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Sample records for cell histiocytosis mimicking

  1. Oral manifestion of Langerhans cell histiocytosis mimicking inflammation

    Directory of Open Access Journals (Sweden)

    K S Divya

    2014-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare idiopathic disease characterized by the clonal proliferation of Langerhans cells. LCH affects five children per million population. The peak incidence is from 1 to 4 years of age. LCH involves the head and neck region quite commonly. Oral soft tissue lesions are also common. The differential diagnosis of oral LCH includes leukemia, neutropenia, prepubertal periodontitis, hypophosphatasia, fibrous dysplasia, and Papillon-Lefevre syndrome. The prognosis of LCH depends on early detection and appropriate management. Surgical management alone is used in 50% of cases with an additional 23% of the lesions being treated with both surgery and radiation therapy. A case of LCH in a 6-year-old girl involving the mid root level of developing first permanent molar with a floating developing tooth bud of permanent second molar mimicking an inflammation is reported.

  2. Generalized eruptive histiocytosis mimicking leprosy

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    B C Sharath Kumar

    2011-01-01

    Full Text Available Generalized eruptive histiocytosis (GEH is a rare cutaneous histiocytosis that mainly affects adults and presents with multiple symmetric papules on face, trunk, and proximal extremities. GEH is included in type IIa (histiocytes involving cells of dermal dendrocyte lineage of histiocytic disorders. Clinical and pathological correlations are required for differentiating GEH from other histiocytic disorders and from lepromatous leprosy which clinically mimic GEH and is prevalent in India. We report a case of a middle-aged woman who presented with generalized asymptomatic papules and nodules and was treated for leprosy but was finally diagnosed to have GEH after clinical, histopathological, and immunohistochemical correlation. Furthermore, the newer lesions also showed features of progressive nodular histiocytosis.

  3. Generalized eruptive histiocytosis mimicking leprosy.

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    Sharath Kumar, B C; Nandini, A S; Niveditha, S R; Gopal, M G

    2011-01-01

    Generalized eruptive histiocytosis (GEH) is a rare cutaneous histiocytosis that mainly affects adults and presents with multiple symmetric papules on face, trunk, and proximal extremities. GEH is included in type IIa (histiocytes involving cells of dermal dendrocyte lineage) of histiocytic disorders. Clinical and pathological correlations are required for differentiating GEH from other histiocytic disorders and from lepromatous leprosy which clinically mimic GEH and is prevalent in India. We report a case of a middle-aged woman who presented with generalized asymptomatic papules and nodules and was treated for leprosy but was finally diagnosed to have GEH after clinical, histopathological, and immunohistochemical correlation. Furthermore, the newer lesions also showed features of progressive nodular histiocytosis.

  4. Histiocytosis mimicking a pineal gland tumour

    International Nuclear Information System (INIS)

    We report an unusual case of isolated Langerhans cell histiocytosis of the central nervous system. A 19-year-old man presented with an incomplete ocular palsy. MRI revealed a solitary mass in the pineal gland with marked contrast enhancement. Complete microsurgical excision was followed by local radiotherapy. Histological examination revealed histiocytosis. Unifocal brain involvement by histiocytosis X is rare with few cases in the literature; the most commonly involved areas are the hypothalamus and the pituitary gland. (orig.)

  5. Histiocytosis mimicking a pineal gland tumour

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    Gizewski, E.R.; Forsting, M. [Dept. of Neuroradiology, Univ. of Essen (Germany)

    2001-08-01

    We report an unusual case of isolated Langerhans cell histiocytosis of the central nervous system. A 19-year-old man presented with an incomplete ocular palsy. MRI revealed a solitary mass in the pineal gland with marked contrast enhancement. Complete microsurgical excision was followed by local radiotherapy. Histological examination revealed histiocytosis. Unifocal brain involvement by histiocytosis X is rare with few cases in the literature; the most commonly involved areas are the hypothalamus and the pituitary gland. (orig.)

  6. Cutaneous T cell lymphoma mimicking cutaneous histiocytosis: differentiation by flow cytometry.

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    Baines, S J; McCormick, D; McInnes, E; Dunn, J K; Dobson, J M; McConnell, I

    2000-07-01

    A two-year-old, neutered female cross-bred labrador had multiple cutaneous nodules, biopsies of which revealed pathological changes consistent with cutaneous histiocytosis. During a period of one month the dog developed multicentric lymphadenopathy, a retrobulbar mass and masses within the quadriceps and cervical muscles. Fine needle aspiration cytology of the cutaneous nodules and lymph nodes and histological examination of the cutaneous nodules and muscle masses suggested the presence of lymphoblastic lymphoma. A definitive diagnosis of CD8+ T cell lymphoma was achieved by immunophenotyping the tumour cells by flow cytometry.

  7. A Gluteal Mass Of Langerhans Cell Histiocytosis Mimicking Malignancy In A Two-Year-Old Boy: A Case Report

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    Ibrahim, Zainal Abidin; Lung, Wong Siong; Long, Pan Kok

    2009-01-01

    Langerhans cell histiocytosis is a disease primarily affects the bone. More than 50 percent of the disease occurs between the age of 1 and 15. We reported a case of a 2 year old boy who presented with a gluteal mass. Radiographic imaging showed an osteolytic lesion suspicious of malignancy. However, the histological diagnosis was Langerhans cell histiocytosis.

  8. Langerhans cell histiocytosis (histiocytosis X).

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    Lam, K Y

    1997-01-01

    There has been a renewed interest in Langerhans cell histiocytosis in recent years due both to advances in basic research and to improvements in diagnostic and treatment approaches. In this article, we review the various aspects of the disease and the potential implications of these recent scientific researches for our understanding and management of the disorder.

  9. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

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    Anjan Madasu

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.

  10. Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

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    Madasu, Anjan; Noor Rana, Asim; Banat, Saleh; Humad, Hani; Mustafa, Rashid; AlJassmi, Abdulrahman Mohd

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported. Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever. PMID:26587301

  11. Histiocytosis

    Science.gov (United States)

    Histiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe disease

  12. Langerhans cell histiocytosis

    OpenAIRE

    Xiao-ling YAN

    2014-01-01

    A case report and literature review. We present a boy with a multisystemic presentation of Langerhans cell histiocytosis and severe pulmonary lesion.Skin lesion helped to suspect and confirm diagnosis by scalpbiopsy. Chemotherapy was important for favorable result.

  13. Benign isolated histiocytosis mimicking chicken pox in a neonate: report of two cases with ultrastructural study.

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    Valderrama, E; Kahn, L B; Festa, R; Lanzkowsky, P

    1985-01-01

    Two cases of benign isolated cutaneous histiocytosis X in newborn infants are reported. Clinically, the lesions mimicked chicken pox, and the histologic findings in skin biopsies were indistinguishable from those described in infantile Letterer-Siwe disease. Electron microscopy showed Langerhans' cells with the classical trilaminar, racket-shaped granules. Our cases a well as 10 of the 21 previously reported cases showed spontaneous resolution of the skin lesions. Since this is a benign spontaneous resolving histiocytosis, we recommend that no therapy be given provided that no other signs of systemic involvement are found.

  14. Langerhans cell histiocytosis

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    Aruna, D. R.; G Pushpalatha; Sushma Galgali; Prashanthy,

    2011-01-01

    Langerhans cell histiocytosis (LCH) is a group of rare disorders histologically characterized by the proliferation of Langerhans cells. Multiple organs and systems may be involved by the disease. Typically, there is bone involvement and, less frequently, lesions may be found in the lungs, liver, lymph nodes, skin, and mucosa. Oral soft tissue lesions without bone involvement are rare. We present a case of oral lesions associated with LCH in a young woman.

  15. Pulmonary langerhans cell histiocytosis

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    Suri Harpreet S; Yi Eunhee S; Nowakowski Gregorz S; Vassallo Robert

    2012-01-01

    Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events r...

  16. Indeterminate cell histiocytosis that presented clinically as benign cephalic histiocytosis.

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    Haimovic, Adele; Chernoff, Karen; Hale, Christopher S; Meehan, Shane A; Schaffer, Julie V

    2014-12-16

    Indeterminate cell histiocytosis (ICH) is a rare, heterogeneous disorder that is characterized by immunophenotypic features of both Langerhans cell histiocytosis (LCH) and non-LCH. We describe a 12-month-old boy with a four-month history of asymptomatic, small, pink-tan papules on his face. Histopathologic evaluation showed a superficial, dermal infiltrate of histiocytes that was positive for S100, CD1a, CD68, and Factor XIIIa. To our knowledge, this represents the first report of the clinical presentation of benign cephalic histiocytosis with immunohistochemical findings of ICH. We review the classification of histiocytic disorders and the clinical and immunohistochemical features of both ICH and benign cephalic histiocytosis.

  17. Adult Onset Langerhans’ Cell Histiocytosis

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    Rahime İnci; Hamide Sayar; Mehmet Fatih İnci; Perihan Öztürk

    2014-01-01

    Langerhans’ cell histiocytosis (LCH) is a group of diseases of unknown cause resulting from abnormal proliferation of bone marrow-originated dendritic cells called histiocytes. The incidence is between 0.5-5.4 per million. More common in childhood, it is extremely rare in adults. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease. In this article, the clinical, radiological and histopathological findings of a 51-year-old male patient...

