of the founder effect and consanguineous marriage. Because of extensive genetic and clinical heterogeneity (the age of onset, clinical manifestations and survival, diagnosis of FA on the basis of clinical data alone is unreliable and its molecular diagnosis is difficult. The diagnosis of FA exploits the hypersensitivity of FA lymphocytes and fibroblasts to bifunctional alkylating agents such as mitomycin C (MMC, diepoxybutane (DEB and nitrogen mustard and differentiates it from idiopathic aplastic anemia. In this study, in addition to the patients' clinical profiles, a cytogenetic test using MMC was implemented for an accurate diagnosis of Fanconi anemia.Methods: In this study, the lymphocytes of 20 patients referred for FA, and those of their normal sex-matched controls, were treated with three different concentrations of mitomycin C (20, 30, 40 ng/ml. Slides were prepared and solid stained. In order to determine the number and kind of chromosome abnormalities, 50 metaphase spreads from each culture were analyzed. Clinical information was obtained from patient files.Results: Five patients manifested increased chromosome breakage with MMC, confirming the FA diagnosis. Two different concentrations of MMC (30, 40 ng/ml were most effective.Conclusion: The chromosomal breakage test is important for the accurate diagnosis of Fanconi anemia. DNA crosslinking agents used to treat idiopathic aplastic anemia may be

  8. Hemoglobin C, S-C, and E Diseases

    Science.gov (United States)

    ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ... Anemia Vitamin Deficiency Anemia Anemia of Chronic Disease Aplastic Anemia Autoimmune Hemolytic Anemia Sickle Cell Disease Hemoglobin C, S- ...

  9. Anemia in pregnancy.

    Science.gov (United States)

    Horowitz, Kari M; Ingardia, Charles J; Borgida, Adam F

    2013-06-01

    Hemodynamic changes occur in pregnancy to prepare for expected blood loss at delivery. Physiologic anemia occurs in pregnancy because plasma volume increases more quickly than red cell mass. Anemia is most commonly classified as microcytic, normocytic, or macrocytic. Iron deficiency anemia accounts for 75% of all anemias in pregnancy. Oral iron supplementation is the recommended treatment of iron deficiency anemia in pregnancy. Parenteral iron and erythropoietin can also be used in severe or refractory cases. Outcomes and treatments for other forms of inherited and acquired anemias in pregnancy vary by disease, and include nutritional supplementation, corticosteroids, supportive transfusions, and splenectomy.

  10. 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 ... red blood cells. This leads to anemia. The sickle cells also get stuck in blood vessels, blocking blood ...

  11. 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.

  12. Anemia in the Newborn

    Science.gov (United States)

    ... Doctor About Emotional Struggles Additional Content Medical News Anemia in the Newborn By Arthur E. Kopelman, MD, ... of Prematurity Necrotizing Enterocolitis (NEC) Jaundice in Newborns Anemia in the Newborn Polycythemia in the Newborn Thyroid ...

  13. [Common anemias in neonatology].

    Science.gov (United States)

    Humbert, J; Wacker, P

    1999-01-28

    We describe the four most common groups of neonatal anemia and their treatments, with particular emphasis on erythropoietin therapy. The hemolytic anemias include the ABO incompatibility (much more frequent, nowadays, than the Rh incompatibility, which has nearly disappeared following the use of anti-D immunoglobulin in postpartum Rh-negative mothers), hereditary spherocytosis and G-6-PD deficiency. Among hypoplastic anemias, that caused by Parvovirus B19 predominates, by far, over Diamond-Blackfan anemia, alpha-thalassemia and the rare sideroblastic anemias. "Hemorrhagic" anemias occur during twin-to-twin transfusions, or during feto-maternal transfusions. Finally, the multifactorial anemia of prematurity develops principally as a result of the rapid expansion of the blood volume in this group of patients. Erythropoietin therapy, often at doses much higher than those used in the adult, should be seriously considered in most cases of non-hypoplastic neonatal anemias, to minimise maximally the use of transfusions.

  14. The Anemias of Athletes.

    Science.gov (United States)

    Eichner, Edward R.

    1986-01-01

    Diagnosing anemia in athletes is complicated because athletes normally have a pseudoanemia that needs no treatment. Athletes, however, can develop anemia from iron deficiency or footstrike hemolysis, which require diagnosis and treatment. (Author/MT)

  15. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... or an inability to absorb enough iron from food. Overview Iron-deficiency anemia is a common type ... of the condition. Treatments may include dietary changes, medicines, and surgery. Severe iron-deficiency anemia may require ...

  16. Megaloblastic anemia in pregnancy.

    Science.gov (United States)

    Campbell, B A

    1995-09-01

    Megaloblastic anemia is one of the acquired nutritional anemias that may complicate pregnancy. It is most often secondary to folic acid deficiency because folate requirements are increased during gestation. When the diagnosis of megaloblastic anemia is confirmed, appropriate therapy will initiate a rapid reversal of the anemia process. Because of the association between neural tube defects and folate deficiency, it is recommended that women of reproductive age take folic acid supplementation.

  17. 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 ...

  18. Iron deficiency anemia

    Science.gov (United States)

    Anemia - iron deficiency ... iron from old red blood cells. Iron deficiency anemia develops when your body's iron stores run low. ... You may have no symptoms if the anemia is mild. Most of the time, ... slowly. Symptoms may include: Feeling weak or tired more often ...

  19. Iron-Deficiency Anemia

    Science.gov (United States)

    ... 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 ...

  20. Your Guide to Anemia

    Science.gov (United States)

    ... 19 Who Is At Risk for Iron-Deficiency Anemia? Infants and children. Getting enough iron is essential for normal growth ... checkup to see whether they also might have anemia. If you have children or teens who have anemia, talk to them ...

  1. 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 ...

  2. Fungal arthritis of the hip in patient with aplastic anaemia.

    Science.gov (United States)

    Le Calloch, Ronan; Ianotto, Jean-Christophe; Guillerm, Gaëlle; Tonnelier, Jean Marie

    2013-08-13

    Aplastic anaemia is a rare and serious disease characterised by severe immunosuppression due to prolonged neutropenia and the use of immunosuppressants such as corticosteroids, cyclosporine and antithymocyte globulin. Candida species are pathogens of low virulence colonising the skin and the digestive tract of many healthy individuals. Nonetheless, the incidence of invasive candidal infection is increasing. The widespread use of central intravascular catheters, invasive procedures, broad-spectrum antibiotics and immunosuppresion predisposes patients to these infections. Eye, skin, cardiac, liver, spleen and brain infection are the most common sites of invasive candidiasis. Bone and joint infections are less frequent and Candida hip septic arthritis is extremely rare. We present here a patient treated for aplastic anaemia, who developed fungal arthritis of the hip and systemic candidaemia.

  3. AN UNUSUAL CASE OF ASYMPTOMATIC APLASTIC RENAL DYSPLASIA

    Institute of Scientific and Technical Information of China (English)

    LouisTsun-CheungChow; Wing-HingChow

    1995-01-01

    The postmortem examination of a 59-year-old man who had suffered from acute abdominal pain for two days revealed that the cause of death was peritonitis secondary to perforated peptic ulceration. During examination,it was found that the urinary tract was abnormal. Here,we report a postmortem finding of aplastic renal dysplasia of the kidney and malformation of Lhe urinary tract.

  4. Combination Chemotherapy and Donor Stem Cell Transplant in Treating Patients With Aplastic Anemia or Hematologic Cancer

    Science.gov (United States)

    2017-03-28

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Diseases; Nonmalignant Neoplasm; Unspecified Adult Solid Tumor, Protocol Specific; Unspecified Childhood Solid Tumor, Protocol Specific

  5. Aplastic anemia associated with dyskeratosis congenita treated with antilymphocyte globulin and cyclosporine: a case report

    Institute of Scientific and Technical Information of China (English)

    Hsiu-Mei Huang; Wen-Liang Yu; Yu-Lun Huang; Wei-Shiou Hwang; Chao-Jung Tsao; Hsiao-Sheng Liu; Guan-Cheng Huang

    2005-01-01

    @@ Dyskeratosis congenita (DC) is a severe inherited disease characterized by a triad of clinical manifestations including abnormal skin pigmentation, nail dystrophy, and mucosal leukoplakia.1 Bone marrow failure is the principal cause of early mortality, together with an increased predisposition to malignancy and fatal pulmonary complications. According to the dyskeratosis congenita registry, a peripheral blood cytopenia of one or more lineages is reported in 93% of patients, with 51% developing pancytopenia before the age of 10 years.2 In patients with DC, bone marrow failure or bone marrow failure treatment-associated complications account for 67% of total mortality.3 Therefore, management of bone marrow failure syndrome is crucial in patients with DC.

  6. Methemoglobinemia in Young Patients With Hematologic Cancer or Aplastic Anemia Treated With Dapsone

    Science.gov (United States)

    2010-11-04

    Chronic Myeloproliferative Disorders; Leukemia; Lymphoma; Lymphoproliferative Disorder; Methemoglobinemia; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Nonmalignant Neoplasm

  7. Anemia in Chronic Kidney Disease

    Science.gov (United States)

    ... High Blood Pressure Heart Disease Mineral & Bone Disorder Anemia in Chronic Kidney Disease What is anemia? Anemia is a condition in which the body ... function as well as they should. How is anemia related to chronic kidney disease? Anemia commonly occurs ...

  8. Laboratory Evaluation of Anemia

    OpenAIRE

    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...

  9. Iron deficiency anemia

    OpenAIRE

    Naigamwalla, Dinaz Z.; Webb, Jinelle A.; Giger, Urs

    2012-01-01

    Iron is essential to virtually all living organisms and is integral to multiple metabolic functions. The most important function is oxygen transport in hemoglobin. Iron deficiency anemia in dogs and cats is usually caused by chronic blood loss and can be discovered incidentally as animals may have adapted to the anemia. Severe iron deficiency is characterized by a microcytic, hypochromic, potentially severe anemia with a variable regenerative response. Iron metabolism and homeostasis will be ...

  10. 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 ...

  11. 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 ...

  12. Visual and quantitative approach to bone marrow foci of increased glucose uptake on PET/CT in a case of aplastic anaemia

    Energy Technology Data Exchange (ETDEWEB)

    Cicone, F. [Sant' Andrea Hospital, Univ. La Sapienza, Rome (Italy). Nuclear Medicine Dept.; Centre Hospitalier Univ. Vaudois (Switzerland). Nuclear Medicine; Lausanne Univ. (Switzerland); Stalder, M. [Institut Central des Hopitaux Valaisans, Sion (Switzerland). Service of Hematology; Cairoli, A. [Centre Hospitalier Univ. Vaudois (Switzerland). Service of Hematology; Lausanne Univ. (Switzerland); Bischof Delaloye, A.; Prior, J.O. [Centre Hospitalier Univ. Vaudois (Switzerland). Nuclear Medicine; Lausanne Univ. (Switzerland); Geiger, D.

    2010-07-01

    This case report shows the clinical impact of a FDG-PET/CT in the assessment of bone marrow (BM) of a patient with aplastic anemia. The feasibility of a quantitative approach to BM intensities on FDG-PET is also discussed. In the authors' opinion, a deeper understanding of the factors that might independently affect FDG uptake and the definition of normal ranges of BM SUV (standardized uptake value) might help to interpret PET/CT images. Further research is needed to understand the physio-pathological basis of FDG uptake in BM and the potential value of its quantification. The analysis of the bone marrow on PET/CT is an interesting field of research. A PET/CT scan contributed to differential diagnosis in a patient with suspected bone marrow aplasia for guiding bone marrow biopsies.

  13. Inborn anemias in mice

    Energy Technology Data Exchange (ETDEWEB)

    Bernstein, S.E.; Barker, J.E.; Russell, E.S.

    1981-06-01

    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 ..cap alpha..-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.

  14. Anemia in Pregnancy

    Directory of Open Access Journals (Sweden)

    Umran Kucukgoz Gulec

    2013-06-01

    Full Text Available Iron deficiency anemia (IDA is the most frequent form of anemia in pregnant women. Folic acid, vitamin B12 deficiency, and hemoglobinopathies are other causes of anemia in pregnancy. Finding the underlying cause are crucial to the management of the anemia. Anemia is defined as hemoglobin of <11 g/dl in the first and third trimester and <10.5 g/dl in second trimester. According to the literature, anemia, particularly severe anemia (Hb<7g/dl is associated with increased risk of maternal and perinatal mortality and morbidity, and long term adverse effects in the newborn. The association of hemoglobin levels to perinatal outcome has been shown to be U shaped with both high and low hemoglobin levels being associated adverse perinatal outcome such as low birth weight, increased stillbirths. Anemia in pregnancy is a major public health problem. Ideally a woman should have adequate iron stores when she conceives, in order meet to additional requirements of pregnancy. This review focuses on the occurrence, types, maternal and perinatal outcomes, prevention and treatment of anemia during pregnancy. [Archives Medical Review Journal 2013; 22(3.000: 300-316

  15. Anemia in the Elderly

    OpenAIRE

    Sparling, Terence G.

    2013-01-01

    As the population ages, increasing attention has become focused on the prevalence of anemia in elderly individuals. Anemia occurs in more than 10% of individuals who are older than the age of 65 years, and it increases to more than 50% in individuals who are older than the age of 80 years. Although the anemia is typically mild and unlikely to result in symptoms, it is uniformly associated with increased morbidity and mortality as assessed in large cohort studies. Anemia is an independent pred...

  16. Xiv conferencia : anemias

    OpenAIRE

    Paredes Manrique, Raúl; Camacho Gamba, Jorge

    2012-01-01

    La anemia es la disminución por debajo de lo normal de la Hb. o del número de globulos rojos o del hematocrito, pero es difícil encontrar un cuadro clínico que corresponda a la realidad de la anemia en el cual no estén descendidos los tres elementos.

  17. Living with Anemia

    Science.gov (United States)

    ... by an inherited or chronic disease or trauma. Anemia and Children/Teens Infants and young children have a greater ... birth-weight babies often are watched closely for anemia. Talk with your child's doctor if you're feeding your infant breast ...

  18. 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 ...

  19. 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.

  20. [Aplastic crisis due to parvovirus B19 and Epstein-Barr virus in a patient with hereditary spherocytosis].