  18. [Pulmonary Langerhans cell histiocytosis].

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    Popper, H H

    2015-09-01

    Pulmonary Langerhans cell histiocytosis is regarded as a reactive proliferation of the dendritic Langerhans cell population stimulated by chronic tobacco-derived plant proteins due to incomplete combustion but can also occur in childhood as a tumor-like systemic disease. Currently, both these forms cannot be morphologically distinguished. In the lungs a nodular proliferation of Langerhans cells occurs in the bronchial mucosa and also peripherally in the alveolar septa with an accompanying infiltration by eosinophilic granulocytes and destruction of the bronchial wall. Langerhans cells can be selectively detected with antibodies against CD1a and langerin. In the reactive isolated pulmonary form, abstinence from tobacco smoking in most patients leads to regression of infiltration and improvement of symptoms. In high-resolution computed tomography (HRCT) the small star-like scars can still be detected even after complete cessation of tobacco smoking. PMID:26289803

  19. Langerhans cell histiocytosis

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    Arpita J Nishal

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which Langerhans′s cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry findings suggested diagnosis of LCH.

  20. CASE REPORT OF LANGERHANS CELL HISTIOCYTOSIS

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    Umesh Reddy; Somaiah; Ravikanth; Navneeth Reddy; Gowthami

    2014-01-01

    : Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which pathological Langerhans cells (LCs) accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  1. Genetics Home Reference: Langerhans cell histiocytosis

    Science.gov (United States)

    ... Association Histio UK Histiocytosis Association of America National Organization for Rare Disorders Genetic Testing Registry (1 link) Langerhans cell histiocytosis, multifocal Scientific articles on PubMed (1 link) PubMed OMIM (1 link) ...

  2. Pulmonary Langerhans cell Histiocytosis.

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    Harari, S; Comel, A

    2001-10-01

    Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocytes, and their accumulation in the distal bronchioles; these cells express on their surface the CD1a and CD1c antigens, and the B7 molecule, essential for activating quiescent T lymphocytes. In its evolution the granuloma is characterized by the progressive reduction in the LC number, with the increase of fibrosis, surrounding and destroying the bronchiolar lumen; the remaining of the lumen, or the traction exerted by fibrous tissue on the adjacent alveolar spaces leads to the cyst development. Vascular involvement occurs frequently, and may explain the onset of pulmonary hypertension in advanced cases of the disease. The disease may be asymptomatic, or it may present with aspecific respiratory signs and symptoms, and has characteristic radiological findings, being included in the group of cysticaerial parenchymal alterations. The diagnosis could be suggested by the finding of a number of LC in BAL greater than 5%. Different therapies have been proposed, but it seems that the most important measure is smoking cessation. PMID:11587096

  3. Pulmonary langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Suri Harpreet S

    2012-03-01

    Full Text Available Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

  4. Adult Onset Langerhans’ Cell Histiocytosis

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    Rahime İnci

    2014-12-01

    Full Text Available Langerhans’ cell histiocytosis (LCH is a group of diseases of unknown cause resulting from abnormal proliferation of bone marrow-originated dendritic cells called histiocytes. The incidence is between 0.5-5.4 per million. More common in childhood, it is extremely rare in adults. In adults, pulmonary involvement with Langerhans’ cell histiocytosis usually occurs as a single-system disease. In this article, the clinical, radiological and histopathological findings of a 51-year-old male patient with both skin, bone and pulmonary involvement were presented and discussed with recent literature.

  5. Early-onset sarcoidosis mimicking refractory cutaneous histiocytosis.

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    Ohga, Shouichi; Ichino, Kiyomi; Urabe, Kazunori; Ishimura, Masataka; Takada, Hidetoshi; Nishikomori, Ryuta; Furue, Masutaka; Hara, Toshiro

    2008-03-01

    A 10-year-old female was diagnosed as having early-onset sarcoidosis (EOS) after a prolonged skin disease. A granuloma emerged on the face at age 2 and massive lesions extended to the rest of the body. Repeated biopsies indicated histiocytic proliferation. At age 7, fever, disseminated macular eruptions, and multinucleated giant cells in the bone marrow prompted vinblastine and prednisolone therapy. Five months after stopping therapy, hypercalcemic crisis occurred along with fever, cytopenias, and interferon-gamma-nemia indicating a macrophage activation syndrome. A biopsy of nodules confirmed the diagnosis of sarcoidosis. The atypical EOS should be differentiated from histiocytosis.

  6. Multifocal Skeletal Tuberculosis Mimicking Langerhans Cell Histiocytosis in a Child: a Case Report With a Long-Term Follow-Up

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    Haghighatkhah, Hamidreza; Jafroodi, Yousef; Sanei Taheri, Morteza; Pourghorban, Ramin; Sadeghian Dehkordy, Afarin

    2015-01-01

    Introduction: Multifocal skeletal tuberculosis is a rare condition that may masquerade as Langerhans cell histiocytosis, especially in children. Case Presentation: We report a case of multifocal osseous tuberculosis in a 5-year-old female patient admitted to our hospital with a complaint of low back pain but no history of respiratory symptoms or malaise. Radiological findings included vertebra plana and multiple lytic lesions in both the frontal and pelvic bones. An initial diagnosis of Langerhans cell histiocytosis was made based on imaging findings; however, the patient underwent further evaluation for Mycobacterium tuberculosis, and histopathologic findings confirmed the diagnosis of tuberculosis. The patient showed a nearly complete response after receiving a course of anti-tuberculosis drugs. Conclusions: A high index of suspicion is required for the early diagnosis and prompt treatment of patients with osseous tuberculosis. Given the high prevalence of tuberculosis in developing countries, tuberculosis should be considered in the differential diagnosis of multifocal lytic lesions and vertebra plana, especially in children. PMID:26744631

  7. CASE REPORT OF LANGERHANS CELL HISTIOCYTOSIS

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    Umesh Reddy

    2014-08-01

    Full Text Available : Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which pathological Langerhans cells (LCs accumulate in a variety of organs. This report describes a boy with Langerhans' cell histiocytosis who presented with primarily soft tissue swellings, without pain. A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  8. Langerhans Cell Histiocytosis in Otorhinolaryngology

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    Fernandes, Humberto de Barros

    2009-12-01

    Full Text Available Introduction: The Langerhans cell histiocytosis is an inflammatory cells proliferative disorder of unknown etiology. It is uncommon disease in children. Objective: To proceed with a literature review on Langerhans cell histiocytosis, with focus on the otorhinolaryngological complications. Materials and methods: The methodology used was advised based on online data from MEDLINE, between 1966 and 2008, with research of terms related to Langerhans cell histiocytosis, temporal bones and otorhinolaryngology. Literature Review: The manifestations in the head and neck are the most common ones and their diagnosis becomes difficult once it mimetizing other more common diseases the otorhinolaryngologist sees as external ear eczema, acute mastoiditis and gingivitis. The temporal bone disease manifests as recurrent otorrhea and external auditory meatus and retroauricular granulomas. The radiological evaluation confirms lytic lesions especially in the cranial cap, jaw, temporal bones and spines. The definitive diagnosis is made by biopsy through the histopathological discoveries and immunohistochemistry detection of the CD1a antigen. The main form of treatment is by chemotherapy and, in a lower scale, radiotherapy or surgery. Conclusions: he otorhinolaryngological manifestations must be suspected for recurrent otological symptoms and the presence of retroauricular granulation tissue or and the external auditory meatus. The biopsy with characteristic histological discoveries and immunohistochemistry positive for CD1a were diagnostic. The chemotherapy may be the initial treatment in most cases or secondary in refractory or recurrent forms.

  9. The histopathology of Langerhans cell histiocytosis.

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    Favara, B. E.; Jaffe, R.

    1994-01-01

    Selected aspects of the histopathology of Langerhans cell histiocytosis representing diagnostic difficulty and/or controversy are presented with emphasis on the composition of pathological lesions. Lesional cell phenotypes and the factors influencing variations are noted. Features of several skin-based histiocytic disorders, dermatopathic lymphadenopathy and Rosai-Dorfman disease are compared. Associations between Langerhans cell histiocytosis and juvenile xanthogranuloma and malignant disord...

  10. Multisystem Langerhans' cell histiocytosis with pancreatic involvement.

    OpenAIRE

    Yu, R C; Attra, A; Quinn, C M; Krausz, T; Chu, A C

    1993-01-01

    Langerhans' cell histiocytosis, a rare disorder of unknown cause affecting both children and adults, can affect many different organs and present to a wide range of medical specialties. An infant with fatal multisystem Langerhans' cell histiocytosis in whom the pancreas and the intestine were extensively affected is reported. The direct pancreatic involvement by this disease has not previously been described.

  11. Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

    International Nuclear Information System (INIS)

    Pulmonary Langerhans' cell histiocytosis is an uncommon granulomatous disorder of unknown cause. The authors retrospectively evaluated radiography and computed tomographic findings of five patients with biopsy proven pulmonary Langerhans' cell histiocytosis. The main structural abnormalities consisted of small nodules and cystic air spaces, but one cases showed only pneumothorax due to bullae rupture. Its distribution has been known predominantly in the upper lung fields, but in our cases, the lung lesions were distributed in the entire lung fields or predominently in the lower lung fields. We propose that pulmonary Langerhans' cell histiocytosis is extremely variable of its structural abnormalities and distribution

  12. Cerebral and brain stem Langerhans cell histiocytosis

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    Breidahl, W.H. (Dept. of Radiology, Royal Perth Hospital, Nedlands (Australia)); Ives, F.J. (Dept. of Radiology, Royal Perth Hospital, Nedlands (Australia)); Khangure, M.S. (Dept. of Magnetic Resonance Imaging, Sir Charles Gairdner Hospital, Nedlands (Australia))

    1993-05-01

    Two patients with central nervous system manifestations of Langerhans cell histiocytosis, both with brain stem involvement, are reported. The onset of symptoms was at an age when the diagnosis might not have been considered. (orig.)

  13. Cerebral and brain stem Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Two patients with central nervous system manifestations of Langerhans cell histiocytosis, both with brain stem involvement, are reported. The onset of symptoms was at an age when the diagnosis might not have been considered. (orig.)

  14. Recurrence of recipient Langerhans' cell histiocytosis following bilateral lung transplantation

    OpenAIRE

    Habib, S.; Congleton, J; Carr, D; Partridge, J; Corrin, B.; Geddes, D; Banner, N.; Yacoub, M; Burke, M.

    1998-01-01

    Langerhans' cell histiocytosis may cause irreversible respiratory failure due to progressive destruction of lung parenchyma and widespread cystic change. Transplantation offers a therapeutic option. A case is described of recurrence of Langerhans' cell histiocytosis which was associated with deterioration in lung function four years following bilateral lung transplantation. Patients transplanted for Langerhans' cell histiocytosis should be followed up with this complication in min...

  15. Langerhans Cell Histiocytosis Involving Maxilla and Mandible

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    M. Guna Shekhar

    2009-06-01

    Full Text Available Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults. Jaws are involved in less than 10% of children with the disease while mandibular involvement in young children is uncommon and bilateral affection is very rare. The purpose of this report is to describe a unique and very rare case of simultaneous and bilateral occurrence of Langerhans cell histiocytosis in both the jaws of a four-year-old boy.

  16. Langerhans cell histiocytosis followed by Hodgkin's lymphoma.

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    Park, Ik Soo; Park, In Keun; Kim, Eun Kyoung; Kim, Shin; Jeon, Sang Ryong; Huh, Joo Ryung; Suh, Cheol Won

    2012-12-01

    A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurrence was observed on follow-up imaging. This report discusses the association between Langerhans cell histiocytosis and Hodgkin's lymphoma. PMID:23269889

  17. Indeterminate cell histiocytosis: report of a case.

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    Alireza Ghanadan

    2014-10-01

    Full Text Available Indeterminate cell histiocytosis is a very rare disorder of histiocytes proliferation. It has both Langerhans and non-Langerhans cell histiocytosis immunophenotypic features. We described a 45-year-old man with a 2 years history of multiple yellow-brown papules and a few red nodules on his trunk and extremities. No internal involvement was detected first and after 8 months. As his lesions were asymptomatic and sparse, he didn't receive any treatment. He will be followed at regular intervals.

  18. Langerhans' cell histiocytosis in an adult.

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    Lindelöf, B; Forslind, B; Hilliges, M; Johansson, O; Aström, L

    1991-01-01

    A woman with typical skin lesions of histiocytosis X is reported. Electron microscopic and immunohistochemical investigations revealed a large number of markedly long Birbeck Langerhans' cell granulae. During treatment with Interferon alpha -2b, the patient developed infarctus cerebri and died. PMID:1675535

  19. Langerhans cell histiocytosis of the sacrum

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    M.A. Hatem

    2014-01-01

    Full Text Available Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  20. Langerhans cell histiocytosis of the sacrum

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    Hatem, M.A.

    2015-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age. PMID:27186251

  1. Langerhans cell histiocytosis of the sacrum

    OpenAIRE

    Hatem, M.A.

    2014-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  2. Langerhans Cell Histiocytosis and Frontal Headache

    OpenAIRE

    J Gordon Millichap

    2002-01-01

    A most recent case record of the Massachusetts General Hospital involved a clinicopathological presentation of a 15-year-old boy admitted with frontal headache, right retro-orbital pain, and a retro-orbital mass that proved to be a Langerhans’ cell histiocytosis involving the right sphenoid sinus and orbit.

  3. Langerhans cell histiocytosis of the sacrum

    OpenAIRE

    Hatem, M.A.

    2015-01-01

    Langerhans cell histiocytosis is a rare disease with a wide spectrum of clinical presentations. It is a multisystemic disease with organ system involvement ranging from simple—where it involves only one organ—to widespread progressive disease. Although it can affect any age group, the peak incidence is between 1 and 3 years of age.

  4. [Pulmonary manifestations of Langerhans cell histiocytosis].

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    Obert, J; Tazi, A

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is a rare diffuse cystic interstitial pneumonia of unknown etiology that occurs selectively in young smokers of both genders. The multicenter studies conducted by the reference center have better defined the short and medium terms natural history of the disease and the clinical management of patients. A substantial proportion of patients experience a dramatic decline in their lung function soon after diagnosis. Importantly, smoking cessation is associated with a decreased risk of subsequent deterioration. Cladribine, a purine analogue, chemotherapy may dramatically improve lung function in patients with progressive pulmonary Langerhans cell histiocytosis, but this treatment should be used only in the setting of clinical research. Specific pulmonary hypertension therapies (anti-endothelin receptors, inhibitors of phosphodiesterases) may be used with caution in specialized centres for patients with severe pulmonary hypertension, and seem to be well tolerated. The recent identification of the V600E mutation of the BRAF oncogene in approximately half of the Langerhans cell histiocytosis lesions, including pulmonary granulomas, represents an important step forward in the understanding of the pathogenesis of Langerhans cell histiocytosis. Potentially it opens the way to targeted therapies. PMID:26003197

  5. Langerhans Cell Histiocytosis of Thyroid—A Diagnostic Dilemma

    OpenAIRE

    Pandyaraj, R. A.; Sathik Mohamed Masoodu, K.; Maniselvi, S.; S Savitha; Divya Devi, H.

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease of antigen presenting cells and involvement of thyroid is really uncommon. The thyroid if involved is usually seen in multisystem disease but isolated thyroid involvement is very rare. The diagnosis of Langerhans cell histiocytosis of the thyroid is very challenging due to its rarity and is usually misdiagnosed as benign goiters, undifferentiated carcinoma, lymphoma, etc. Management of Langerhans cell histiocytosis of the thyroid also rema...

  6. Langerhans Cell Histiocytosis in 2 Cases of Cholestasis

    OpenAIRE

    Erkan, Tülay; Kutlu, Tufan; Çokuğraş, Fügen; Ceyhan, İpek; Yıldız, İnci; Özbay, Gülşen; T.Tümay, Güngör

    1995-01-01

    Two cases of Langerhans cell histiocytosis who had multiorgan involvement with obstructive jaundice anemia and hepatosplenomegaly painful ulceration purulent exudation squamous and maculopapular rash in the skin were presented The diagnosis of Langerhans cell histiocytosis was made by skin biopsies that showed the presence of S 100 staining cells We suggest that the diagnosis of Langerhans cell histiocytosis should be considered in patients presenting with clinical and biochemical evidence of...

  7. Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

    Science.gov (United States)

    Gholami, Narges

    2016-01-01

    Introduction Langerhans cell histiocytosis (LCH) is a proliferative disorder of histiocytes in multiple organs. Langerhans cell histiocytosis involves bones, skin, lung and other organs. Case Presentation This study describes a seven-month-old Iranian girl who presented with skin rash and cervical lymphadenopathy. Langerhans cell histiocytosis was suspected when it was associated with anemia, splenomegaly and lytic bone lesions. A skin biopsy confirmed the diagnosis of Langerhans cell histiocytosis. During hospitalization, the patient looked ill with respiratory distress. A chest X-ray showed a ground glass view, and echocardiography showed moderate pericardial effusion. Conclusions Pericardial effusion was a rare finding in this case of Langerhans cell histiocytosis. Pericardial effusion in Langerhans cell histiocytosis, which is an unusual presentation, should be considered when the patient experiences respiratory distress. PMID:27621925

  8. Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

    OpenAIRE

    Rawlins, Frederic A.; Hull, James E.; Morgan, Julia A.; Morris, Michael J.

    2013-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but...

  9. Bronchoscopic Diagnosis of Langerhans Cell Histiocytosis and Lymphangioleiomyomatosis

    OpenAIRE

    Harari, Sergio; Torre, Olga; Cassandro, Roberto; Taveira-DaSilva, Angelo M.; Moss, Joel

    2012-01-01

    Limited data are available regarding the role of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBB) as diagnostic tools in pulmonary Langerhans’ Cell Histiocytosis (LCH) and lymphangioleiomyomatosis (LAM).