    Science.gov (United States)

    Leoz Gordillo, I; Pérez Suárez, E

    2015-01-01

    Anemic syndrome in childhood requires a diagnosis and urgent treatment guided by systematic protocols that can avoid unnecessary additional testing. The case of a 4 year-old girl with fatigue and intermittent fever of 7 days duration, accompanied by abdominal pain is presented. She had regular general health status, with mucocutaneous jaundice, a grade III/VI/iv murmur, and painful abdomen with hepatosplenomegaly. The blood analysis showed a hypo-regenerative anemia with increased LDH and indirect bilirubin. The Coombs Test was negative, with spherocytes being observed in the peripheral blood smear. The IgM and IgG were positive for parvovirus B19 IgM and Epstein Barr virus, leading to the diagnosis of aplastic crisis in a patient with hereditary spherocytosis. No specific treatment was required. Under the suspicion of anemic syndrome in emergencies, the ABCDE sequence must be followed. Through the history, physical examination and basic laboratory tests, an initial diagnostic approach can be made. Specific etiological tests should be based on this first study.

  1. Anemia in Antiretroviral Naïve HIV/AIDS Patients: A Study from Eastern India

    Directory of Open Access Journals (Sweden)

    Arindam Pande

    2012-01-01

    Full Text Available Background: Hematological manifestations are common throughout the course of HIV infection. Impact of anemia is the most significant among them. The present study was undertaken to evaluate the etiologies underlying anemia in HIV/AIDS. Methods This was a non randomized cross sectional observational study conducted in a tertiary care hospital of India over a period of 2 years. One hundred and fifty HIV patients were screened. Thorough clinical and laboratory evaluation was done in 50 randomly selected anemic cases. Results: Proper etiological diagnosis could be reached in 46 patients. Among them correlation between Hb% and CD4 count was statistically insignificant (p = 0.074, r = 0.47 whereas it was significant with absolute lymphocyte and CD4 count (p = 0.006, r = 0.41. There was better correlation of bone marrow iron status with percent saturation of transferrin (p = 0.003, r = 0.54 than with serum ferritin (p = 0.055, r = 0.09. Bone marrow iron status did not have any relationship with CD4 count. Anemia of chronic disease was the commonest etiology (37% followed by HIV related myelodysplastic syndrome (31%, iron deficiency anemia (13%, bone marrow suppression due to direct involvement by some infective process (7%. Aplastic anemia, multiple myeloma, Hodgkin’s disease, pure red cell aplasia, hemophagocytic lymphohistiocytosis and vitamin B12 deficiency were detected in one case (2% each. Conclusions: Etiologies of anemia in HIV/AIDS are multifactorial with anemia of chronic disease being the commonest. For screening of iron deficiency in this group, percent saturation is a better tool than serum ferritin. Absolute lymphocyte count can sometimes be used as a surrogate marker of immunological status in antiretroviral naïve HIV patients, particularly in resource poor areas.

  2. 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.

  3. Morphological differentiation of severe aplastic anaemia from hypocellular refractory cytopenia of childhood

    DEFF Research Database (Denmark)

    Baumann, Irith; Führer, Monika; Behrendt, Sonja;

    2012-01-01

    To evaluate the reproducibility and reliability of the histomorphological criteria differentiating severe aplastic anaemia (SAA) and hypoplastic refractory cytopenia of childhood (RCC), the most frequently acquired hypocellular bone marrow conditions of childhood.......To evaluate the reproducibility and reliability of the histomorphological criteria differentiating severe aplastic anaemia (SAA) and hypoplastic refractory cytopenia of childhood (RCC), the most frequently acquired hypocellular bone marrow conditions of childhood....

  4. What Is Anemia?

    Science.gov (United States)

    ... Treatment will depend on the cause of the anemia and how severe it is. Rate This Content: NEXT >> Updated: May 18, 2012 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  5. Living with Fanconi Anemia

    Science.gov (United States)

    ... from the NHLBI on Twitter. Living With Fanconi Anemia Improvements in blood and marrow stem cell transplants ... November 1, 2011 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  6. How Is Anemia Treated?

    Science.gov (United States)

    ... such as beef or liver), as well as chicken, turkey, pork, fish, and shellfish. Nonmeat foods that ... cause of anemia. Some of these medicines include: Antibiotics to treat infections. Hormones to treat heavy menstrual ...

  7. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... chest pain, and other symptoms. Severe iron-deficiency anemia can lead to heart problems, infections, problems with growth and development in children, and other complications. Infants and young children and ...

  8. Equine Infectious Anemia

    OpenAIRE

    Hoopes, Karl H.

    2017-01-01

    This fact sheet gives information on equine infectious anemia, a blood-borne infectious viral disease of horses, donkeys, and mules. It describes transmission, clinical disease, diagnosis and control.

  9. 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 ...

  10. Iron-Deficiency Anemia

    Medline Plus

    Full Text Available ... Digg. Share this page from the NHLBI on Facebook. Add this link to the NHLBI to my ... Deficiency Anemia article. Updated: March 26, 2014 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA ...

  11. Aplastic Anemia: Alternative Immunosuppressive Treatments and Eltrombopag. A report from the 2014 EBMT Educational Meeting from the Severe Aplastic Anaemia and Infectious Diseases Working Parties.

    Science.gov (United States)

    Risitano, Antonio M

    2015-01-25

    Acquired idiopathic AA is the most typical form of immune-mediated bone marrow failure; the standard treatment of AA for patients who lack a transplant option is immunosuppressive treatment (IST). The standard IST regimen is horse anti-thymocyte globuline (h-ATG) combined with cyclosporine A (CsA), which results in a long term survival around 65-70%. In the past two decades several efforts have been made to improve these results, however results were quite disappointing. Indeed, the addition of a third immunosuppressive agent (micophenolate and sirolimus) on the h-ATG/CsA platform has not resulted in any benefit. Furthermore, the use of anti-lymphocyte agents other than h-ATG (rabbit-ATG, cyclophosphamide, alemtuzumab) has not reproduced the positive outcome seen with standard h-ATG + CsA, irrespective of a more pronounced lymphocyte depletion observed with these alternative compounds. Thus, at least with the strategies tested so far, the strengthening of IST has not led to improved results. More recently, the use of the thrombopoietin mimetic agent eltrombopag has emerged as a possible option to rescue patients failing IST. Nowadays the protection and/or stimulation of residual hematopoietic stem cells by eltrombopag seem an interesting strategy which, once combined with IST, may improve its clinical efficacy. Prospective studies which combine h-ATG and CsA with eltrombopag are currently ongoing in the United States and in Europe, eventually aiming to establish the best non-transplant treatment of AA in the new millennium.

  12. Nutritional anemia and AIDS

    OpenAIRE

    Ruíz, Óscar; Instituto de Investigaciones Clínicas, UNMSM; Díaz, David; Instituto de Investigaciones Clínicas, Facultad de Medicina, UNMSM, y Hospital Nacional Dos de Mayo; Castillo, Óscar; Instituto de Investigaciones Clínicas, UNMSM; Reyes, Rafael; Instituto de Investigaciones Clínicas, UNMSM; Marangoni, Manuela; Programa PROCETSS, Hospital Nacional Dos de Mayo; Ronceros, Gerardo; Instituto de Investigaciones Clínicas, Facultad de Medicina, UNMSM

    2013-01-01

    Objectives: To determine the type of anemia most frequent in patients with AIDS and the various degrees of anemia. Material and methods: One hundred patients 18 to 60 year-old infected with human immune deficiency virus (HIV) with residence in Lima and Callao were studied from January to December 2001 for blood count bone marrow aspiration, serum iron, transferrin, ferritin, folate and vitamin B12 levels. Samples were evaluated at the “Dos de Mayo” Hospital Clinical Pathology Department. Resu...

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    Sant'Anna, Anadayr L M; Garcia, Rita de Cássia N Cubel; Marzoche, Mônica; da Rocha, Heloisa Helena A Gallo; Paula, Maria Tereza M; Lobo, Clarisse C; Nascimento, Jussara P

    2002-01-01

    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.

  15. 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.

  16. Association of aplastic anaemia and Fanconi's disease with HLA-DRB1 alleles.

    Science.gov (United States)

    Yari, F; Sobhani, M; Vaziri, M Z; Bagheri, N; Sabaghi, F; Talebian, A

    2008-12-01

    One of the most fascinating areas of research within the field of histocompatibility at present time concerns an observation that a major human histocompatibility system, human leucocyte antigen (HLA), is deeply involved in the development of a great number of diseases. Major histocompatibility complex is the most polymorphic system in the genome of different species. Recognition of HLA alleles could be useful in transplantation and disease studies. Genetic construct of HLA DRB1 was studied in Iranian normal populations and patients with aplastic anaemia and Fanconi's disease. DNA was extracted from the whole blood of 466 normal, 35 aplastic anaemia and 10 Fanconi's individuals. Then DRB1 gene polymorphism was studied by polymerase chain reaction-sequence-specific primer method. The HLA DRB1 gene analysis showed increase of DRB1*07 in aplastic anaemia patients compared to normal population (P = 0.02). According to this study, the frequency of DRB1*07 in normal individuals was 8.3, and in aplastic anaemia patients, 15.7%. Additionally, the frequency of DRB1*04 in normal, aplastic anaemia and Fanconi's individuals was 10, 5.7 and 20%, respectively. Our results of investigation showed correlation between some HLA alleles with the studied diseases. We reported the frequency of various DR types in aplastic and Fanconi's patients. This study could imply the possible role of HLA-DRB1*07 in the incidence of aplastic anaemia. Moreover, the frequency of DRB1*04, DRB1*03 and DRB1*15 alleles showed intermediate correlation with Fanconi's anaemia.

  17. Could Anemia Cause Hearing Loss?

    Science.gov (United States)

    ... https://medlineplus.gov/news/fullstory_162793.html Could Anemia Cause Hearing Loss? Iron deficiency might keep ear ... Hearing loss may be linked to iron deficiency anemia -- a combination of low levels of iron and ...

  18. Iron refractory iron deficiency anemia

    OpenAIRE

    De Falco, Luigia; Sanchez, Mayka; Silvestri, Laura; Kannengiesser, Caroline; Muckenthaler, Martina U; Iolascon, Achille; Gouya, Laurent; Camaschella, Clara; Beaumont, Carole

    2013-01-01

    Iron refractory iron deficiency anemia is a hereditary recessive anemia due to a defect in the TMPRSS6 gene encoding Matriptase-2. This protein is a transmembrane serine protease that plays an essential role in down-regulating hepcidin, the key regulator of iron homeostasis. Hallmarks of this disease are microcytic hypochromic anemia, low transferrin saturation and normal/high serum hepcidin values. The anemia appears in the post-natal period, although in some cases it is only diagnosed in ad...

  19. [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.

  20. DIAGNOSIS LABORATORIK ANEMIA DEFISIENSI BESI

    OpenAIRE

    Dina Sophia Margina; Sianny Herawati; I W P Sutirta Yasa

    2014-01-01

    Iron deficiency anemia is a decreament of iron level in the body. Iron deficiency anemiais oftenly seen, especially in the tropical countries or the third world country thatassociated with social economic rate. Iron deficiency anemia happens in more than onethird world’s population. Iron deficiency anemia can be caused by chronic hemorrhage,low intake of iron, absorption disturbance, and increasement of demand. To diagnose irondeficiency anemia, laboratoric examination is needed. The treatmen...

  1. Anemia in People with Cancer

    Science.gov (United States)

    ... Managing Cancer-related Side Effects Low Blood Counts 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 ...

  2. 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 ...

  3. 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,

  4. Evaluation of anemia in children.

    Science.gov (United States)

    Janus, Jennifer; Moerschel, Sarah K

    2010-06-15

    Anemia is defined as a hemoglobin level of less than the 5th percentile for age. Causes vary by age. Most children with anemia are asymptomatic, and the condition is detected on screening laboratory evaluation. Screening is recommended only for high-risk children. Anemia is classified as microcytic, normocytic, or macrocytic, based on the mean corpuscular volume. Mild microcytic anemia may be treated presumptively with oral iron therapy in children six to 36 months of age who have risk factors for iron deficiency anemia. If the anemia is severe or is unresponsive to iron therapy, the patient should be evaluated for gastrointestinal blood loss. Other tests used in the evaluation of microcytic anemia include serum iron studies, lead levels, and hemoglobin electrophoresis. Normocytic anemia may be caused by chronic disease, hemolysis, or bone marrow disorders. Workup of normocytic anemia is based on bone marrow function as determined by the reticulocyte count. If the reticulocyte count is elevated, the patient should be evaluated for blood loss or hemolysis. A low reticulocyte count suggests aplasia or a bone marrow disorder. Common tests used in the evaluation of macrocytic anemias include vitamin B12 and folate levels, and thyroid function testing. A peripheral smear can provide additional information in patients with anemia of any morphology.

  5. 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.…

  6. 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…

  7. 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- ...

  8. 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

  9. Anemia (For Parents)

    Science.gov (United States)

    ... rich foods to a child's diet or reducing milk intake. If your teenage daughter is anemic and has heavy or irregular menstrual periods, the doctor may prescribe hormonal treatment to help regulate the bleeding. Folic acid and vitamin B12 supplements may be recommended for anemia due to ...

  10. Sickle cell anemia

    Science.gov (United States)

    ... Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease Images Red blood cells, sickle cell Red blood cells, normal Red blood ... multiple sickle cells Red blood cells, sickle cells Red blood cells, sickle and ... Heeney MM, Ware RE. Sickle cell disease. In: Orkin SH, Fisher DE, Ginsburg D, Look ...

  11. Autoimmune Hemolytic Anemia.

    Science.gov (United States)

    Liebman, Howard A; Weitz, Ilene C

    2017-03-01

    Autoimmune hemolytic anemia is an acquired autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens causing shortened erythrocyte survival. The disorders can present as a primary disorder (idiopathic) or secondary to other autoimmune disorders, malignancies, or infections. Treatment involves immune modulation with corticosteroids and other agents.

  12. 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.

  13. 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.

  14. Screening of aplastic anaemia-related genes in bone marrow CD4+ T cells by suppressive subtractive hybridization

    Institute of Scientific and Technical Information of China (English)

    ZHENG Miao; LIU Wen-li; FU Jin-rong; SUN Han-ying; ZHOU Jian-feng; XU Hui-zhen

    2007-01-01

    Background CD4+ T cells play a crucial role in the pathogenesis of aplastic anaemia. However, the mechanisms of over-proliferation, activation, infiltration of bone marrow and damage to haematopoietic cells of CD4+ T cells in aplastic anaemia are unclear. Therefore, we screened differentially expressed genes of bone marrow CD4+ T cells of aplastic anaemia patients and normal donors by suppressive subtractive hybridization to investigate the pathogenesis of aplastic anaemia.Methods The bone marrow mononuclear cells of a first visit aplastic anaemia patient and a healthy donor of the same age and sex were isolated using lymphocyte separating medium by density gradient centrifugation. With the patients as "tester" and donor as "driver", their CD4+T cells were separated with magnetic bead sorting and a cDNA library established by suppressive subtractive hybridization. Then 15 of the resulting subtracted cDNA clones were randomly selected for DNA sequencing and homological analysis. With semiquantitative RT-PCR, bone marrow samples from 20 patients with aplastic anaemia and 20 healthy donors assessed the expression levels of differentially expressed genes from SSH library.Results PCR detected 89 clones in the library containing an inserted fragment of 100 bp to 700 bp. Among 15 sequenced clones, 12 were known genes including 3 repeated genes. Compared with normal donors, there were 9/12 genes over-expressed in bone marrow CD4+T cells of patients with aplastic anaemia. The effects of these genes included protein synthesis, biology oxidation, signal transduction, proliferative regulation and cell migration. Not all these genes had been reported in the mechanisms of haematopoietic damage mediated by CD4+ T cells in aplastic anaemia.Conclusions Screening and cloning genes, which regulate functions of CD4+ T cells, are helpful in elucidating the mechanisms of over proliferation, activation, infiltrating bone marrow and damaging haematopoietic cells of CD4+ T cells in

  15. [Anemia in the elderly].

    Science.gov (United States)

    Maerevoet, M; Sattar, L; Bron, D; Gulbis, B; Pepersack, T

    2014-09-01

    Anaemia is a problem that affects almost 10% over 65 years and 20% over 85 years. There is no physiological anaemia in the elderly. Any anaemia expresses the existence of a pathological process, regardless of its severity. Anaemia in the elderly is always associated with a poor prognosis that is in terms of mortality, morbidity and risk of fragility. The diagnostic approach to anemia in the elderly is the same as in younger individual. There are many causes of anaemia; anaemia balance is a complex diagnostic process. Most anaemias are due to a deficiency, chronic inflammation or comorbidity. However, in the elderly, the etiology of anaemia is often multifactorial. In a number of cases remain unexplained anaemia. In a number of cases, anemia remain unexplained. Treatment of anaemia is the treatment of the cause, but specific therapeutic aspects to the elderly should be considered, as among other martial substitution or use of erythropoietin (EPO).

  16. 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.

  17. Iron deficiency anemia in pregnancy.

    Science.gov (United States)

    Di Renzo, Gian Carlo; Spano, Filippo; Giardina, Irene; Brillo, Eleonora; Clerici, Graziano; Roura, Luis Cabero

    2015-11-01

    Anemia is the most frequent derailment of physiology in the world throughout the life of a woman. It is a serious condition in countries that are industrialized and in countries with poor resources. The main purpose of this manuscript is to give the right concern of anemia in pregnancy. The most common causes of anemia are poor nutrition, iron deficiencies, micronutrients deficiencies including folic acid, vitamin A and vitamin B12, diseases like malaria, hookworm infestation and schistosomiasis, HIV infection and genetically inherited hemoglobinopathies such as thalassemia. Depending on the severity and duration of anemia and the stage of gestation, there could be different adverse effects including low birth weight and preterm delivery. Treatment of mild anemia prevents more severe forms of anemia, strictly associated with increased risk of fetal-maternal mortality and morbidity.

  18. 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.

  19. [Anemia: guidelines comparison].

    Science.gov (United States)

    Del Vecchio, Lucia

    2009-01-01

    The development of recombinant human erythropoietin and its introduction into the market in the late 1980s has significantly improved the quality of life of patients with chronic kidney disease (CKD) and reduced the need for blood transfusions. Starting from a cautious target, a progressive increase in the recommended hemoglobin levels has been observed over the years, in parallel with an increase in the obtained levels. This trend has gone together with the publication of findings of observational studies showing a relationship between the increase in hemoglobin levels and a reduction in the mortality risk, with the conduction of clinical trials testing the effects of complete anemia correction, and with the compilation of guidelines on anemia control in CKD patients by scientific societies and organizations. In the last two years, evidence of a possible increase in the mortality risk in those patients who were randomized to high hemoglobin levels has resulted in a decrease in the upper limit of the recommended Hb target to be obtained with erythropoietin stimulating agents (ESA), and consequently in a narrowing of the target range. Comparison of guidelines on anemia control in CKD patients is an interesting starting point to discuss single recommendations, strengthen their importance, or suggest new topics of research to fill up important gaps in knowledge.

  20. Management of Iron Deficiency Anemia

    OpenAIRE

    Jimenez, Kristine; Kulnigg-Dabsch, Stefanie; Gasche, Christoph

    2015-01-01

    Anemia affects one-fourth of the world’s population, and iron deficiency is the predominant cause. Anemia is associated with chronic fatigue, impaired cognitive function, and diminished well-being. Patients with iron deficiency anemia of unknown etiology are frequently referred to a gastroenterologist because in the majority of cases the condition has a gastrointestinal origin. Proper management improves quality of life, alleviates the symptoms of iron deficiency, and reduces the need for blo...

  1. Iron-Deficiency Anemia (For Parents)

    Science.gov (United States)

    ... Your 1- to 2-Year-Old 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 ...

  2. Drug-induced immune hemolytic anemia

    Science.gov (United States)

    Immune hemolytic anemia secondary to drugs; Anemia - immune hemolytic - secondary to drugs ... Drugs that can cause this type of hemolytic anemia include: Cephalosporins (a class of antibiotics), most common ...

  3. Iron-Deficiency Anemia (For Parents)

    Science.gov (United States)

    ... Your 1- to 2-Year-Old Iron-Deficiency Anemia KidsHealth > For Parents > Iron-Deficiency Anemia A A ... common nutritional deficiency in children. About Iron-Deficiency Anemia Every red blood cell in the body contains ...

  4. 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.

  5. 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.

  6. ANEMIA SEL SABIT

    Directory of Open Access Journals (Sweden)

    Gede Agus Suwiryawan

    2013-09-01

    Full Text Available Normal 0 false false false EN-US X-NONE X-NONE MicrosoftInternetExplorer4 Anemia is a form of blood disorder most often occurs in the community. Sickle cell anemia is anemia due to hemoglobinopathy caused by a change in the amino acid-6 of the ? globin chain. Sickle cell anemia is common in tropical areas of Africa and some parts of the region Saudi Arabia, India and the Mediterranean as well as black people in America. In addition there are also careers in various European countries. In pathophysiology, there are amino acid change from glutamic acid to valine in the ?-globin chain that causes red blood cells become sickle-shaped when deoxygenatied, but still be able to return to its normal shape when experiencing oxygenation. When the red blood cell membrane have been amended, the polymerization of red blood cells has become irreversible. Clinical picture seen in sickle cell anemia can be divided into two, namely: acute and chronic. Diagnosis can be done is to distinguish between heterozygous or homozygous sickle cell. Treatment provided in accordance with the clinical picture appears. Treatment that can be done is by blood transfusion, bone marrow transplant, anti-sickling drug delivery, and drug delivery to trigger the synthesis of HbF. Treatment still in the development stage is to use stem cells. /* 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;}

  7. Hemolytic anemia caused by chemicals and toxins

    Science.gov (United States)

    ... This list is not all-inclusive. Alternative Names Anemia - hemolytic - caused by chemicals or toxins References Michel M. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Schafer ...

  8. Identification of the c-kit ligand: end of the road for understanding aplastic anemia in steel mutant mice?

    Science.gov (United States)

    Pantel, K; Boertman, J; Nakeff, A

    1991-09-15

    We here report the initiation of hematopoietic recovery in congenitally hypoplastic S1/S1d mice by the cytotoxic ablation of cells bearing the natural killer (NK) phenotype (NK 1.1+). The most striking finding was the early several-fold increase in the cycling fraction of stem and progenitor cells (with the exception of progenitors committed to megakaryocytopoiesis) in the anti-NK 1.1+ antibody-treated group. This increase resulted in an early, complete restoration of total marrow cellularity to the normal (+/+) littermate level. Our data suggest that NK 1.1+ cells exert functions critical to the negative control of hematopoietic cell proliferation in S1/S1d mice.

  9. Recurrent life-threatening reactions to platelet transfusion in an aplastic anaemia patient with a paroxysmal nocturnal haemoglobinuria clone.

    Science.gov (United States)

    Mohamed, M; Bates, G; Richardson, D; Burrows, L

    2014-09-01

    A 60-year-old woman was diagnosed with non-severe aplastic anaemia when she presented with anaemia and thrombocytopenia. She developed recurrent life-threatening hypotensive reactions during transfusion of leukodepleted platelet concentrates, and washed platelet concentrates prevented the development of such reactions subsequently. A paroxysmal nocturnal haemoglobinuria clone was detected on investigating for aplastic anaemia, which has been speculated to play a role in the recurrent hypotensive reactions.

  10. CD19 (+) CD56 (–) myeloma arising in a patient who failed two courses of immunosupressive therapy for aplastic anaemia

    Science.gov (United States)

    Murray, Nigel P; Ruiz, M Amparo; Miranda, G Maximiliano

    2017-01-01

    Patients diagnosed with severe aplastic anaemia and without a compatible bone marrow transplant donor are treated with immunosuppressive therapy. These patients are found with time to develop a clonal disease such as myelodysplasia or paroxysmal nocturnal haemoglobinuria. However, the development of plasma cell dyscrasias is rare. We report the case here of a patient treated with immunosuppressive therapy who went on to develop myeloma 11 months after being diagnosed with severe aplastic anaemia. We include here a review of the literature. PMID:28275389

  11. Warm autoimmune hemolytic anemia.

    Science.gov (United States)

    Naik, Rakhi

    2015-06-01

    Warm autoimmune hemolytic anemia (AIHA) is defined as the destruction of circulating red blood cells (RBCs) in the setting of anti-RBC autoantibodies that optimally react at 37°C. The pathophysiology of disease involves phagocytosis of autoantibody-coated RBCs in the spleen and complement-mediated hemolysis. Thus far, treatment is aimed at decreasing autoantibody production with immunosuppression or reducing phagocytosis of affected cells in the spleen. The role of complement inhibitors in warm AIHA has not been explored. This article addresses the diagnosis, etiology, and treatment of warm AIHA and highlights the role of complement in disease pathology.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. [Analysis of clinical characteristics in 45 cases of Diamond-Blackfan anemia].

    Science.gov (United States)

    Chen, Yu-Mei; Ruan, Min; Wang, Ya-Qin; Zou, Yao; Zhang, Li; Liu, Tian-Feng; Zhu, Xiao-Fan

    2012-06-01

    In order to explore the diagnosis and therapy of Diamond Blackfan anemia (DBA), the clinical data of 45 cases of DBA admitted in our hospital from February 1994 to July 2011 were analyzed retrospectively. The clinical characteristics, results of laboratory examination, treatment reaction and outcome of disease were investigated. The results indicated that out of 45 children diagnosed as DBA, 14 cases (31.1%) had short stature and physical malformation. All patients had anemia with reticulocytopenia. Thirty-four patients (75.6%) had mean corpuscular volume. Eleven patients (24.4%) had macrocytic anemia. Bone marrow examination showed a marked erythroid hypoplasia in all patients. Out of 29 cases tested for fetal hemoglobin (HbF), 13 cases (44.8%) had high level of HbF. Erythroid colony-forming unit of bone marrow was tested in 25 patients, among them 12 patients (48%) showed normal plasia, 13 (52%) showed hypoplasia. The erythropoietin (EPO) levels of 17 patients were elevated. Karyotypes were examined in 28 patients, and showed all normal. The treatment was based on corticosteroids and Cyclosporine A. Thirty patients had good response to corticosteroid therapy, and 10 of them obtained a sustained corticosteroid-induced remission. Twenty cases discontinued corticosteroid therapy after remission, as a result, 15 cases (75%) relapsed, moreover all the relapsed cases still had good response to corticosteroid. Two relapsed patients suffered from aplastic anemia, one of them died of therapy failure. Six patients were unresponsive to corticosteroid, 1 of which achieved remission with cyclosporine A and the others continued to receive regular transfusions. 3 patients received iron chelation therapy. It is concluded that the clinical characteristics, complete blood count, bone marrow smear, HbF level and EPO level are useful to make a diagnosis of DBA. Most patients have a good response to corticosteroid therapy, but relapse rate is high when drug was discontinued. Patients

  14. Severe autoimmune hemolytic anemia with renal neoplasm.

    Science.gov (United States)

    Rhodes, Emily C; Parikh, Sahil P; Bhattacharyya, Nishith

    2014-02-01

    Autoimmune hemolytic anemia is a type of hemolytic anemia characterized by autoantibodies directed against red blood cells shortening their survival. When autoimmune hemolytic anemia is secondary to a paraneoplastic process, severe anemia can occur leading to significant morbidity and even mortality. Here we discuss the literature and present the case of a child with autoimmune hemolytic anemia from a paraneoplastic syndrome secondary to a renal tumor.

  15. 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...... distribution and for previously proposed definitions of anemia. Blood hemoglobin concentration and mean corpuscular volume was measured in participants of the Malmö diet and cancer study-a prospective cohort study, and related to baseline characteristics and outcomes during follow-up. Primary endpoints were...... of anemia, hazard ratio: 1.36, 1.94 and 2.16 for hemoglobin

  16. [Relapse of autoimmune hemolytic anemia in Evans syndrome after thymectomy for pure red cell aplasia].