  10. Langerhans' Cell Histiocytosis (Histiocytosis X) of the Temporal Bone

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Objectives Langerhans' Cell histiocytosis (LCH) is a rare disease, which remains poorly understood and whose cellular origin remains unknown. To increase understanding of temporal bone LCH, it is necessary to study recent advances in the diagnosis and treatment of this disease. Methods The long term(5 to 30 years) results of 21 temporal bone LCH cases treated between 1973 and 2003 were reviewed. Surgery, radiotherapy,pharmacologic therapy or a combination of these treatments were employed in these cases. Results Eighteen patients were cured (18/21, 85%). Six patients developed residual diabetes insipidus (DI) and dwarfism (28%).Three patients died (14%). Conclusions The Alessi classification system for LCH based on the extent of disease accurately predicts prognosis and is a useful guide in selecting treatment methodologies. X-ray, computed tomography and magnetic resonance imaging have proved useful in defining the extent of osseous and soft tissue diseases. Diagnosis of LCH is based on clinical presentations, radiographic findings and histopathological results.Surgery and radiotherapy are the main treatment modalities. Pharmacologic therapy should be used in patients with aggressive, disseminate, and refractory lesions. LCH has a predilection for children and prognosis depends on age and extent of vital organ involvement.

  11. Langerhans cell histiocytosis of the sternum

    OpenAIRE

    Tsuchie, Hiroyuki; Okada, Kyoji; Nagasawa, Hiroyuki; Yano, Michihiro; Nanjyo, Hiroshi; SHIMADA Yoichi

    2009-01-01

    We report a rare case of Langerhans cell histiocytosis involving the sternum. The patient was a 12-year-old girl presenting with anterior chest pain and swelling. Radiographs and computed tomography showed an osteolytic lesion in the sternum. Technetium bone scintigraphy revealed increased uptakes in the sternum, the greater trochanter of the right femur, and the right distal tibia. Incisional biopsy for the sternum lesion was performed, and the histopathologic diagnosis was Langerhans cell h...

  12. Multisystem langerhans cell histiocytosis in adult

    OpenAIRE

    Anubhav Garg; Pramod Kumar

    2012-01-01

    Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy sh...

  13. Langerhans cell histiocytosis in sequential discordant lymphoma

    OpenAIRE

    Adu-Poku, K; Thomas, D. W.; Khan, M. K.; Holgate, C S; Smith, M E F

    2005-01-01

    B cell non-Hodgkin lymphoma of the follicular subtype (grade 3/3) affecting the nasopharynx and breast, and containing foci of Langerhans cell histiocytosis, was diagnosed in a 56 year old white woman who was a longstanding heavy smoker. Four years before this she had developed stage 1a mixed cellularity Hodgkin lymphoma affecting the right inguinal region, which was treated by irradiation and chemotherapy without recurrence. Review of the original Hodgkin lymphoma histology demonstrated a sm...

  14. Langerhans Cell Histiocytosis Followed by Hodgkin's Lymphoma

    OpenAIRE

    Park, IK Soo; Park, In Keun; Kim, Eun Kyoung; Kim, Shin; Jeon, Sang Ryong; Huh, Joo Ryung; Suh, Cheol Won

    2012-01-01

    A 22-year-old man was referred to our institution due to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar spine. The patient achieved complete remission with radiotherapy and chemotherapy. One year later, right cervical lymphadenopathy was observed and Hodgkin's lymphoma was confirmed on biopsy. The patient was treated with chemotherapy and autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of recurren...

  15. Indeterminate cell histiocytosis successfully treated with phototherapy.

    Science.gov (United States)

    Zerbini, Maria Claudia Nogueira; Sotto, Mirian Nacagami; de Campos, Fernando Peixoto Ferraz; Abdo, Andre Neder Ramires; Pereira, Juliana; Sanches, José Antônio; Martins, Jade Cury

    2016-01-01

    First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome. PMID:27547741

  16. Langerhans Cell Histiocytosis: A Case Report

    Directory of Open Access Journals (Sweden)

    Roro Suzy-Indharty

    2012-05-01

    Full Text Available Objective: Langerhans Cell Histiocytosis (LCH is a rare proliferative disorder of a distinct cell type that is similar to Langerhans cell. Prevalence is estimated at 1:200.000/year in children. Clinical presentations are variable, ranging from a single location in the bone to severe multivisceral involvement leading to dysfunction of vital organs. Therefore, objective of this study was to explore LCH based on clinical findings, skull x-ray, head CT-Scan, cytology, histopathology. and Immunohistochemistry.Method: We report a case of Langerhans Cell Histiocytosis of a 3-year-old boy. Fine needle aspiration biopsy was performed from a nodule in parietal area on the scalp; dry fixation was made and stained with Giemsa. Immunohistochemistry examination was performed with CD1a and S100. Histopathology examination was done post-operatively and cytology examination were consistent with LCH, composing Langerhans cells with complex, clefted, grooved, irregular or convoluted nuclei with fine chromatins and one or more small nucleoli and moderate to abundant quantities of eosinophilic cytoplasm, mixed with multinucleated eosinophils.Results: The finding of pentalaminar Birbeck granules by electron microscope is diagnostic of LCH, but this examination cannot be performed in our institution, so diagnosis was supported by immunohistochemistry with CD1a and S 100. Conclusions: Based on clinical findings, skull x ray, Head CT-Scan, cytology, histopathology and Immunohistochemistry, this case was concluded as a Langerhans Cell Histiocytosis.

  17. An unusual radiological presentation of pulmonary Langerhans’ cell histiocytosis

    OpenAIRE

    Burr, Lucy; Fanning, Steven; Wong, David; Bowler, Simon

    2012-01-01

    Langerhans’ cell histiocytosis is a proliferative disease of the dendritic cell lineage. It usually has a characteristic radiological presentation of apical nodules and small cysts occurring predominantly in young adult smokers. Here we report a case of multisystem Langerhans cell histiocytosis in a 75-year-old woman with unusual chest radiology.

  18. Langerhans cell histiocytosis of the orbit.

    Directory of Open Access Journals (Sweden)

    Shetty S

    2001-01-01

    Full Text Available Langerhans cell histiocytosis (LCH accounts for less than 1% of all orbital tumours. Though rare, orbital involvement in LCH is not uncommon. Most reports so far have been in Western literature. We report here a case of LCH of orbit with intracranial extension. To the best of our knowledge, this is the first case of single system multifocal LCH reported from India.

  19. Langerhans cell histiocytosis with multiple spinal involvement

    OpenAIRE

    Jiang, Liang; Liu, Xiao Guang; Zhong, Wo Quan; Ma, Qing Jun; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting; Liu, Zhong Jun

    2010-01-01

    To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four sof...

  20. Paediatric neuroradiological aspects of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Demaerel, Philippe [University Hospital K.U.Leuven, Department of Radiology, Leuven (Belgium); Gool, Stefaan van [University Hospital K.U.Leuven, Paediatric Haemato-oncology/Neuro-oncology, Leuven (Belgium)

    2008-01-15

    Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse. (orig.)

  1. Paediatric neuroradiological aspects of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse. (orig.)

  2. Langerhans' cell histiocytosis: possible association with malignant germ cell tumour.

    OpenAIRE

    Ng, W K; Lam, K Y; Ng, I O

    1995-01-01

    A rare case of adult onset Langerhans' cell histiocytosis associated with dysgerminoma in a 35 year old Chinese woman is reported. The patient had a history of dysgerminoma of left ovary 15 years previously and had undergone surgery followed by radiotherapy and an uneventful recovery. She presented again in March 1994, this time with a left clavicular mass, which was shown histologically to be Langerhans' cell histiocytosis. The report illustrates the probable association between the two lesi...

  3. Langerhans cell histiocytosis of the temporal bone in a child

    OpenAIRE

    Chakravarti, A; Rajeev Gupta; Sahni, J. K.

    2011-01-01

    Langerhans cell histiocytosis of temporal bone is a benign tumor like condition with variable clinical course. This rare clinical entity may mimic common ENT clinical condition like otitis media , mastoiditis and otitis externa . A high index of suspicion is required to reach to a diagnosis of langerhans cell histiocytosis to prevent delay in proper management of this disease.

  4. Langerhans Cell Histiocytosis of the Temporal Bone.

    Science.gov (United States)

    Ginat, Daniel Thomas; Johnson, Daniel N; Cipriani, Nicole A

    2016-06-01

    Langerhans cell histiocytosis involving the temporal bone region is uncommon and can resemble malignant neoplasms on imaging due to high cellularity. Although recognizing the presence of sharp margins with beveled-edges can be helpful, tissue sampling is often necessary for confirming the diagnosis. Cytology classically demonstrates kidney-bean shaped nuclei within the Langerhans cells and immunohistochemical staining is positive for S-100, peanut agglutinin (PNA), MHC class II, CD1a, and Langerin (CD 207). These features are exemplified in this sine qua non radiology-pathology correlation article. PMID:25903273

  5. [Pulmonary Langerhans cell histiocytosis in adults].