    Science.gov (United States)

    Higuchi, Masakazu; Osaki, Koichi; Yamano, Yujiro

    2007-06-01

    A 81-year-old woman was referred to our hospital with pure red cell aplasia (PRCA) associated with a thymoma in May 2005. She had previously suffered from Evans syndrome which had been improved by prednisolone in 1999. She underwent a thymectomy, however her anemia subsequently got worse. Reticulocytopenia was noted and a marrow erythroid series was aplastic. Furthermore, both direct and indirect Coombs tests were positive, and the serum haptoglobin level was low. Based on these findings, the patient was diagnosed as having PRCA complicated with relapsed autoimmune hemolytic anemia (AIHA). The PRCA and AIHA were successfully treated with prednisolone and cyclosporine. To our knowledge, only one case of the PRCA complicated with AIHA after thymectomy has been reported. FoxP3 positive regulatory T-cells (Treg), which maintain immunological self-tolerance, were readily detectable in the excised thymoma. Thus, thymectomy resulted in removal of the Treg pool and might explain the autoimmune activation, believed to be one of the mechanisms of the post-thymectomy recurrence of the AIHA in this case.

  17. 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.

  18. The Sirt1 activator SRT3025 expands hematopoietic stem and progenitor cells and improves hematopoiesis in Fanconi anemia mice.

    Science.gov (United States)

    Zhang, Qing-Shuo; Deater, Matthew; Schubert, Kathryn; Marquez-Loza, Laura; Pelz, Carl; Sinclair, David A; Grompe, Markus

    2015-07-01

    Fanconi anemia is a genetic bone marrow failure syndrome. The current treatment options are suboptimal and do not prevent the eventual onset of aplastic anemia requiring bone marrow transplantation. We previously showed that resveratrol, an antioxidant and an activator of the protein deacetylase Sirt1, enhanced hematopoiesis in Fancd2 mutant mice and improved the impaired stem cell quiescence observed in this disease. Given that Sirt1 is important for the function of hematopoietic stem cells, we hypothesized that Sirt1 activation may improve hematopoiesis. Indeed, Fancd2(-/-) mice and wild-type mice treated with the selective Sirt1 activator SRT3025 had increased numbers of hematopoietic stem and progenitor cells, platelets and white blood cells. SRT3025 was also protective against acetaldehyde-induced hematopoietic damage. Unlike resveratrol, however, SRT3025 did not affect stem cell quiescence, suggesting distinct mechanisms of action. Conditional deletion of Sirt1 in hematopoietic cells did not abrogate the beneficial effects of SRT3025, indicating that the drug did not act by directly stimulating Sirt1 in stem cells, but must be acting indirectly via extra-hematopoietic effects. RNA-Seq transcriptome analysis revealed the down-regulation of Egr1-p21 expression, providing a potential mechanism for improved hematopoiesis. Overall, our data indicate that SRT3025 or related compounds may be beneficial in Fanconi anemia and other bone marrow failure syndromes.

  19. The Sirt1 activator SRT3025 expands hematopoietic stem and progenitor cells and improves hematopoiesis in Fanconi anemia mice

    Directory of Open Access Journals (Sweden)

    Qing-Shuo Zhang

    2015-07-01

    Full Text Available Fanconi anemia is a genetic bone marrow failure syndrome. The current treatment options are suboptimal and do not prevent the eventual onset of aplastic anemia requiring bone marrow transplantation. We previously showed that resveratrol, an antioxidant and an activator of the protein deacetylase Sirt1, enhanced hematopoiesis in Fancd2 mutant mice and improved the impaired stem cell quiescence observed in this disease. Given that Sirt1 is important for the function of hematopoietic stem cells, we hypothesized that Sirt1 activation may improve hematopoiesis. Indeed, Fancd2−/− mice and wild-type mice treated with the selective Sirt1 activator SRT3025 had increased numbers of hematopoietic stem and progenitor cells, platelets and white blood cells. SRT3025 was also protective against acetaldehyde-induced hematopoietic damage. Unlike resveratrol, however, SRT3025 did not affect stem cell quiescence, suggesting distinct mechanisms of action. Conditional deletion of Sirt1 in hematopoietic cells did not abrogate the beneficial effects of SRT3025, indicating that the drug did not act by directly stimulating Sirt1 in stem cells, but must be acting indirectly via extra-hematopoietic effects. RNA-Seq transcriptome analysis revealed the down-regulation of Egr1–p21 expression, providing a potential mechanism for improved hematopoiesis. Overall, our data indicate that SRT3025 or related compounds may be beneficial in Fanconi anemia and other bone marrow failure syndromes.

  20. ANEMIA DAN ANEMIA GIZI BESI PADA KEHAMILAN: HUBUNGANNYA DENGAN ASUPAN PROTEIN DAN ZAT GIZI MIKRO

    OpenAIRE

    Nur Handayani Utami; Bunga CH. Rosha

    2015-01-01

    Anemia masih menjadi permasalahan kesehatan pada wanita hamil. Zat besi dianggap sebagai salah satu zat gizi mikro yang berperan terhadap terjadinya anemia. Kekurangan gizi besi dalam tingkat lanjut dapat menyebabkan anemia, yang disebut sebagai anemia gizi besi. Tujuan studi ini adalah untuk menganalisis perbedaan antara asupan protein dan gizi mikro serta menghitung odd ratio (OR) kejadian anemia dan anemia gizi besi akibat asupan protein dan gizi mikro pada wanita hamil di lokasi studi. An...

  1. How Is Hemolytic Anemia Treated?

    Science.gov (United States)

    ... G6PD) deficiency can avoid substances that may trigger anemia. For example, avoid fava beans, naphthalene (a substance found in some moth balls), and certain ... INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  2. Malarial anemia and STAT6

    OpenAIRE

    Robson, Kathryn J.H.; Weatherall, David J

    2009-01-01

    Understanding the mechanisms behind malarial anemia should lead to new approaches to the management and treatment of children. In this perspective article Drs. Robson and Weatherall examine the pathophysiology of this condition. See related article on page 195.

  3. 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.

  4. Sexuality and sickle cell anemia

    Directory of Open Access Journals (Sweden)

    Viviane de Almeida Côbo

    2013-01-01

    Full Text Available 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 adults with sickle cell anemia by investigating the patient's perception of their sex life, as well as the information they had and needed on this subject. METHODS: Twenty male and female sickle cell anemia patients treated at the Hemocentro Regional de Uberaba (UFTM with ages between 19 and 47 years old were enrolled. A socioeconomic questionnaire and a semi-structured interview on sexuality, reproduction and genetic counseling were applied. RESULTS: This study shows that the sickle cell anemia patients lacked information on sexuality especially about the risks of pregnancy and the possible inheritance of the disease by their children. Moreover, the sexual life of the patients was impaired due to pain as well as discrimination and negative feelings experienced in close relationships. CONCLUSION: The health care of sickle cell anemia patients should take into account not only the clinical aspects of the disease, but also psychosocial aspects by providing counseling on sexuality, reproduction and genetics, in order to give this population the possibility of a better quality of life.

  5. 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

  6. Estudos sôbre a anemia produzida em cães por benzoato de estradiol

    Directory of Open Access Journals (Sweden)

    W. O. Cruz

    1944-08-01

    Full Text Available A anemia que se processa em caes quando se administra grandes doses de benzoato de estradiol, nao parece ser produzida por processes conhecidos de destrui?ao intra-organica. Esta substantia paraliza os fenomenos de rege¬nerate hematica, parece interferir por processo desconhecido na fisiologia sanguinea, produz graves lesoes na rede circulatoria que irriga a mucosa do intestino, principalmente jejuno, ocasipnando nesse orgao «extravasamento variavel de sangue, fator seguramente coadjuvante na formação da anemia.The characteristic findings in the pathological picture presented by 60 dogs when administered large doses of estradiol benzoate subcutaneously in an oil vehicle are: thrombocytopenia, aplastic anemia, which follows a brief latent period, intestinal tract haemorrhages, haemorrhagic lesions occurring principally in the mucosa of the jejunum, followed by prostration and death generally within 1 to 3 weeks. Irregular findings are leucocytosis, purpuric skin lesions, visceral haemorrhages and pyoid bone marrow. The total circulating hemoglobin was determined in 9 dogs before and after the anemia was established, thus verifying the total hemoglobin loss. The blood loss through intestinal haemorrhages during the observation period was determined by benzidine test. The quantity of hemoglobin recovered from the feces in the form of hematin varied between 30 and 70% of the total hemoglobin which disappeared from the circulation. In order to ascertain the degree of intra-organic blood destruction the bilirubin eliminated in the urine of dogs with gall-bladder renal type of fis¬tula was determined daily and these values compared with those obtained for normal animals. The blood destruction as measured by this method was practically nil. An attempt was made to determine whether the presence of large quan¬tities of blood in the intestine might exert a paralyzing effect on the regene¬ration of hemoglobin in dogs rendered anemic through

  7. 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 ( ...

  8. 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 ...

  9. 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.

  10. Anemia y enfermedad inflamatoria intestinal Anemia and inflammatory bowel disease

    Directory of Open Access Journals (Sweden)

    F. de la Morena

    2008-05-01

    Full Text Available La anemia es una de las complicaciones más comunes de la enfermedad inflamatoria intestinal. La alta frecuencia de valores bajos de hemoglobina en estos enfermos provoca en muchas ocasiones una infravaloración por parte del médico de esta circunstancia, lo que se traduce en la falta de un tratamiento eficaz. Por otro lado, el carácter complejo de los mecanismos de producción de la anemia en la enfermedad inflamatoria intestinal con frecuencia plantea dudas acerca del tratamiento más adecuado. La identificación correcta de los pacientes con anemia así como la instauración del tratamiento más idóneo serán los dos pilares fundamentales para la mejoría de la calidad de vida de los enfermos. El uso correcto de los suplementos de hierro y las nuevas formulaciones de hierro parenteral, con o sin eritropoyetina asociada, han revolucionado nuestro abordaje de esta complicación evolutiva de la enfermedad inflamatoria intestinalAnemia is a most common complication of inflammatory bowel disease. A high frequency of low hemoglobin values in these patients often leads physicians to subestimate this condition, which translates into ineffective treatment. On the other hand, the complex nature of anemia-inducing mechanisms in inflammatory bowel disease frequently raises doubt about the most appropriate therapy. A correct identification of patients with anemia, and adequate therapy are the essential pillars for improved quality of life. The right use of iron supplementation, and novel parenteral iron formulations, either with or without associated erythropoietin, have revolutionized our approach of this complication in the course of inflammatory bowel disease

  11. Classification of anemia for gastroenterologists.

    Science.gov (United States)

    Moreno Chulilla, Jose Antonio; Romero Colás, Maria Soledad; Gutiérrez Martín, Martín

    2009-10-07

    Most anemia is related to the digestive system by dietary deficiency, malabsorption, or chronic bleeding. We review the World Health Organization definition of anemia, its morphological classification (microcytic, macrocytic and normocytic) and pathogenic classification (regenerative and hypo regenerative), and integration of these classifications. Interpretation of laboratory tests is included, from the simplest (blood count, routine biochemistry) to the more specific (iron metabolism, vitamin B12, folic acid, reticulocytes, erythropoietin, bone marrow examination and Schilling test). In the text and various algorithms, we propose a hierarchical and logical way to reach a diagnosis as quickly as possible, by properly managing the medical interview, physical examination, appropriate laboratory tests, bone marrow examination, and other complementary tests. The prevalence is emphasized in all sections so that the gastroenterologist can direct the diagnosis to the most common diseases, although the tables also include rare diseases. Digestive diseases potentially causing anemia have been studied in preference, but other causes of anemia have been included in the text and tables. Primitive hematological diseases that cause anemia are only listed, but are not discussed in depth. The last section is dedicated to simplifying all items discussed above, using practical rules to guide diagnosis and medical care with the greatest economy of resources and time.

  12. Who Is at Risk for Anemia?

    Science.gov (United States)

    ... history of inherited anemia, such as sickle cell anemia or thalassemia Rate This Content: NEXT >> Updated: May 18, 2012 Twitter Facebook YouTube Google+ SITE INDEX ACCESSIBILITY PRIVACY STATEMENT FOIA NO FEAR ACT OIG ...

  13. Undetectable Glycosylated Hemoglobin in Autoimmune Hemolytic Anemia

    OpenAIRE

    Mitani, Noriyuki; Taguchi, Akihiko; Sakuragi, Shizu; Matsui, Kumiko; Tanaka, Yoshinori; Matsuda, Kazuhiro; Shinohara, Kenji

    2005-01-01

    We encountered two cases of autoimmune hemolytic anemia (AIHA) with undetectable glycosylated hemoglobin (HbA1C) level at diagnosis. Hemolytic anemia improved by administration of prednisolone (PSL) and HbA1C became measurable after response.

  14. 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: 146,000 ...

  15. 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 ...

  16. Anemia caused by low iron - children

    Science.gov (United States)

    ... Disorders of iron and copper metabolism, the sideroblastic anemias, and lead toxicity. In: Orkin SH, Fisher DE, Ginsburg D, ... Philadelphia, PA: Elsevier; 2016:chap 455. Read More Anemia Hemoglobin Lead poisoning Review Date 2/11/2016 Updated by: ...

  17. Retkulocyte production index in various anemia

    OpenAIRE

    Setyawati, Setyawati

    2015-01-01

    Background: Reticulocyte production index (IPR) is used to determine the classification of functional anemia. More specific laboratory parameters are used in managing patient. There were some inappropriate of IPR correlation with the diagnosis of anemia in clinical practice.Objectives: The study was designed to know normal hematocrit in Sardjito General Hospital, to determine IPR in various anemia and to compare with standard IPR and final diagnosis in anemia. Methods: Observational study was...

  18. Manifestasi Anemia Pernisiosa Di Rongga Mulut

    OpenAIRE

    Nona Aini

    2008-01-01

    Anemia pernisiosa adalah salah satu penyakit kronis berupa berkurangnya produksi sel darah merah akibat defisiensi vitamin 812 dan asam folat, Salah satu fungsi vitamin 812 adalah untuk pembentukan sel darah merah di dalam sum-sum tulang menjadi aktif. Akibat defisiensi vitamin 812 dapat menyebabkan terganggunya sintesa DNA dan RNA. Terganggunya sintesa DNA akan menyebabkan anemia di sum-sum tulang dalam bentuk anemia makrositik dan di dalam darah dalam bentuk anemia megaloblastik. Sedangk...

  19. Th17/Treg细胞失衡与再生障碍性贫血研究进展%Recent Progress of Study on Imbalance of Th17/Treg Cells in Aplastic Anemia——Review

    Institute of Scientific and Technical Information of China (English)

    杨友卫; 郑智茵; 姚红章

    2012-01-01

    再生障碍性贫血( aplastic anemia,AA)是一种以效应T细胞功能亢进导致造血组织损伤为特征的自身免疫性疾病.近年来研究发现,AA与Th17细胞数量增加及Treg细胞数量减少密切相关,且Th17和Treg细胞功能上相互拮抗.动物实验表明,IL-17抗体靶向治疗及Treg细胞回输治疗均可改善免疫介导的AA小鼠骨髓衰竭状态.本文就Th17/Treg细胞与AA关系最新研究进展作一综述,旨在进一步探讨AA发病机制,为临床治疗提供新思路.本综述讨论的主要问题有:Th1 7/Treg细胞一般生物学特性,Th17/Treg细胞平衡的调节及相关细胞因子,Th17/Treg细胞与再生障碍性贫血关系等.%Aplastic anemia (AA) is an autoimmune disease characterized by destruction of hematopoietic tissue resulting in hyperfunction of effector T-lymphocytes. Recent studies indicate that Thl7 and Treg cells are functionally antagonistic each other, and the increase of Thl7 cells and decrease of Treg cells are closely related with AA. In vivo experiments showed that both anti-IL-17 treatment and Treg cell infusion can protect against immune-mediated bone marrow failure in mouse with AA. This review summarizes the recent progress of study on imbalance of Thl7/ Treg cells in AA, so as to explore the pathogenesis of AA and provide approach to clinical treatment. The main problems that are discussed in this review include biological characteristics of Thl7/Treg cells, the regulation of Thl7/Treg cell balance and related cytokines, the relationship between Thl7/Treg cells and AA.

  20. 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.

  1. Pernicious Anemia with Autoimmune Hemolytic Anemia: A Case Report and Literature Review

    OpenAIRE

    Sri Lakshmi Hyndavi Yeruva; Raj Pal Manchandani; Patricia Oneal

    2016-01-01

    Pernicious anemia is a common cause of vitamin B12 deficiency. Here, we discuss a case of a young woman who presented with severe anemia along with a history of iron deficiency anemia. After a review of her clinical presentation and laboratory data, we identified an autoimmune hemolytic anemia and a concomitant pernicious anemia. The concurrence of both these hematological diagnoses in a patient is rare.

  2. Pernicious Anemia with Autoimmune Hemolytic Anemia: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Sri Lakshmi Hyndavi Yeruva

    2016-01-01

    Full Text Available Pernicious anemia is a common cause of vitamin B12 deficiency. Here, we discuss a case of a young woman who presented with severe anemia along with a history of iron deficiency anemia. After a review of her clinical presentation and laboratory data, we identified an autoimmune hemolytic anemia and a concomitant pernicious anemia. The concurrence of both these hematological diagnoses in a patient is rare.

  3. A comparative immunohistochemical study of aplastic thymuses for lymphocytic, epithelial, proliferative and apoptotic indices

    Directory of Open Access Journals (Sweden)

    Mahjoub F.

    2008-03-01

    Full Text Available Background: Immune deficiency is one of the major causes of morbidity and mortality in the modern world. Primary immunodeficiency comprises a wide range of disorders that mainly manifest in early childhood as devastating infections with opportunistic organisms. Thymic aplasia is found on autopsy of some patients afflicted with immune deficiency disorders, such as DiGeorge syndrome and severe combined immunodeficiency (SCID. After a thorough search of the literature, we found little information on the cellular characteristics of these thymuses. Our study aims to elucidate role of apoptosis in the pathogenesis of thymic aplasia and compare various lymphocytic and epithelial markers in normal and aplastic thymuses. Methods: We selected 12 subjects who died of severe infections with aplastic thymus found on autopsy, and 11 control subjects who died of unrelated causes, such as congenital heart disease. The presence of several markers, including Bcl2, P53, lymphocytic markers, and CD68, was examined using immunohistochemical methods on paraffin-embedded thymus sections. Positively-stained cells were counted per 1000 cells and the results stated as percentage of positive cells.                        Results: The mean age of the control group was between 7 days to 18 months (mean: 4.5 months. Parental consanguinity was present in 45.5% and 9.1% of the control and case groups, respectively; however, this was not statistically significant. We found significantly lower expression of Bcl2 in the case group (p value: 0.038. Furthermore, expression of CD68 was significantly higher in the case group. Epithelial markers were significantly higher in case subjects, although CD8 expression was higher in the control group. The presence of other markers was not significantly different between the two groups.Conclusions: Increase in apoptosis has a role in aplastic thymuses and prevention of apoptosis may halt this process. Also high CD68

  4. Aplastic anaemia preceding acute lymphoblastic leukaemia in an adult with isolated deletion of chromosome 9q.

    LENUS (Irish Health Repository)

    Kelly, Kevin

    2008-12-01

    Aplastic anaemia (AA) can precede acute lymphoblastic leukaemia (ALL) in 2% of children but this is rarely reported to occur in adults. A 21-year-old male presented with bone marrow failure and bone marrow biopsy showed a profoundly hypocellular marrow. He recovered spontaneously but represented 2 months later when he was diagnosed with pre-B acute lymphoblastic leukaemia. Chromosomal examination revealed 46,XY,del(9)(q13q34). To the best of our knowledge this is the first case to be reported of aplasia preceding ALL with 9q minus as the sole chromosomal abnormality.

  5. 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...

  6. Hubungan Anemia Defisiensi Besi Terhadap Gangguan Konsentrasi

    OpenAIRE

    Meutirani, Riska

    2015-01-01

    Iron deficiency anemia is dedecrease in the number of red blood cells caused by too little iron. Iron deficiency anemia is the most common form of anemia. About 20% of women, 50% of pregnant womenand 3% of men do not have enough iron on their body. Anemia develops slowly after the normal iron stores in the body and bone marrowhave run out. In general, womwn have smaller stores of iron than men because they lose more through menstruation. Iron deficiency anemia may also be caused by poor absor...

  7. Hereditary sideroblastic anemia: pathophysiology and gene mutations.

    Science.gov (United States)

    Harigae, Hideo; Furuyama, Kazumichi

    2010-10-01

    Sideroblastic anemia is characterized by anemia with the emergence of ring sideroblasts in the bone marrow. Ring sideroblasts are erythroblasts characterized by iron accumulation in perinuclear mitochondria due to impaired iron utilization. There are two forms of sideroblastic anemia, i.e., inherited and acquired sideroblastic anemia. Inherited sideroblastic anemia is a rare and heterogeneous disease caused by mutations of genes involved in heme biosynthesis, iron-sulfur (Fe-S) cluster biogenesis, or Fe-S cluster transport, and mitochondrial metabolism. The most common inherited sideroblastic anemia is X-linked sideroblastic anemia (XLSA) caused by mutations of the erythroid-specific δ-aminolevulinate synthase gene (ALAS2), which is the first enzyme of heme biosynthesis in erythroid cells. Sideroblastic anemia due to SLC25A38 gene mutations, which is a mitochondrial transporter, is the next most common inherited sideroblastic anemia. Other forms of inherited sideroblastic anemia are very rare, and accompanied by impaired function of organs other than hematopoietic tissue, such as the nervous system, muscle, or exocrine glands due to impaired mitochondrial metabolism. Moreover, there are still significant numbers of cases with genetically undefined inherited sideroblastic anemia. Molecular analysis of these cases will contribute not only to the development of effective treatment, but also to the understanding of mitochondrial iron metabolism.

  8. Sickle Cell Anemia (For Teens)

    Science.gov (United States)

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Sickle Cell Disease KidsHealth > For Teens > Sickle Cell Disease Print ... Stay Well? en español Anemia falciforme What Is Sickle Cell Disease? Sickle cell disease is a blood disorder ...

  9. 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…

  10. Management of Iron Deficiency Anemia

    Science.gov (United States)

    Jimenez, Kristine; Kulnigg-Dabsch, Stefanie

    2015-01-01

    Anemia affects one-fourth of the world’s population, and iron deficiency is the predominant cause. Anemia is associated with chronic fatigue, impaired cognitive function, and diminished well-being. Patients with iron deficiency anemia of unknown etiology are frequently referred to a gastroenterologist because in the majority of cases the condition has a gastrointestinal origin. Proper management improves quality of life, alleviates the symptoms of iron deficiency, and reduces the need for blood transfusions. Treatment options include oral and intravenous iron therapy; however, the efficacy of oral iron is limited in certain gastrointestinal conditions, such as inflammatory bowel disease, celiac disease, and autoimmune gastritis. This article provides a critical summary of the diagnosis and treatment of iron deficiency anemia. In addition, it includes a management algorithm that can help the clinician determine which patients are in need of further gastrointestinal evaluation. This facilitates the identification and treatment of the underlying condition and avoids the unnecessary use of invasive methods and their associated risks. PMID:27099596

  11. Iron deficiency anemia in children

    OpenAIRE

    Pochinok, T. V.

    2016-01-01

    In the article the role of iron in the human body is highlighted. The mechanism of development of iron deficiency states, their consequences and the basic principles of diagnosis and correction of children of different ages are shown.Key words: children, iron deficiency anemia, treatment.

  12. [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

  13. Anemia in peritoneal dialysis patients

    Directory of Open Access Journals (Sweden)

    Laušević Mirjana

    2006-01-01

    Full Text Available A normocytic normochromic anemia is one of the first signs of renal failure. Since anemia increases morbidity and mortality, its elimination is one of the essential objectives of the treatment. Human recombinant erythropoietin (rHuEPO has changed the therapeutical approach to anemia. The aim of the present study was to compare efficacy of anemia correction in peritoneal dialysis patients depending on treatment and dialysis modality. The study is the retrospective analysis of 64 patients who presented to our Clinic in 2003. Eighteen (28.13% patients were treated with rHuEPO, 14 (28% underwent continuous ambulatory peritoneal dialysis (CAPD, 2 (100% - automated peritoneal dialysis (APD and 2 (33.3% - intermittent peritoneal dialysis (IPD. Mean hemoglobin level was 98.6±17.82 g/l in patients treated with rHuEPO versus 98.81±15.14 g/I in patients without rHuEPO treatment. Erythropoietin requirements were 3392.85±1211.77 IU/week. AII patients received iron supplementation during rHuEPO therapy. Mean serum ferritin levels were 463.41 ±360 μg/l. Transferrin saturation (TSAT was 0.35±0.16%. No difference of serum iron and TSAT levels was found between CAPD and IPD patients. The degree of anemia significantly differed between CAPD and IPD patients. A total of 17.11% of PD patients were given blood transfusions, most frequently during the first three months after the onset of dialysis. Our conclusion is that the number of patients receiving rHuEPO should be increased, as 50% of our patients should be substituted, while only 28% are being treated. As 50% of patients receiving rHuEPO failed to reach target Hgb levels, higher EPO doses should be considered. Iron stores should be continuously monitored, particularly in patients receiving rHuEPO, since iron deficiency is an important problem for patients undergoing peritoneal dialysis, especially during erythropoietin therapy. Oral iron supplementation is satisfactory in the majority of patients, and

  14. PERBEDAAN TINGKAT PENGETAHUAN ANEMIA REMAJA PUTRI SEKOLAH MENENGAH UMUM ANEMIA DAN NON ANEMIA DI ENAM DATI II PROPINSI JAWA BARAT

    OpenAIRE

    Edwi Saraswati; Iman Sumarno

    2012-01-01

    Telah dilakukan survei cepat tentang prevalensi anemia dan pengetahuan anemia pada remaja putri SMU di enam Dati II di Propinsi Jawa Barat. Remaja putri merupakan generasi penerus yang perlu diperhatikan, karena kelak menjadi ibu dan atau tenaga pekerja. Terhadap remaja putri sampel dilakukan pemeriksaan Hb dan pengumpulan data mengenai pengetahuan remaja putri tentang anemia. Informasi ini sangat berguna sebagai dasar penetapan prioritas program kesehatan dan gizi pada kelompok remaja putri ...

  15. Fetal Outcome pada Kehamilan Aterm Anemia dan Tidak Anemia di RS Achmad Mochtar Bukittinggi

    Directory of Open Access Journals (Sweden)

    Daulat Azhari

    2016-01-01

    Full Text Available AbstrakAnemia pada kehamilan merupakan faktor resiko gangguan pada fetal outcome dan memiliki komplikasi yang meningkatkan maternal dan perinatal mortality. Tujuan penelitian ini adalah menentukan perbedaan fetal outcome pada kehamilan aterm dengan anemia dan tidak anemia..Penelitian ini menggunakan data sekunder dengan rancangan cross sectional. Total sampel adalah 110 yang terdiri dari 55 ibu hamil aterm dengan anemia dan 55 ibu hamil aterm tidak anemia. Tekhnik pengambilan sampel adalah consecutive sampling dan analisis data menggunakan tes Mann- Whitney. Hasil uji diperoleh rerata berat badan lahir bayi pada ibu hamil aterm anemia adalah 3097,27 gr± 366,93 gr, yang sedikit lebh rendah dibandingkan pada ibu hamil aterm tidak anemia 3200,55 gr± 343,02 gr dengan nilai p= 0,214. Rerata APGAR skor pada menit pertama pada kelompok anemia adalah 7,04± 1,39, yang sedikit lebih rendah jika dibandingkan pada ibu hamil aterm tidak anemia 7.36± 0,65 dengan nilai p= 0,480. Rerata APGAR skor pada menit kelima pada kelompok anemia 8,11± 1,20 sedikit lebih rendah dibandingkan ibu hamil aterm tidak anemia 8,40± 0,62 dengan nilai p= 0,483. Rerata panjang badan lahir pada kelompok anemia adalah 48,58 cm± 1,52 cm hampir tidak memiliki perbedaan dibandingkan ibu hamil aterm tidak anemia 48,89 cm± 1,56 cm  dengan nilai p=0,310. Disimpulkan bahwa tidak ada perbedaan berat badan lahir, APGAR skor menit pertama dan kelima, dan panjang badan lahir pada kehamilan aterm dengan anemia dan tidak anemia.Kata kunci: berat badan lahir, APGAR skor, panjang badan lahir,  wanita hamil aterm dengan anemia AbstractAnemia in pregnancy is a risk factor of fetal outcome disorder and it have complication that increase of matenal and perinatal mortality. The objective of this study was to determine the differences of fetal outcome between aterm pregnant women with anemia and non anemia.This research uses secondary data by using cross sectional study design. Total sample is

  16. Anemias Hereditárias

    OpenAIRE

    Ferreira, Fátima; Carvalho, Fernanda; Costa, Vítor; Farinha, Nuno; Gil-da-Costa, M.ª João; Norton, Lucília; Reis, Ilidia Lima

    2014-01-01

    Os autores efectuam a revisão das anemias hereditárias da consulta de hematologia pediátrica do Hospital S. João no período compreendido entre 1982 e 1995. Dividem-nas em três grandes grupos: Hemoglobinopatias, Doenças da Membrana do glóbulo rubro e Enzimopatias. Calculam a sua frequência relativa, os parâmetros clínicos e analíticos relevantes no diagnóstico, a abordagem terapêutica e respectiva evolução.Destacam a especificidade desta consulta em que as anemias hereditárias correspondem a 4...