    Science.gov (United States)

    Feuillet, Séverine; Giroux-Leprieur, Bénédicte; Tazi, Abdellatif

    2010-01-01

    Pulmonary Langerhans-cell histiocytosis in adults is a rare condition of unknown etiology characterized by the accumulation of Langerhans cells organized in granulomas involving the distal bronchioles and destroying their walls. It occurs in young subjects who smoke, with frequency peaking between 20 and 40 years. High-resolution thoracic CT is essential for diagnosis; in typical forms it shows a combination of nodules, cavitary nodules, thick-walled cysts, and thin-walled cysts. Diagnostic certainty requires a surgical lung biopsy, by videothoracoscopy, but only if a specialist considers it indicated. It is difficult to predict the disease course for any given patient. A prospective multicenter cohort study currently underway should provide more information about the natural history of this disease. Management is empirical, for efficacy has not been proved for any treatment. Stopping smoking is especially important to prevent the added development of chronic obstructive pulmonary disease (COPD), cardiovascular complications, or the onset of bronchopulmonary cancer, the frequency of which appears elevated in these patients. Oral corticosteroids are used to treat disease progression, especially in the symptomatic mainly nodular forms, but their efficacy for respiratory function has not been shown. Vinblastine, the reference treatment for multisystem forms of Langerhans-cell histiocytosis, is not indicated for pulmonary involvement in adults. Better knowledge of the pathogenic mechanisms involved in this condition should eventually make it possible to develop innovative treatment strategies. The creation of the national reference center for Langerhans-cell histiocytosis has given new momentum to clinical and pathophysiologic research on this orphan disease. PMID:19959324

  6. Langerhans cell histiocytosis of bone: MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    George, J.C. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Buckwalter, K.A. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Cohen, M.D. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Edwards, M.K. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology; Smith, R.R. [Indiana Univ., Indianapolis, IN (United States). Dept. of Radiology

    1994-03-01

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies. (orig.)

  7. Liver involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Wong, Adelaine; Ortiz-Neira, Clara L.; Abou Reslan, Walid; Kaura, Deepak [Alberta Children' s Hospital, Department of Diagnostic Imaging, Calgary, Alberta (Canada); Sharon, Raphael; Anderson, Ronald [Alberta Children' s Hospital, Department of Oncology, Calgary, AB (Canada); Pinto-Rojas, Alfredo [Alberta Children' s Hospital, Department of Pathology, Calgary, AB (Canada)

    2006-10-15

    Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. (orig.)

  8. Langerhans cell histiocytosis of bone: MR imaging.

    Science.gov (United States)

    George, J C; Buckwalter, K A; Cohen, M D; Edwards, M K; Smith, R R

    1994-01-01

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies.

  9. Langerhans Cell Histiocytosis of the Clavicle

    Science.gov (United States)

    Wang, Shaowu; Zhang, Weisheng; Na, Shengbo; Zhang, Lina; Lang, Zhijin

    2014-01-01

    Abstract We report a rare case of solitary Langerhans cell histiocytosis (LCH) involving the clavicle of an adult female. The patient was a 32-year-old female presenting with 1 month history of progressive pain, swelling, and tenderness in the region near the left sternoclavicular joint. Radiograph, computed tomography, and magnetic resonance imaging showed an osteolytic lesion in the clavicle with tumor extension and soft tissue edema. Surgical curettage of the lesion was performed, and the histopathologic diagnosis was LCH. Because of its rarity and possibly variable presentation, LCH should be included and considered in the differential diagnosis when we encounter a clavicle lesion. PMID:25365405

  10. Liver involvement in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. (orig.)

  11. Langerhans cell histiocytosis of bone: MR imaging

    International Nuclear Information System (INIS)

    Magnetic resonance (MR) images of 12 pathologically proven lesions of Langerhans cell histiocytosis (LCH) of bone were reviewed retrospectively. MR identified all lesions, three of which were not identified on plain radiographs. In all cases, MR showed greater abnormality than did plain radiographs. With one exception, all lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesions and associated soft tissue abnormalities were very conspicuous on short TI inversion sequences and T1-weighted post-contrast images. Follow-up MR studies in two patients after chemotherapy showed decreased size and enhancement of lesions compared with baseline studies. (orig.)

  12. Rapid resolution of pulmonary Langerhans cell histiocytosis

    OpenAIRE

    Chong, Sy Giin; Samaha, Mario; Samaha, Georges; Casserly, Brian

    2013-01-01

    A 38-year-old woman with a long-term history of smoking presented to the lung cancer clinic with weight loss and recurrent lower respiratory tract infections. A diagnosis of Langerhans cell histiocytosis was made by specimen samples taken from video-assisted thoracoscopic surgery.  She was advised to stop smoking, which she adhered to. She was followed up in the respiratory clinic with pulmonary function tests every 6 months, which were all normal. Follow-up chest x-ray showed complete resolu...

  13. Langerhans cell histiocytosis in the adult.

    Science.gov (United States)

    Malpas, J S; Norton, A J

    1996-12-01

    A study of 47 well-documented patients with Langerhans cell histiocytosis (LCH) showed a slight female preponderance, with onset as late as the ninth decade. The skin was the commonest site of presentation, but pulmonary and bone involvement was frequent. Patients with single-site disease did best. The worst prognosis was seen in the elderly or those with organ dysfunction. A high incidence of associated malignant disease was seen, which could precede, be coincidental with, or occur after a diagnosis of LCH. PMID:8888814

  14. Vulvar Langerhans cell histiocytosis: a case report

    Science.gov (United States)

    Khoummane, Nadia; Guimeya, Cyriane; Lipombi, Dominique; Gielen, François

    2014-01-01

    Langerhans cell histiocytoses (LCH) are a rare group of disorders that comprise a large spectrum of diseases initially known as histiocytosis X. In this case report, we relate a case of LCH affecting the vulva of a 47-year-old female. The patient presented since 3 years with a vulvar lesion characterized by non-healing ulcers and a perineal granuloma on which she underwent surgery. Professionals should keep in mind not to treat straightforwardly lesions of the genital tract as simple sexually transmitted diseases. Chronic, atypical genital lesions seen in women need to be worked up and dealt with accordingly. PMID:25404979

  15. Langerhans cell histiocytosis on the penis: a case report

    OpenAIRE

    Kuroda Isao; Ashimine Satoshi; Ueno Munehisa; Hagiuda Jun; Ishizawa Keisuke; Deguchi Nobuhiro

    2006-01-01

    Abstract Background Langerhans cell histiocytosis affects mainly young children and features an accumulation of CD1a+ dendritic Langerhans cells in the bone, skin, and other organs. A few cases of Langerhans cell histiocytosis on the penis have been reported in the literature. We present a case of Langerhans cell histiocytosis on the penis and review the similar cases in the literature. Case presentation The patient was a 13-year-old boy who had a history of lymph node, femur bone, and pituit...

  16. Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jong Sung; Bang, Duk Ja; Rhim, Hyun Chul; Jeon, Seok Chol; Lee, Seung Ro; Hahm, Chang Kok [Hanyang University College of Medicine, Seoul (Korea, Republic of)

    1993-09-15

    Pulmonary Langerhans' cell histiocytosis is an uncommon granulomatous disorder of unknown cause. The authors retrospectively evaluated radiography and computed tomographic findings of five patients with biopsy proven pulmonary Langerhans' cell histiocytosis. The main structural abnormalities consisted of small nodules and cystic air spaces, but one cases showed only pneumothorax due to bullae rupture. Its distribution has been known predominantly in the upper lung fields, but in our cases, the lung lesions were distributed in the entire lung fields or predominently in the lower lung fields. We propose that pulmonary Langerhans' cell histiocytosis is extremely variable of its structural abnormalities and distribution.

  17. Concomitant nodal involvement by Langerhans Cell Histiocytosis and Hodgkin Lymphoma

    OpenAIRE

    Geurten, Claire; Thiry, Albert; Jamblin, Paul; Demarche, Martine; Hoyoux, Claire

    2015-01-01

    Introduction : Langerhans cell histiocytosis is defined as a clonal neoplastic proliferation of myeloid dendritic cells that upon activation migrate from the mucosal to lymph nodes. Definitive diagnosis is made by anatomo-pathological and immunohistochemical analysis. Langerhans cell histiocytosis is rarely, yet not exceptionally, found coexisting with other malignant neoplasms, suggesting it might arise in reaction to the cytokinic secretion of malignant cells. Case : We report the case o...

  18. Urticating histiocytosis: a mast cell-rich variant of histiocytosis X.

    Science.gov (United States)

    Foucar, E; Piette, W W; Tse, D T; Goeken, J; Olmstead, A D

    1986-05-01

    Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.

  19. Conventional radiology in the bony compromise of Langerhans cells Histiocytosis

    International Nuclear Information System (INIS)

    We present a descriptive study of 47 patients who attended the National Cancer Institute in Bogota, Colombia with pathological diagnosis of Langerhans cell histiocytosis. We reviewed the most frequent conventional x-ray findings

  20. Langerhans cell histiocytosis of the clavicle: a case report

    International Nuclear Information System (INIS)

    A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion. (orig.) (orig.)

  1. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    International Nuclear Information System (INIS)

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  2. Langerhans cell histiocytosis : A clinical and immunological study

    OpenAIRE

    Bernstrand, Cecilia

    2003-01-01

    Langerhans cell histiocytosis (LCH), previously known as histiocytosis X, eosinophilic granuloma, Hand-Schüller-Christian or Letterer-Siwe disease, is a rare disease with a reported incidence in childhood of 5.4 cases per million children per year. The disease can present at any age but young children are most often affected. It is characterized by an accumulation of abnormal and clonal Langerhans cells in various organs such as the skin, bone, lymph nodes, lungs, liver, spl...

  3. Concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma.

    Science.gov (United States)

    Geurten, Claire; Thiry, Albert; Jamblin, Paul; Demarche, Martine; Hoyoux, Claire

    2015-12-01

    A 10-year-old girl with a family history of Hodgkin's lymphoma presented with a 2 month history of cervical lymphadenopathy and weight loss. Biopsy indicated concomitant nodal involvement by Langerhans cell histiocytosis and Hodgkin's lymphoma. Such an association is rare, especially so in children, but is not an isolated phenomenon, thereby prompting the question of whether Langerhans cell histiocytosis is a reactive or a neoplastic process. PMID:26556799

  4. Defective alloantigen-presenting capacity of 'Langerhans cell histiocytosis cells'.

    OpenAIRE

    Yu, R C; Morris, J F; Pritchard, J.; Chu, T C

    1992-01-01

    The functional activity of skin cells derived from an infant who died of multisystem Langerhans cell histiocytosis (LCH) was examined. Involved and non-involved skin was obtained at postmortem examination within three hours of death; normal epidermal Langerhans cells and 'LCH cells' were separated by means of dispase digestion. The functional activity of different populations of CD1a positive cells was assessed using the conventional six day allogeneic mixed cell reaction. Compared with Lange...

  5. An unusual presentation of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Palak Agarwal

    2014-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a relatively rare and unique disease. An incidence of 7.9% in the jaws is reported. We report a case of 9-year-old male child referred to us from dental outpatient department, who presented with a firm swelling in right lower jaw along with bilateral submandibular lymphadenopathy for 1-month. Fine-needle aspiration was done from lytic lesion in the body of mandible and multiple smears were prepared. On the basis of the clinical and cytomorphological findings, a diagnosis of LCH was suggested. The diagnosis was confirmed on histology. Thus, a high possibility of LCH should be considered in children presenting with lytic lesions in head and neck region.

  6. An unusual presentation of Langerhans cell histiocytosis

    Science.gov (United States)

    Agarwal, Palak; Kaushal, Manju

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease. An incidence of 7.9% in the jaws is reported. We report a case of 9-year-old male child referred to us from dental outpatient department, who presented with a firm swelling in right lower jaw along with bilateral submandibular lymphadenopathy for 1-month. Fine-needle aspiration was done from lytic lesion in the body of mandible and multiple smears were prepared. On the basis of the clinical and cytomorphological findings, a diagnosis of LCH was suggested. The diagnosis was confirmed on histology. Thus, a high possibility of LCH should be considered in children presenting with lytic lesions in head and neck region. PMID:25745295

  7. Langerhans Cell Histiocytosis in Bilateral Mastoid Cavity

    Directory of Open Access Journals (Sweden)

    Kazım Bozdemir

    2013-01-01

    Full Text Available A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH. In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.

  8. [Langerhans cell histiocytosis causing cervical myelopathy].

    Science.gov (United States)

    Doléagbénou, A K; Mukengeshay Ntalaja, J; Derraz, S; El Ouahabi, A; El Khamlichi, A

    2012-08-01

    Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corporectomy and fusion. An 8-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying upper and lower limbs motor weakness. CT scans revealed destruction of C5 body and magnetic resonance imaging showed a tumoral process at C5 with cord compression. Interbody fusion using anterior cervical plate packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of limbs. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered. PMID:22552159

  9. The indeterminate cell proliferative disorder: report of a case manifesting as an unusual cutaneous histiocytosis.

    Science.gov (United States)

    Wood, G S; Hu, C H; Beckstead, J H; Turner, R R; Winkelmann, R K

    1985-11-01

    A patient with an unusual, distinctive cutaneous histiocytosis is described. Extensive morphologic, antigenic, and enzymatic studies indicate that this histiocytosis represents a proliferative disorder of cutaneous indeterminate cells. Features that distinguish this disorder from other histiocytoses are discussed.

  10. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    Science.gov (United States)

    Khoddami, Maliheh; Nadji, Seyed-Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad-Reza

    2016-01-01

    Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6), herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR) method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66%) out of 30 patients and in 1 (3.3%) of 30 control samples with a P value of 1 (1.00 > 0.05) using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. PMID:27307972

  11. Cytomegalovirus and Langerhans Cell Histiocytosis: Is There a Link?

    Directory of Open Access Journals (Sweden)

    Maliheh Khoddami

    2016-02-01

    Full Text Available Background: Langerhans cell histiocytosis is a rare proliferative histiocytic disease of unknown etiology. Histologically, it is characterized by granuloma-like proliferation of Langerhans-type dendritic cells derived from bone marrow. Many investigators have suggested the possible role of viruses such as Epstein-Barr virus, human herpesvirus-6 (HHV-6, herpes simplex virus (HSV types 1 and 2, and Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis. Objectives: In this study, we have investigated the presence of Cytomegalovirus in Langerhans cell histiocytosis in Iranian children. Patients and Methods: In this retrospective study, we have investigated the presence of Cytomegalovirus DNA expression, using paraffin-embedded tissue samples of 30 patients with Langerhans cell histiocytosis and 30 age and site-matched controls by qualitative Polymerase Chain Reaction (PCR method. Results: No significant difference in prevalence of Cytomegalovirus presence between patients and controls was found. Cytomegalovirus was found by qualitative PCR in only 2 (6.66% out of 30 patients and in 1 (3.3% of 30 control samples with a P value of 1 (1.00 > 0.05 using chi-square test with OR: 2.07; 95% CI of OR: 0.18 - 24.15. Conclusions: Our findings do not support the hypothesis of a possible role for Cytomegalovirus in the pathogenesis of Langerhans cell histiocytosis.

  12. Squash smear cytology of Langerhans cell histiocytosis

    Science.gov (United States)

    Nahm, Ji Hae; Yoon, Gun; Do, Sung-Im; Kim, Hyun-Soo

    2015-01-01

    Squash smear cytology of Langerhans cell histiocytosis (LCH) has rarely been reported. We described squash cytological findings of cranial LCH. Additionally, based on recent data that suggests an association of LCH with either viral infection or genetic alteration, we investigated the presence of several viruses or mutation of TP53 and BRAF in LCH tissue samples. Intraoperative squash smears of a small tissue fragment excised from the lesion demonstrated a mixed population of eosinophils, neutrophils, small lymphocytes and a high content of histiocytes. The histiocytes possessed abundant dense cytoplasm with round cell shape and eccentrically located nuclei with fine chromatin, delicate nuclear membranes and prominent nuclear grooves, indentations and pseudoinclusions. The cytologic features were consistent with Langerhans cells (LCs). Subsequent histopathologic examination confirmed the diagnosis of LCH. Immunohistochemically, the LCs were positive for S-100, CD1a and langerin, but negative for adenovirus, CMV, EBV, HHV-8, HPV, HSV, SV 40 and p53. BRAF V600E mutation was absent. Our findings did not support the role of viruses and genetic abnormalities in the pathogenesis of LCH. In summary, the presence of a mixed population of inflammatory cells and a high content of histiocytes with characteristic cytomorphology, along with radiologic evidence and appropriate clinical findings, is highly suggestive of LCH on the intraoperative squash smears. Awareness of characteristic cytological features of LCH is necessary for rapid and accurate diagnosis. Squash smear cytology is a potentially useful tool in the intraoperative diagnosis of LCH. PMID:26339366

  13. Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

    Directory of Open Access Journals (Sweden)

    Frederic A. Rawlins

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.

  14. Langerhans cell histiocytosis : a reactive or neoplastic disease?

    NARCIS (Netherlands)

    Teixeira da Costa, Cristiana Elizabete

    2008-01-01

    Although Langerhans cell histiocytosis (LCH) was first described a century ago, the aetiology is still not understood. Recent studies on the role of cytokines, chemokines, immunologic dysfunction, cell surface antigen expression, clonality and cell cycle regulation have provided new insights into t

  15. The presence of cytokines in Langerhans' cell histiocytosis

    NARCIS (Netherlands)

    deGraaf, JH; Tamminga, RYJ; DamMeiring, A; Kamps, WA; Timens, W

    1996-01-01

    Langerhans' cell histiocytosis (LCH) is characterized by an accumulation and/or proliferation of cells with a Langerhans' cell (LC) phenotype. The aetiology and pathogenesis of LCH are unknown; it is suggested that LCH is caused by an immunological dysregulation. Production of cytokines is a central

  16. CT findings of orbital langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Yi, Gina; Yoon, Hye Kyung; Han, Bokyung Kim; Kim, Kyeong Ah; Choo, In Wook [Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul (Korea, Republic of)

    2000-05-01

    To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n=3D5) and MR (n=3D1) imaging findings were also reviewed. Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units (HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatosplenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolved, with bone remodeling and reossification. A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification. (author)