  17. Clinical outcomes of transfusion-associated iron overload in patients with refractory chronic anemia

    Directory of Open Access Journals (Sweden)

    Gao C

    2014-04-01

    Full Text Available Chong Gao, Li Li, Baoan Chen, Huihui Song, Jian Cheng, Xiaoping Zhang, Yunyu SunDepartment of Hematology and Oncology, Key Department of Jiangsu Medicine, Zhongda Hospital, Medical School, Southeast University, Nanjing, Jiangsu Province, People’s Republic of ChinaBackground: The purpose of this study was to evaluate the clinical outcomes of transfusion-associated iron overload in patients with chronic refractory anemia.Methods: Clinical manifestations, main organ function, results of computed tomography (CT, endocrine evaluation, and serum ferritin levels were analyzed retrospectively in 13 patients who were transfusion-dependent for more than 1 year (receiving >50 units of red blood cells to determine the degree of iron overload and efficacy of iron-chelating therapy.Results: Serum ferritin levels increased to 1,830–5,740 ng/mL in all patients. Ten patients had abnormal liver function. The CT Hounsfield units in the liver increased significantly in eleven patients, and were proportional to their serum ferritin levels. Skin pigmentation, liver dysfunction, and endocrine dysfunction were observed in nine patients with serum ferritin >3,500 ng/mL, eight of whom have since died. Interestingly, serum ferritin levels did not decrease significantly in nine transfusion-dependent patients who had received 15–60 days of iron-chelating therapy.Conclusion: Transfusion-dependent patients may progress to secondary iron overload with organ impairment, which may be fatal in those who are heavily iron-overloaded. The CT Hounsfield unit is a sensitive indicator of iron overload in the liver. Iron chelation therapy should be initiated when serum ferritin is >1,000 ng/mL and continued until it is <1,000 ng/mL in transfusional iron-overloaded patients.Keywords: anemia, aplastic, iron overload, myelodysplastic syndromes

  18. Thymoma with Autoimmune Hemolytic Anemia

    OpenAIRE

    Kensuke Suzuki; Minehiko Inomata; Shiori Shiraishi; Ryuji Hayashi; Kazuyuki Tobe

    2014-01-01

    A 38-year-old Japanese male was referred to our hospital with abnormal chest X-ray results and severe Coombs-positive hemolytic anemia. He was diagnosed with a stage IV, WHO type A thymoma and was treated with oral prednisolone (1 mg/kg/day) and subsequent chemotherapy. After chemotherapy, the patient underwent surgical resection of the thymoma. Hemolysis rapidly disappeared and did not return after the discontinuation of oral corticosteroids. Corticosteroid therapy may be preferable to chemo...

  19. Correction of Fanconi Anemia Group C Hematopoietic Stem Cells Following Intrafemoral Gene Transfer

    Directory of Open Access Journals (Sweden)

    Ouassila Habi

    2010-01-01

    Full Text Available The main cause of morbidity and mortality in Fanconi anemia patients is the development of bone marrow (BM failure; thus correction of hematopoietic stem cells (HSCs through gene transfer approaches would benefit FA patients. However, gene therapy trials for FA patients using ex vivo transduction protocols have failed to provide long-term correction. In addition, ex vivo cultures have been found to be hazardous for FA cells. To circumvent negative effects of ex vivo culture in FA stem cells, we tested the corrective ability of direct injection of recombinant lentiviral particles encoding FancC-EGFP into femurs of FancC−/− mice. Using this approach, we show that FancC−/− HSCs were efficiently corrected. Intrafemoral gene transfer of the FancC gene prevented the mitomycin C-induced BM failure. Moreover, we show that intrafemoral gene delivery into aplastic marrow restored the bone marrow cellularity and corrected the remaining HSCs. These results provide evidence that targeting FA-deficient HSCs directly in their environment enables efficient and long-term correction of BM defects in FA.

  20. Iron, Anemia, and Iron Deficiency Anemia among Young Children in the United States

    OpenAIRE

    Gupta, Priya M.; Perrine, Cria G.; Zuguo Mei; Scanlon, Kelley S.

    2016-01-01

    Iron deficiency and anemia are associated with impaired neurocognitive development and immune function in young children. Total body iron, calculated from serum ferritin and soluble transferrin receptor concentrations, and hemoglobin allow for monitoring of the iron and anemia status of children in the United States. The purpose of this analysis is to describe the prevalence of iron deficiency (ID), anemia, and iron deficiency anemia (IDA) among children 1–5 years using data from the 2007–201...

  1. Estimated red blood cell thickness in microcytic anemia due to iron deficiency anemia and thalassemia

    Directory of Open Access Journals (Sweden)

    Viroj Wiwanitkit

    2009-05-01

    Full Text Available "nAnemia is one of the most common hematological disorders that are still the present in all countries around the world. Microcytic anemia is a specific kind of anemia presenting with small red blood cell. In this paper, the author discusses on the estimated red blood cell thickness, a new proposed parameter, comparing between that of iron deficiency anemia and thalassemia and further extrapolate on the clinical implication.

  2. 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.

  3. Role of Complement in Autoimmune Hemolytic Anemia

    OpenAIRE

    Berentsen, Sigbjørn

    2015-01-01

    Summary The classification of autoimmune hemolytic anemias and the complement system are reviewed. In autoimmune hemolytic anemia of the warm antibody type, complement-mediated cell lysis is clinically relevant in a proportion of the patients but is hardly essential for hemolysis in most patients. Cold antibody-mediated autoimmune hemolytic anemias (primary cold agglutinin disease, secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria) are entirely complement-mediated disorder...

  4. The Clinical Pictures of Autoimmune Hemolytic Anemia

    OpenAIRE

    Packman, Charles H.

    2015-01-01

    Summary Autoimmune hemolytic anemia is characterized by shortened red blood cell survival and a positive Coombs test. The responsible autoantibodies may be either warm reactive or cold reactive. The rate of hemolysis and the severity of the anemia may vary from mild to severe and life-threatening. Diagnosis is made in the laboratory by the findings of anemia, reticulocytosis, a positive Coombs test, and specific serologic tests. The prognosis is generally good but renal failure and death some...

  5. Anemia in children with chronic kidney disease

    OpenAIRE

    Koshy, Susan M.; Geary, Denis F.

    2007-01-01

    Anemia is a common feature of chronic kidney disease, but the management of anemia in children is complex. Erythropoietin and supplemental iron are used to maintain hemoglobin levels. The National Kidney Foundation-Kidney Disease Outcomes Quality Initiative (NKF-KDOQI) clinical practice guidelines for the management of anemia specifically in children were recently published. Pediatric nephrologists are encouraged to use current clinical practice guidelines and best evidence in conjunction wit...

  6. Anemia and iron deficiency in heart failure.

    Science.gov (United States)

    Gil, Victor M; Ferreira, Jorge S

    2014-01-01

    Heart failure is a common problem and a major cause of mortality, morbidity and impaired quality of life. Anemia is a frequent comorbidity in heart failure and further worsens prognosis and disability. Regardless of anemia status, iron deficiency is a common and usually unidentified problem in patients with heart failure. This article reviews the mechanisms, impact on outcomes and treatment of anemia and iron deficiency in patients with heart failure.

  7. An unusual cause of anemia and encephalopathy

    OpenAIRE

    Sanjeev Kumar Sharma; Dharma Choudhary; Anil Handoo; Gaurav Dhamija; Gaurav Kharya; Vipin Khandelwal; Mayank Dhamija; Sweta Kothari

    2015-01-01

    The authors present here an interesting case of recent onset anemia that was associated with an encephalopathy of the unusual cause.Although severe anemia can theoretically result in anemic hypoxia and can then lead to hypoxic encephalopathy, it is not a primary cause of encephalopathy. More frequently anemia can contribute together with other multiple causes of encephalopathy, such as infections, metabolic abnormalities, trauma, hepatic dysfunction, hypertension, toxins.

  8. Diagnosis treatment and medical evaluation of aircrew with anemia%飞行人员贫血的临床诊治及医学鉴定

    Institute of Scientific and Technical Information of China (English)

    刘静; 丁丽; 王恒湘; 薛梅; 王志东; 闫侠芳; 朱玲; 董磊; 闫洪敏; 郑晓丽; 韩冬梅

    2015-01-01

    目的:总结飞行人员贫血的病因、诊断、治疗及医学鉴定。方法回顾性分析1993年1月—2013年12月在空军总医院住院的17例飞行人员贫血的病史、诊断分类、治疗经过、疾病转归及医学鉴定。结果缺铁性贫血(iron-deficiency anemia,IDA)6例,失血性贫血2例,IDA合并失血性贫血1例,巨幼细胞贫血(megaloblastic anemia,MA)3例,再生障碍性贫血(aplastic anemia,AA)、β-地中海贫血(轻型)、肺癌合并溶血性贫血(hemolytic anemia,HA)、胸腺瘤合并慢性病贫血(anemia of chronic disease,ACD)、原因未明贫血各1例。IDA、MA、失血性贫血患者经病因治疗贫血均治愈,除1例IDA因地面晕厥飞行不合格、1例MA合并垂体瘤、1例MA合并甲状腺功能亢进飞行暂不合格以外,余均飞行合格;β-地中海贫血(轻型)、原因未明贫血(轻度)各1例未给予治疗,飞行合格;1例AA患者、2例肿瘤合并贫血患者经治疗好转,但飞行不合格。结论飞行人员贫血可由多种原因引起,IDA占大多数,且女性多见,其次为MA、失血性贫血。常见原因有偏食、素食、女性月经过多、痔疮及消化道溃疡出血。多数经治疗痊愈,飞行合格。AA及继发于恶性肿瘤的贫血患者,原则上,一经确诊飞行不合格,治愈者可个别评定。继发于其他良性疾病者,贫血治愈后医学鉴定依原发病治疗结果而定。%ObjectiveTo summarize the etiology, clinical diagnosis, treatment and medical evaluation principles for anemia of aircrew.Methods17 cases of aircrew with anemia, who were hospitalized in Air Force General Hospital from January 1993 to December 2013, were retrospectively analyzed in respect of illness history, clinical diagnosis, therapy, disease outcome and medical evaluation.Results6 cases were diagnosed as Iron deficieny anemia (IDA), 2 cases were hemorrhagic anemia, one case was IDA

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

    Science.gov (United States)

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

  10. 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 ...

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

    Science.gov (United States)

    ... X-ray 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- ...

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

    Science.gov (United States)

    ... Operating Room? 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- ...

  13. [Anemia in patients with rheumatoid arthritis].

    Science.gov (United States)

    Wahle, M

    2012-12-01

    One of the most frequent extra-articular organ manifestations in rheumatoid arthritis (RA) is anemia. As anemia in RA patients may result in severe symptoms and aggravation of other disease manifestations (e.g. arteriosclerosis), the influence on the course of RA is profound. However, the importance of anemia in RA patients is frequently underestimated. The etiology of anemia in RA is complex. Anemia of inflammation (AI) and iron deficiency anemia, alone or in combination are the most frequent forms of anemia in RA. Changes in iron metabolism are the leading causes of anemia in RA patients and mainly induced by the altered synthesis and function of hepcidin and ferroportin. Hepcidin, a peptide produced in the liver and immunocompetent cells, impairs the expression of ferroportin on iron-secreting cells, thus reducing iron bioavailability. The typical changes of iron metabolism and hepcidin synthesis in RA are induced by proinflammatory cytokines, primarily interleukin-6. Hence, the treatment of RA with cytokine antagonists has significant therapeutic implications on anemia in the context of inflammation and impaired iron metabolism.

  14. 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.......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....

  15. Widespread ocular use of topical chloramphenicol: is there justifiable concern regarding idiosyncratic aplastic anaemia?

    Science.gov (United States)

    McGhee, C N; Anastas, C N

    1996-02-01

    A theoretical but as yet not conclusively proved risk of chloramphenicol induced idiosyncratic aplastic anaemia exists with topical ophthalmic therapy, with the absolute, but highly improbable, maximum risk of death (equalling that of systemic therapy) being 1 in 50,000 to 90,000. To put this in realistic perspective, one must note that the comparable risk of fatal anaphylaxis resulting from penicillin therapy, from any route, is similar at 1 in 100,000. Indeed, it has been noted recently that with more than 200 million ocular chloramphenicol products dispensed in the UK in the past 10 years, only 11 reports (all non-fatal) of suspected topical chloramphenicol induced blood dyscrasia have been reported to the Committee on the Safety of Medicines since 1966. One also has to consider that inadvertent exposure to minute quantities of chloramphenicol (ng/ml) may occur through consumption of livestock that have been treated with chloramphenicol. Broad statements condemning topical chloramphenicol need to be tempered with its proved safety, tolerance, cost, and efficacy while acknowledging an extremely remote risk of the very serious adverse effect of drug induced aplastic anaemia. Risk-benefit assessment is the duty of all prescribing physicians and a decision to prescribe or not prescribe must be made on the basis of personal judgment and an awareness of the statistics in perspective. The only known factor to be associated with vulnerability in the case of topical chloramphenicol is family history. There is no evidence to date that suggests children are any more susceptible than adults.