  17. CT manifestations of pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: To analyzes the CT manifestations of pulmonary Langerhans cell histiocytosis (PLCH). Methods: CT features of 11 patients with PLCH proved pathologically were analyzed retrospectively. Results: The main findings in 11 PLCHs were cysts and nodules. Two cases only had cysts, and 1 only had nodules, which most had cavitations. The other 8 cases showed cysts and nodules with 4 cases mainly manifested with cysts and nine mainly manifested with nodules. Two cases had pulmonary interstitial changes. One case only had cysts in the left upper lung field and 10 cases had lesions not only in the upper and middle but in the lower lung field, which 2 cases had more lesions in the lower and costo-phrenic angle field and 8 cases had less lesions in the costo-phrenic angle field. Three of these 8 cases had more lesions in the superior lobe and apical segment of lower lobe. Conclusion: CT manifestations of PLCH are helpful for the early diagnosis. (authors)

  18. Radiological signs of childhood Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    To describe the most prominent radiological sings of Langerhans cell histiocytosis (LCH) in childhood and review the general aspects of greatest interest to the general radiologist. The clinical and radiological histories of 40 children diagnosed on the basis of biopsy and/or cytology as having LCH in our center over a 16-year period were reviewed. Bone involvement was observed in 95% of patients; flat bones were affected in 95% of the cases, most frequently the cranium. Radiological changes in temporal bone were viewed in 15% of cases. There were long-bone lesions in 21% of the patients, involving, metaphyseal-diaphyseal bones in every case; multiple epiphyseal dysplasia was found in one and a purely cortical lesion in another. Vertebral involvement was detected in 18% of patients. Clinical evidence of diabetes insipidus (DI) was present in 22.5% of cases, while there were clinical or radiological signs of lung involvement in 15%. Bone involvement is the most common association reported in childhood LCH and is usually the reason for medical consultation. The flat bones, especially the cranium, are those most often affected. Diagnosis and follow-up studies are mainly based on plain radiography. DI due to hypothalamic infiltration in usually associated with normal CT scan. Lung involvement, uncommon in pediatric patients, is the major clinical and radiological difference with respect to the adult form. (Author) 33 refs

  19. CT findings of orbital langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n=3D5) and MR (n=3D1) imaging findings were also reviewed. Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units (HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatosplenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolved, with bone remodeling and reossification. A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification. (author)

  20. Sudden death of a patient with pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Nakhla, Hassan; Jumbelic, Mary I

    2005-06-01

    We report a case of sudden death due to bilateral pneumothorax in a previously healthy 16-year-old adolescent white girl. She presented with sudden onset of shortness of breath followed by loss of consciousness. Postmortem chest radiograph showed bilateral pneumothoraces. Autopsy confirmed the bilateral pneumothorax and additionally showed emphysematous changes and bullae throughout the lung tissue. Microscopic sections of the lungs showed Langerhans cell histiocytosis. To the best of our knowledge, this is the first reported case of fatal presentation of pulmonary Langerhans cell histiocytosis. PMID:15913433

  1. Complete pathological resolution of pulmonary Langerhans cell histiocytosis

    OpenAIRE

    Ninaber, Maarten; Dik, Hans; Peters, Elke

    2014-01-01

    Cigarette smoking is a major risk factor for pulmonary Langerhans cell histiocytosis (pLCH) and lung cancer. Resolution of pLCH may occur spontaneously, after smoking cessation or other interventions. However, despite clinicoradiological resolution, residual pulmonary Langerhans cells may be present and may lead to recurrent disease. We report the first case of pLCH with a complete histological resolution.

  2. Langerhans cell histiocytosis with multiple spinal involvement.

    Science.gov (United States)

    Jiang, Liang; Liu, Xiao Guang; Zhong, Wo Quan; Ma, Qing Jun; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting; Liu, Zhong Jun

    2011-11-01

    To stress the clinical and radiologic presentation and treatment outcome of Langerhans cell histiocytosis (LCH) with multiple spinal involvements. A total of 42 cases with spinal LCH were reviewed in our hospital and 5 had multifocal spinal lesions. Multiple spinal LCH has been reported in 50 cases in the literature. All cases including ours were analyzed concerning age, sex, clinical and radiologic presentation, therapy and outcome. Of our five cases, three had neurological symptom, four soft tissue involvement and three had posterior arch extension. Compiling data from the eight largest case series of the spinal LCH reveals that 27.2% multiple vertebrae lesions. In these 55 cases, there were 26 female and 29 male with the mean age of 7.4 years (range 0.2-37). A total of 182 vertebrae were involved including 28.0% in the cervical spine, 47.8% in thoracic and 24.2% in the lumbar spine. Extraspinal LCH lesion was documented in 54.2% cases, visceral involvement in 31.1% and vertebra plana in 50% cases. Paravertebral and epidural extension were not documented in most cases. Pathological diagnosis was achieved in 47 cases including 8 open spine biopsy. The treatment strategy varied depending on different hospitals. One patient died, two had recurrence and the others had no evidence of the disease with an average of 7.2 years (range 1-21) of follow-up. Asymptomatic spinal lesions could be simply observed with or without bracing and chemotherapy is justified for multiple lesions. Surgical decompression should be reserved for the uncommon cases in which neurologic compromise does not respond to radiotherapy or progresses too rapidly for radiotherapy. PMID:20496040

  3. Acute leukemia in association with Langerhans cell histiocytosis

    NARCIS (Netherlands)

    R.M. Egeler (Maarten); J.P. Neglia (J.); M. Aricò (Maurizio); B.E. Favara (B.); A. Heitger (A.); M.E. Nesbit (M.)

    1994-01-01

    textabstractLangerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was develope

  4. Thallium-201 uptake in Langerhans` cell histiocytosis of bone

    Energy Technology Data Exchange (ETDEWEB)

    Bar-Sever, Z. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Connolly, L.P. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Jaramillo, D. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States); Treves, S.T. [Division of Nuclear Medicine, Department of Radiology, Children`s Hospital and Harvard Medical School, Boston, MA (United States)

    1996-10-01

    A case in which there was thallium-201 ({sup 201}Tl) uptake in Langerhans` cell histiocytosis (LCH) of the left femoral diaphysis is presented. The authors propose that {sup 201}Tl scintigraphy is potentially useful in the diagnosis and follow-up of patients with LCH. (orig.). With 4 figs.

  5. Langerhans cell histiocytosis of the clavicle: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Verbist, B.; Geusens, E.; Brys, P.; Verslegers, I.; Baert, A.L. [Department of Radiology, University Hospitals, Leuven (Belgium); Samson, I. [Department of Orthopedic Surgery, University Hospitals, Leuven (Belgium); Sciot, R. [Department of Pathology II, University Hospitals, Leuven (Belgium)

    1998-10-01

    A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion. (orig.) (orig.) With 3 figs., 7 refs.

  6. Langerhans cell histiocytosis with involvement of the pons: case report

    Energy Technology Data Exchange (ETDEWEB)

    Vourtsi, A. [Xatzopoulou, Athens (Greece)]|[Department of Radiology, University of Athens Medical School, Athens (Greece); Papadopoulos, A.; Moulopoulos, L.A.; Vlahos, L. [Department of Radiology, University of Athens Medical School, Athens (Greece); Xenellis, J. [Department of Otorhinolaryngology, University of Athens Medical School, Athens (Greece)

    1998-03-01

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.) With 2 figs., 17 refs.

  7. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids

    Science.gov (United States)

    Bourque, Jean-Marc; Lukovic, Jelena; Dar, A. Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  8. Langerhans cell histiocytosis with involvement of the pons: case report

    International Nuclear Information System (INIS)

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.)

  9. Radiotherapy for Langerhans Cell Histiocytosis of Bilateral Eyelids.

    Science.gov (United States)

    Leveson, James; Bourque, Jean-Marc; Lukovic, Jelena; Dar, A Rashid

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder with numerous clinicopathological variants with differing clinical courses, treatment methods, and prognoses. We report one patient with atypical LCH of the bilateral lower eyelids and subsequent successful treatment with local radiation therapy. PMID:27004151

  10. Langerhans cell histiocytosis with multifocal sites. A case report

    International Nuclear Information System (INIS)

    The authors report a case of Langerhans cell histiocytosis in which the patient, at the time of the diagnosis, had lesions at the central nervous system, mediastinum and long bones, all detectable in simple roentgenogram. The recent reclassification of this disease is discussed together with it's radiological findings. (author)

  11. Thallium-201 uptake in Langerhans' cell histiocytosis of bone

    International Nuclear Information System (INIS)

    A case in which there was thallium-201 (201Tl) uptake in Langerhans' cell histiocytosis (LCH) of the left femoral diaphysis is presented. The authors propose that 201Tl scintigraphy is potentially useful in the diagnosis and follow-up of patients with LCH. (orig.). With 4 figs

  12. Oral manifestations of chronic disseminated langerhans cell histiocytosis: a case report

    OpenAIRE

    Namdar Pekiner, Filiz; BORAHAN, M.Oğuz; ÖZBAYRAK, Semih; Alatlı, Canan; Kızılyel, Gizem

    2012-01-01

    The term histiocytosis X was introduced as a collective designation for a spectrum of clinopathologic disorders characterized by proliferation of histiocyte-like cells. The distinctive histiocytic cells present in this lesion have been identified as Langerhans cells, and the condition is now designated as Langerhans cell histiocytosis. Chronic disseminated langerhans cell histiocytosis is a disease involving bone, skin, and viscera (Hand-Schüller-Christian disease). The often-cited Chronic di...