  16. Risk factors associated with anemia, iron deficiency and iron deficiency anemia in rural Nepali pregnant women.

    Science.gov (United States)

    Makhoul, Zeina; Taren, Douglas; Duncan, Burris; Pandey, Pooja; Thomson, Cynthia; Winzerling, Joy; Muramoto, Myra; Shrestha, Ram

    2012-05-01

    We conducted a cross sectional study to investigate risk factors associated with severe anemia [hemoglobin (Hb) iron status among Nepali pregnant women. Socio-demographic, anthropometric, health and dietary data were collected from 3,531 women living in the southeastern plains of Nepal. Stool samples were analyzed for intestinal helminthes. Dark adaptation was assessed using the Night Vision Threshold Test (NVTT). Hb levels were measured in all subjects to detect anemia and the soluble transferrin receptor (sTfR) was measured among a subsample of 479 women. The iron status categories were: 1) normal (Hb> or = 11.0 g/dl and sTfR anemia without iron deficiency (Hbiron deficiency without anemia (Hb > or = 11.0 g/dl and sTfR>8.5 mg/l); and 4) iron deficiency anemia (IDA): (Hb8.5 mg/l). Factors associated with severe anemia and poor iron status were determined using logistic regression. Hookworm infection increased the risk for developing severe anemia [adjusted odds ratio (AOR): 4.26; 95% CI 1.67-10.89; piron deficiency with and without anemia. Intake of iron supplements as tablets and/or tonic was protective against severe anemia, anemia without iron deficiency and IDA. Dietary heme iron was significantly associated with iron deficiency without anemia (RRR: 0.1; 95% CI 0.02-0.47; panemia and associated nutrient deficiencies.

  17. Comparison of Bone Marrow and Blood Cell Morphology Between Refractory Anemia and Other Anemia Disease%难治性贫血与其它贫血性疾病骨髓及血细胞形态学比较分析

    Institute of Scientific and Technical Information of China (English)

    程虹; 江明; 杜伟; 钟笛; 郝建萍; 李玲

    2012-01-01

    本研究观察骨髓增生异常综合征(MDS),主要是难治性贫血(RA)骨髓及外周血细胞形态学特点,并与其它贫血性疾病(慢性再生障碍性贫血、溶血性贫血、巨幼细胞性贫血)进行比较分析.取患者骨髓及外周血制成涂片行瑞氏染色,骨髓分类500个有核细胞,外周血分类100个有核细胞,观察红系、粒系、巨核系病态细胞特点.结果发现,外周血中性分叶核细胞浆内颗粒稀少或缺如、Pelger核异常改变、幼粒细胞数及检出率、单核细胞检出率以及骨髓粒系各阶段细胞出现颗粒缺如,红系奇数核、核出芽,巨核系小巨核及单圆核巨核细胞等在RA与慢性再生障碍性贫血、溶血性贫血、巨幼细胞性贫血比较均有显著性差异(P<0.05).结论:细胞形态学是MDS诊断的基础,外周血及骨髓细胞形态学异常在MDS与其他贫血性疾病的鉴别诊断中具有重要作用.%This study was purposed to investigate the cell morphological features of bone marrow and peripheral blood in patients with myelodysplastic syndrome, mainly with refractory anemia, and to compare them with other anemia diseases including chronic aplastic anemia, hemolytic anemia and megaloblastic anemia. The bone marrow and peripheral blood were taken from patients for preparing the smears with Wright staining. 500 karyocytes in bone marrow and 100 karyocytes in peripheral blood were detected, and the features of morbid cells of erythrocyte, granulocyte and megakaryocytic series were observed. The results showed that differences between refractory anemia, chronic aplastic anemias and hemolytic anemia as well as megaloblastic anemia were statistically significant (P < 0.05) in the granules scarce and absence in the intracytoplasm of segmented neutrocyte in peripheral blood, Pelger dyskaryosis, the numbers and detected rate of immature granulocytes, monocyte detected rate, the granules scarce in all stage of granulocytic series in bone

  18. Case 40. Misdiagnosis of refractory macrocytic anemia.

    Science.gov (United States)

    Stringaris, Kate; Bain, Barbara

    2008-11-01

    A diagnosis of myelodysplastic syndrome, refractory anemia subtype, was made in an elderly Indian woman on the basis of a refractory macrocytic anemia with normal vitamin B(12) and folate assays, normal thyroid function, essentially normal liver function and normal cytogenetic analysis. Disease evolution revealed that the diagnosis was erroneous.

  19. Silent Infarcts with Sickle Cell Anemia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-01-01

    Full Text Available 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.

  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. 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...

  2. 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...

  3. Anemia in pregnancy:laboratory diagnostic procedures

    OpenAIRE

    Panova, Gordana

    2010-01-01

    Anemia is common condition in pregnancy and laboratory diagnostic procedures are essential for its detection and categorization. Complete blood count, serum iron, TIBC and ferritin are basic clinical laboratory parameters that provide information about iron status of mother’s body and possible presence of anemia.

  4. Salmonella osteomyelitis by sickle cell anemia

    Energy Technology Data Exchange (ETDEWEB)

    Rausch, H.; Tran, V.T.; Boeckmann, U.

    1985-10-01

    Case report of a 28 year old black sickle cell anemia patient with salmonella osteomyelitis of the radius. Aside from sickle cell anemia patients this skeletal complication of enteric salmonellosis is an extreme rarity. Description of the typical roentgenological features includes intracortical fissures and sequestration.

  5. Duodenal Amyloidosis Masquerading as Iron Deficiency Anemia

    Science.gov (United States)

    Hurairah, Abu

    2016-01-01

    The present study is a unique illustration of duodenal amyloidosis initially manifesting with iron deficiency anemia. It underscores the importance of clinical suspicion of amyloidosis while performing upper gastrointestinal endoscopy with a biopsy to establish the definite diagnosis in patients with unexplained iron deficiency anemia. PMID:27625911

  6. Transplante renal na anemia falciforme

    OpenAIRE

    Friedrisch, Joao Ricardo; Barros, Elvino José Guardão; Manfro, Roberto Ceratti; Bittar,Christina Matzenbacher; Silla, Lucia Mariano da Rocha

    2003-01-01

    Embora a anemia falciforme e as síndromes falciformes freqüentemente causem várias alterações funcionais renais, não é comum a insuficiência renal terminal. Nestes casos, o transplante renal é uma alternativa que se acompanha de resultados comparáveis aos obtidos em receptores sem hemoglobinopatias. Esta estratégia terapêutica tem sido, no entanto, pouco relatada para portadores de hemoglobinopatia SC. Este relato descreve a evolução de dois pacientes portadores de hemoglobinopatia SC que for...

  7. 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.

  8. 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.

  9. 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.

  10. 老年贫血218例病因分析%Etiopathogenisis analysis of 218 cases of anemia in the elderly

    Institute of Scientific and Technical Information of China (English)

    高丽霞; 吴克雄; 冀红红; 谢静荣

    2010-01-01

    目的 探讨老年贫血的诊断特点.方法 分析218例老年贫血病例的血常规、骨髓红系增生情况、骨髓形态学及病理学诊断、病因诊断等项目为观察指标.结果 218例老年贫血病例中,男性数量稍多于女性,中位年龄67.9岁.中重度贫血占绝大多数,形态学上以小细胞性贫血比例最高,占42.66%;其次为正细胞性贫血,占35.85%.骨髓红系增生活跃以上者占82.35%;缺铁性贫血为老年贫血最常见的病种(39.44%),其次为巨幼细胞性贫血、骨髓增生异常综合征.缺铁性贫血的病因中摄入不足最常见,占25.66%,肿瘤、溃疡及胃大部切除术后也较多见.骨髓增生异常综合征、巨幼细胞性贫血和慢性再生障碍性贫血为老年全血细胞减少病例的常见病种.结论 老年人属弱势群体,老年贫血病例中缺铁性贫血和营养不良性贫血比例高.%Objective To explore the diagnostic characteristics of anemia in the elderly. Methods To measure and analyze the eric status of bone marrow, diagnosis according to morphology and biopsy of bone marrow, and etiology of anemia(IDA) in 218 cases of anemia in the aged. Results In these 218 cases male patients were slightly more than female patients. Median age was 68.9. Ratio of cases with macrocytic anemia was the highest(42.66%), ratio of cases with active erythropoietic status was 82. 35%. Iron deficiency anemia was the most common anemia in the elderly(39. 44%), and megaloblastic anemia and myelodysplastic syndrome (MDS) were also frequent. Insufficient ingestion was the most common cause of IDA(25.66 %),and tumors,digestive ulcers and postgastrectomy were also the common causes. MDS,megaloblastic anemia and chronic aplastic anemia(CAA) were the common anemia in geriatric population with pancytopenia. Conclusions Geriatric population is the fragile group. Ratio of cases with anemia due to IDA and malnutrition is high in cases of anemia in the elderly.

  11. Iron Deficiency, Anemia and Mortality in Renal Transplant Recipients

    NARCIS (Netherlands)

    Eisenga, Michele F.; Minovic, Isidor; Berger, Stefan P.; Kootstra-Ros, Jenny E.; van den Berg, Else; Riphagen, Ineke J.; Navis, Gerjan; van der Meer, Peter; Bakker, Stephan J. L.; Gaillard, Carlo A. J. M.

    2016-01-01

    Anemia, iron deficiency anemia (IDA), and iron deficiency (ID) are highly prevalent in renal transplant recipients (RTR). Anemia is associated with poor outcome, but the role of ID is unknown. Therefore, we aimed to investigate the association of ID, irrespective of anemia, with all-cause mortality

  12. Iron Deficiency, Anemia and Mortality in Renal Transplant Recipients

    NARCIS (Netherlands)

    Eisenga, Michele F; Minovic, Isidor; Berger, Stefan P; Kootstra-Ros, Jenny E; van den Berg, Else; Riphagen, Ineke J; Navis, Gerjan; van der Meer, Peter; Bakker, Stephan J L; Gaillard, Carlo A J M

    2016-01-01

    Anemia, iron deficiency anemia (IDA) and iron deficiency (ID) are highly prevalent in renal transplant recipients (RTR). Anemia is associated with poor outcome, but the role of ID is unknown. Therefore, we aimed to investigate the association of ID, irrespective of anemia, with all-cause mortality i

  13. 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. .

  14. [Sideropenic anemia in infants and toddlers].

    Science.gov (United States)

    Jakovljević, G; Votava-Raić, A; Tjesić-Drinković, D; Rajić, L; Femenić-Kes, R; Konja, J; Goluza, I; Bilić, E; Leskovar, V

    2001-01-01

    Iron-deficiency anemia is the most common anemia in infants. In the routine pediatric care this problem is encountered every day. Numerous factors in infancy (low birth weight, rapid growth, insufficient nutrition) are involved in the development of iron-deficiency anemia, and they must be considered when establishing diagnosis, counselling parents, and prescribing oral iron preparations. Data on 119 patients aged up to two years treated in the Division of Gastroenterology and Nutrition, Pediatric Department, University Hospital Centre Zagreb Salata between 1994 and 1999, were analyzed. We were prompted to do so because of great frequency of iron-deficiency anemia as one of associated diagnoses, and unfortunately often the only diagnosis requiring hospitalization. Out of 119 patients with iron-deficiency anemia, nine (7.7%) patients (four premature newborns and three from twin pregnancy) had to receive transfusion of erythrocyte concentrate due to very bad general condition and low red blood count, accompanied by clinical signs of anemic hypoxia. We also analyzed prenatal and perinatal history, socioeconomic living conditions of these children, i.e. their nutrition, and if they had previously received oral iron preparations. Some of the results, such as inadequate alimentation with flour, as well as insufficient prophylaxis of iron deficiency, which were found in most cases of severe anemia, point to the need of paying greater attention to this problem, better parents education, and more adequate screening for iron deficiency anemia.

  15. Iron deficiency anemia in the elderly

    Directory of Open Access Journals (Sweden)

    Indra Kurniawan

    2011-02-01

    Full Text Available The numbers of older people in the world have been growing rapidly. Anemia is the most common hematologic problem encountered in older adults. However, anemia should not be accepted as an inevitable consequence of aging. Anemia in the elderly signifies an underlying disease. Iron Defi ciency Anemia (IDA is being one of the most common causes of anemia in older people. IDA in the elderly is often associated with such non specific symptoms. The diagnosis of IDA is typically based on laboratory results. Hence, the utilization of the various laboratory tests plays an important role for the diagnosis of IDA. The presence of IDA in the elderly is usually related with gastrointestinal disorders. Thus,  gastrointestinal evaluation should be contemplated in all patients with IDA unless there is a history of clinically important non gastrointestinal blood loss. Older people with IDA should have iron supplementation both to correct anemia and to replenish body iron stores. However, the underlying cause should always be treated to prevent further iron loss. (Med J Indones 2011; 20:71-7Keywords: anemia, elderly, gastrointestinal, iron deficiency

  16. Inborn anemias in mice: (Annual report, 1981-1982)

    Energy Technology Data Exchange (ETDEWEB)

    Bernstein, S.E.

    1982-07-19

    Hereditary anemias of mice are the chief objects of investigation, specificially four macrocytic anemias, 3 types of hemolytic anemia, nonhemolytic microcytic anemia, transitory siderocytic anemia, sex-linked iron-transport anemia, the autoimmune hemolytic anemia of NZB mice, an ..cap alpha..-thalassemia and a new hypochromic anemia with hemochromatosis. New types of anemia may be analyzed as new mutations appear. Three new mutations have been identified during the past 18 months. These anemias are studied through characterization of peripheral blood values, determinations of radiosensitivity under a variety of conditions, measurements of iron metabolism and heme synthesis, study of normal and abnormal erythrocyte membrane proteins, histological and biochemical characterization of blood-forming tissue, functional tests of the stem-cell component, examination of responses to erythroid stimuli, and transplantation of tissue and parabiosis between individuals of differently affected genotypes. 31 refs.

  17. 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 ...

  18. 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

  19. Prevalence of pernicious anemia in patients with macrocytic anemia and low serum B12

    OpenAIRE

    2014-01-01

    Objective: The current research evaluated the prevalence of pernicious anemia (PA) in patients with macrocytic anemia (high MCV) and low serum B12 in Riyadh. Methods: Blood testing was done in 77 patients (males: 45.5%, females: 54.5%) with macrocytic anemia; 84 patients; (males: 23.8%, females: 76.2%) with low serum B12 and 30 healthy subjects. Complete blood count, differential count, folic acid, vitamin B12, intrinsic factor, gastric parietal cell antibodies and holotranscobalamin II were ...

  20. Immune-mediated hemolytic anemia.

    Science.gov (United States)

    Rosse, Wendell F; Hillmen, Peter; Schreiber, Alan D

    2004-01-01

    Hemolytic anemia due to immune function is one of the major causes of acquired hemolytic anemia. In recent years, as more is known about the immune system, these entities have become better understood and their treatment improved. In this section, we will discuss three areas in which this progress has been apparent. In Section I, Dr. Peter Hillmen outlines the recent findings in the pathogenesis of paroxysmal nocturnal hemoglobinuria (PNH), relating the biochemical defect (the lack of glycosylphosphatidylinositol [GPI]-linked proteins on the cell surface) to the clinical manifestations, particularly hemolysis (and its effects) and thrombosis. He discusses the pathogenesis of the disorder in the face of marrow dysfunction insofar as it is known. His major emphasis is on innovative therapies that are designed to decrease the effectiveness of complement activation, since the lack of cellular modulation of this system is the primary cause of the pathology of the disease. He recounts his considerable experience with a humanized monoclonal antibody against C5, which has a remarkable effect in controlling the manifestations of the disease. Other means of controlling the action of complement include replacing the missing modulatory proteins on the cell surface; these studies are not as developed as the former agent. In Section II, Dr. Alan Schreiber describes the biochemistry, genetics, and function of the Fc gamma receptors and their role in the pathobiology of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura due to IgG antibodies. He outlines the complex varieties of these molecules, showing how they vary in genetic origin and in function. These variations can be related to three-dimensional topography, which is known in some detail. Liganding IgG results in the transduction of a signal through the tyrosine-based activation motif and Syk signaling. The role of these receptors in the pathogenesis of hematological diseases due to IgG antibodies is

  1. [A simple algorithm for anemia].

    Science.gov (United States)

    Egyed, Miklós

    2014-03-09

    The author presents a novel algorithm for anaemia based on the erythrocyte haemoglobin content. The scheme is based on the aberrations of erythropoiesis and not on the pathophysiology of anaemia. The hemoglobin content of one erytrocyte is between 28-35 picogram. Any disturbance in hemoglobin synthesis can lead to a lower than 28 picogram hemoglobin content of the erythrocyte which will lead to hypochromic anaemia. In contrary, disturbances of nucleic acid metabolism will result in a hemoglobin content greater than 36 picogram, and this will result in hyperchromic anaemia. Normochromic anemia, characterised by hemoglobin content of erythrocytes between 28 and 35 picogram, is the result of alteration in the proliferation of erythropoeisis. Based on these three categories of anaemia, a unique system can be constructed, which can be used as a model for basic laboratory investigations and work-up of anaemic patients.

  2. Sideroblastic anemias: variations on imprecision in diagnostic criteria, proposal for an extended classification of sideroblastic anemias.

    Science.gov (United States)

    Koc, S; Harris, J W

    1998-01-01

    Sideroblastic anemias are caused by a diversity of hereditary, congenital, or acquired disorders. Criteria used in describing sideroblastic anemias vary widely among standard medical textbooks and even so have been imprecisely applied in the literature. Recent discoveries concerning the basic pathophysiologic mechanisms involving the molecular biology of nuclear and mitochondrial DNA, erythroid ALA synthase (ALAS-2), and iron transport have made the classification of sideroblastic anemias very complex. We recommend a more precise evaluation and documentation of the components that characterize the sideroblastic abnormality and propose an extended classification of the sideroblastic anemias.

  3. Anemia of Inflammation and Chronic Disease

    Science.gov (United States)

    ... and Prevention website. www.cdc.gov/chronicdisease/overview/index.htm . Updated August 13, 2012. Accessed July 24, 2013. [3] Besarab A, Coyne DW. Iron supplementation to treat anemia in patients with chronic kidney disease. Nature Reviews ...

  4. Iron-Deficiency Anemia and Stroke

    OpenAIRE

    J Gordon Millichap

    2007-01-01

    The prevalence of iron-deficiency anemia (IDA) in young children at the time of stroke and in age-matched healthy controls was compared in a case-control study conducted at the Hospital for Sick Children, Toronto, Canada.

  5. Role of Complement in Autoimmune Hemolytic Anemia.

    Science.gov (United States)

    Berentsen, Sigbjørn

    2015-09-01

    The classification of autoimmune hemolytic anemias and the complement system are reviewed. In autoimmune hemolytic anemia of the warm antibody type, complement-mediated cell lysis is clinically relevant in a proportion of the patients but is hardly essential for hemolysis in most patients. Cold antibody-mediated autoimmune hemolytic anemias (primary cold agglutinin disease, secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria) are entirely complement-mediated disorders. In cold agglutinin disease, efficient therapies have been developed in order to target the pathogenic B-cell clone, but complement modulation remains promising in some clinical situations. No established therapy exists for secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria, and the possibility of therapeutic complement inhibition is interesting. Currently, complement modulation is not clinically documented in any autoimmune hemolytic anemia. The most relevant candidate drugs and possible target levels of action are discussed.

  6. I-131 therapy for hyperthyroidism in carbimazole induced acute aplastic anaemia

    Energy Technology Data Exchange (ETDEWEB)

    Jenkin, B.A.; Van Every, B.; Kelly, M.J. [Alfred Hospital, Prahran, VIC (Australia). Nuclear Medicine Department

    1998-06-01

    Full text: We present a case of a 39 year old previously well woman who developed carbimazole-induced acute aplastic anaemia. Following 6 weeks of carbimazole for Grave`s disease she presented with fever, sore throat and lethargy and was found to be pancytopaenic. Haemoglobin fell to 79g/l (normal>120g/l), neutrophils to an undetectable level and platelets to 4,000/ml (normal>150,000/ml). From the MIRD schema it was estimated that a therapeutic dose of 444 MBq (12mCi) to treat her hyperthyroidism would deliver a red marrow radiation dose of about 30 mSv, at least an order of magnitude below any expected demonstrable deleterious effect on the marrow. Subsequently a dose of 444 MBq of I-131 was given and within 2 weeks the marrow had begun to recover, eventually to normal. This case would support the use of 1-131 for hyperthyroidism in carbimazole-induced marrow aplasia, even in profoundly depressed marrow

  7. [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.

  8. Iron deficiency or anemia of inflammation?

    OpenAIRE

    Nairz, Manfred; Theurl, Igor; Wolf, Dominik; Weiss, Günter

    2016-01-01

    Summary Iron deficiency and immune activation are the two most frequent causes of anemia, both of which are based on disturbances of iron homeostasis. Iron deficiency anemia results from a reduction of the body’s iron content due to blood loss, inadequate dietary iron intake, its malabsorption, or increased iron demand. Immune activation drives a diversion of iron fluxes from the erythropoietic bone marrow, where hemoglobinization takes place, to storage sites, particularly the mononuclear ph...

  9. A case report of mercury poisoning relative aplastic anemia%1例汞中毒并发再生障碍性贫血

    Institute of Scientific and Technical Information of China (English)

    菅向东; 隋宏; 阚宝甜; 张玲; 郭广冉

    2006-01-01

    目的 报告1例汞中毒并发再生障碍性贫血,为汞中毒的防治研究积累经验和临床资料.方法 对我们临床诊治的1例汞中毒并发再生障碍性贫血病例进行综合分析.结果 汞中毒可能引起再生障碍性贫血.结论 对于汞的血液损害应引起重视.

  10. Clinical analysis of 20 pregnancy and aplastic anemia%妊娠合并再生障碍性贫血20例临床分析

    Institute of Scientific and Technical Information of China (English)

    沈晔

    2000-01-01

    目的探讨如何正确处理再生障碍性贫血(再障)合并妊娠.方法回顾性分析20例妊娠合并再障的临床资料.结果妊娠前已患再障者 17例,其中12例孕期有症状.7例合并重度妊高征,10例有低蛋白血症.12例(63.0%)剖宫产分娩,7例阴道分娩.20例中无产妇死亡、无产褥感染.除1例中期流产及1例与再障无直接关连的新生儿死亡外,18例均获活婴.结论再障病人如果需要,是能够耐受妊娠与分娩的,但需对此种患者加强孕期监护以早期发现并治疗并发症.分娩方式不必过分强调阴道分娩.产后尽早使用宫缩剂及抗生素,以预防产后出血及感染,并警惕可能发生的颅内出血及心力衰竭.

  11. 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.

  12. An Approach to Iron-Deficiency Anemia

    Directory of Open Access Journals (Sweden)

    Imran Rasul

    2001-01-01

    Full Text Available Iron-deficiency anemia is a common reason for referral to a gastroenterologist. In adult men and postmenopausal women, gastrointestinal tract pathology is often the cause of iron-deficiency anemia, so patients are frequently referred for endoscopic evaluation. Endoscopy may be costly and at times difficult for the patient. Therefore, physicians need to know what lesions can be identified reliably and, more importantly, the importance of ruling out life-threatening conditions such as occult malignancy. Over the past decade, a number of prospective studies have been completed that examined the yield of endoscopy in the investigation of iron-deficiency anemia. The present article provides a broad overview of iron-deficiency anemia, with particular emphasis on hematological diagnosis, etiology, the use of endoscopy in identifying lesions and iron-repletion therapy. Other clinical scenarios, including assessment of patients on anti-inflammatory or anticoagulation therapy and patients with bleeding of obscure origin, are also addressed. The present article provides a diagnostic algorithm to iron-deficiency anemia, which describes a more systematic manner in which to approach iron-deficiency anemia.

  13. 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.

  14. Mitochondrial iron metabolism and sideroblastic anemia.

    Science.gov (United States)

    Sheftel, Alex D; Richardson, Des R; Prchal, Josef; Ponka, Prem

    2009-01-01

    Sideroblastic anemias are a heterogeneous group of disorders, characterized by mitochondrial iron overload in developing red blood cells. The unifying characteristic of all sideroblastic anemias is the ring sideroblast, which is a pathological erythroid precursor containing excessive deposits of non-heme iron in mitochondria with perinuclear distribution creating a ring appearance. Sideroblastic anemias may be hereditary or acquired. Hereditary sideroblastic anemias are caused by defects in genes present on the X chromosome (mutations in the ALAS2, ABCB7, or GRLX5 gene), genes on autosomal chromosomes, or mitochondrial genes. Acquired sideroblastic anemias are either primary (refractory anemia with ring sideroblasts, RARS, representing one subtype of the myelodysplastic syndrome) or secondary due to some drugs, toxins, copper deficiency, or chronic neoplastic disease. The pathogenesis of mitochondrial iron loading in developing erythroblasts is diverse. Ring sideroblasts can develop as a result of a heme synthesis defect in erythroblasts (ALAS2 mutations), a defect in iron-sulfur cluster assembly, iron-sulfur protein precursor release from mitochondria (ABCB7 mutations), or by a defect in intracellular iron metabolism in erythroid cells (e.g. RARS).

  15. 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.

  16. Iron deficiency anemia in celiac disease

    Science.gov (United States)

    Freeman, Hugh James

    2015-01-01

    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. PMID:26309349

  17. Cardio-renal-anemia syndrome: A report of three cases

    Directory of Open Access Journals (Sweden)

    U H Okafor

    2012-01-01

    Full Text Available Patients with chronic kidney diseases (CKDs and cardiovascular diseases (CVDs present with various degree of anemia. Anemia has been associated with poor outcome in patients with CKD and CVD. CVD is the commonest cause of morbidity and mortality in patients with CKD. CKD causes anemia and CVD, and this rapidly deteriorates when anemia is not corrected. This triad of CVD, CKD, and anemia has been termed cardio-renal-anemia syndrome. The objec-tive of this study is to highlight the importance of cardio-renal-anemia syndrome, their relation-ship, and management. Three patients with various stages of CKD who presented with anemia and cardiovascular abnormalities are reported. The patients responded well to various interventional measures, with improvement in their clinical and laboratory parameters. Cardio-renal-anemia syndrome is an entity that should be identified. Early and appropriate intervention leads to better outcome.

  18. 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.

  19. Iron, Anemia, and Iron Deficiency Anemia among Young Children in the United States

    Science.gov (United States)

    Gupta, Priya M.; Perrine, Cria G.; Mei, Zuguo; Scanlon, Kelley S.

    2016-01-01

    Iron deficiency and anemia are associated with impaired neurocognitive development and immune function in young children. Total body iron, calculated from serum ferritin and soluble transferrin receptor concentrations, and hemoglobin allow for monitoring of the iron and anemia status of children in the United States. The purpose of this analysis is to describe the prevalence of iron deficiency (ID), anemia, and iron deficiency anemia (IDA) among children 1–5 years using data from the 2007–2010 National Health and Nutrition Examination Survey (NHANES). Prevalence of ID, anemia, and IDA among children 1–5 years was 7.1% (5.5, 8.7), 3.2% (2.0, 4.3), and 1.1% (0.6, 1.7), respectively. The prevalence of both ID and anemia were higher among children 1–2 years (p < 0.05). In addition, 50% of anemic children 1–2 years were iron deficient. This analysis provides an update on the prevalence of ID, anemia, and IDA for a representative sample of US children. Our results suggest little change in these indicators over the past decade. Monitoring of ID and anemia is critical and prevention of ID in early childhood should remain a public health priority. PMID:27249004

  20. Fanconi's Anemia Effect or Sickle Cell Anemia Effect: That is the Question.

    Science.gov (United States)

    Unal, Sule; Chui, David H K; Gumruk, Fatma

    2015-01-01

    A 16-year-old boy who was diagnosed to have sickle cell anemia was referred to our center. The parental consanguinity, growth retardation and dysmorphic features prompted a search for possible Fanconi's Anemia (FA). The diepoxybutane (DEB) test was positive, confirming FA. The interaction of both diseases might account for his relatively mild phenotype in terms of both sickle cell anemia (or Hb S, HBB: c.20A > T) and FA. The high Hb F level that might be related to concomitant FA, may have caused a milder phenotype of sickle cell anemia, whereas nitric oxide (NO) depletion as a consequence of sickle cell anemia, may have caused a delay in the bone marrow failure of FA.