  13. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

    OpenAIRE

    Saede Atarbashi Moghadam; Ali Lotfi; Batool Piroozhashemi; Sepideh Mokhtari

    2015-01-01

    Statement of the Problem: Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose: The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Meth...

  14. Langerhans Cell Histiocytosis Presenting as Brown Lichenoid Patches

    OpenAIRE

    Kwon, Hyuk; Lee, Jang Hyun; Kim, Sung Keun; Park, Young Lip; Lee, Jong Suk; Cho, Moon Kyun

    2009-01-01

    Langerhans cell histiocytosis (LCH) is related diseases characterized by proliferation of Langerhans cell with involvement of bone, skin, lung and other organs. LCH usually occurs in childhood and are presented as multiple small papules or eczematoid lesion mostly. We report a 50-year-old man with 3 brown lichenoid patches on left dorsal foot. He was diagnosed pulmonary LCH 5 years ago. Typical LC cells on skin lesion and CD1 complex positive staining confirm the diagnosis of LCH. We consider...

  15. Juvenile xanthogranuloma developing after treatment of Langerhans cell histiocytosis: case report and literature review

    OpenAIRE

    Strehl, Johanna D; Stachel, Klaus-Daniel; Hartmann, Arndt; Agaimy, Abbas

    2012-01-01

    The synchronous or metachronous development of Langerhans cell histiocytosis and non-Langerhans cell histiocytosis in the same patient is rare. To date, only seven cases of xanthogranulomas developing in young patients with a history of Langerhans cell histiocytosis and systemic therapy have been reported in the literature. As of yet, the pathogenesis and the clinical significance of this phenomenon are unclear. We report the case of a 3 year old boy who developed juvenile Xanthogranulomas on...

  16. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    Directory of Open Access Journals (Sweden)

    Anupam Patra

    2015-01-01

    Full Text Available Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a indicated a diagnosis of Langerhans cell histiocytosis. Clinicians should consider pulmonary Langerhans cell histiocytosis in differential diagnoses in dealing such a case.

  17. Langerhans cell histiocytosis presenting with complicated pneumonia, a case report

    OpenAIRE

    Ottink, Mark; Feijen, Simone; Rosias, Philippe; Robben, Simon; Granzen, Bernd; Heynens, Jan; Jöbsis, Rijn

    2013-01-01

    We describe a 2 ½ year old boy presenting with fever, abdominal pain and splinter haemorrhages of the nails. On further examination there were signs of pneumonia with pleural effusion. This was treated with mini-thoracotomy, drainage and intravenous antibiotics. Further diagnostic workup for underlying causes showed diffuse cystic lung disease, suggestive of Langerhans cell histiocytosis. This was confirmed on pathology specimens, which showed Langerhans cells in lung tissue, nail bed and ski...

  18. Proteome analysis of bronchoalveolar lavage in pulmonary langerhans cell histiocytosis

    OpenAIRE

    2011-01-01

    Background Pulmonary Langerhans-cell histiocytosis (PLCH) is a rare interstitial lung disease characterized by clusters of Langerhans cells, organized in granulomas, in the walls of distal bronchioles. It is a diffuse lung disease related to tobacco smoking but otherwise of unknown etiopathogenesis. Methods In this study we used a proteomic approach to analyze BAL protein composition of patients with PLCH and of healthy smoker and non-smoker controls to obtain insights into the pathogenetic m...

  19. Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

    OpenAIRE

    Kapoor, Rohit; Loizides, Anthony M; Sachdeva, Soumya; Paul, Premila

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely describe...

  20. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    OpenAIRE

    Bruno Araujo; Francisco Costa; Joanne Lopes; Ricardo Castro

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocyt...

  1. Uncommon features of pulmonary Langerhans' cell histiocytosis:analysis of 11 cases and a review of the literature

    Institute of Scientific and Technical Information of China (English)

    LING Chun-hua; JI Cheng; Daniel P Raymond; Patricia A Bourne; XU Hao-dong

    2010-01-01

    @@ Pulmonary Langerhans' cell histiocytosis (PLCH) is an isolated form of Langerhans' cell histiocytosis that usually develops in cigarette smokers.~(1,2) PLCH usually has typical light microscopic morphology with a spectrum of progressive changes.~(2-4)

  2. Langerhans cell histiocytosis: Current concepts in dentistry and case report

    Science.gov (United States)

    Ramos-Gutiérrez, Efraín; Alejo-González, Francisco; Ruiz-Rodríguez, Socorro; Garrocho-Rangel, José-Arturo

    2016-01-01

    Langerhans cell histiocytosis (LCH), which is a rare granulomatous pediatric disease of unknown etiology, is characterized by the idiopathic proliferation and accumulation of abnormal and clonal Langerhans cells or their marrow precursors, resulting in localized, solitary or multiple destructive lesions. These lesions are most commonly eosinophilic granuloma, which are found in craniofacial bone structures such as the skull and mandible, skin and other organs. In children, the disease has a variable initial presentation, and the clinical course, prognosis and survival are unpredictable. The aims of this report were to present an LCH case in a girl aged 2 years, 8 months and her clinicopathological features, to describe the bucodental management provided, and to discuss special dental considerations of this disease. Key words:Children, dental management, histiocytosis, Langerhans cells. PMID:26855698

  3. 99mTc bone scan and fluorodeoxyglucose positron emission tomography in evaluation of disseminated langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a rare histiocytic disorder in which pathological langerhans cells accumulate in a variety of organs. Manifestations may include lung infiltrates, lymph node involvements, bone lesions, hepatic, hematopoietic and endocrine dysfunctions. In this case report we present fluorine-18 positron emission tomography (F-18 PET/CT) and bone scintigraphy findings of a 18-year-old male patient with disseminated LCH, mimicking multiple hypermetabolic metastatic lesions. Clinicians should be aware that LCH infiltrations can be seen as intense uptake and to differentiate infiltrations from other metastatic intense uptake with fluorodeoxyglucose PET/CT and bone scintigraphy, clinical and laboratory findings should be kept in mind. (author)

  4. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Saatci, I.; Baskan, O.; Haliloglu, M.; Aydingoz, U. [Department of Radiology, Hacettepe University Hospital, Sihhiye 06100, Ankara (Turkey)

    1999-06-01

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.) With 5 figs., 22 refs.

  5. Disseminated Langerhans Cell Histiocytosis Presenting as Cholestatic Jaundice

    Science.gov (United States)

    Loizides, Anthony M.; Sachdeva, Soumya; Paul, Premila

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta. PMID:25859497

  6. Disseminated langerhans cell histiocytosis presenting as cholestatic jaundice.

    Science.gov (United States)

    Kapoor, Rohit; Loizides, Anthony M; Sachdeva, Soumya; Paul, Premila

    2015-02-01

    Langerhans cell histiocytosis (LCH) is a disorder associated with proliferation of Langerhans cells in various organs. LCH secondary to multisystem involvement can present in a variety of ways. Because of its infiltrative nature, LCH can involve the skin, lymph nodes, the lung or the liver. Jaundice in LCH is a manifestation of liver disease; biliary dilatation secondary to lithiasis or may be due to coexistent Niemann-Pick disease. However, a case of cholestasis has been very rarely described. Cholestasis may result from lymph nodes obstructing the porta hepatis. In this report, we describe a case of type II histiocytosis X with obstructive cholestasis and pulmonary involvement in the form of cysts without significant lymphadenopathy at the porta. PMID:25859497

  7. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.)

  8. Photodynamic therapy for multi-resistant cutaneous Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Arjen F. Nikkels

    2010-06-01

    Full Text Available Langerhans cell histiocytosis is a rare group of proliferative disorders. Beside cutaneous involvement, other internal organs can be affected. The treatment of cutaneous lesions is difficult and relies on topical corticosteroids, carmustine, nitrogen mustard, and photochemotherapy. Systemic steroids and vinblastine are used for recalcitrant skin lesions. However, some cases fail to respond. An 18-month old boy presented a CD1a+, S100a+ Langerhans cell histocytosis with cutaneous and severe scalp involvement. Topical corticosteroids and nitrogen mustard failed to improve the skin lesions. Systemic corticosteroids and vinblastine improved the truncal involvement but had no effect on the scalp lesions. Methyl-aminolevulinate (MAL based photodynamic therapy (PDT resulted in a significant regression of the scalp lesions. Control histology revealed an almost complete clearance of the tumor infiltrate. Clinical follow-up after six months showed no recurrence. Although spontaneous regression of cutaneous Langerhans cell histiocytosis is observed, the rapid effect of photodynamic therapy after several failures of other treatment suggests that photodynamic therapy was successful. As far as we know this is the first report of photodynamic therapy for refractory skin lesions. Larger series are needed to determine whether photodynamic therapy deserves a place in the treatment of multiresistant cutaneous Langerhans cell histiocytosis.

  9. Langerhans cell histiocytosis involving the liver of a male smoker: a case report

    OpenAIRE

    Savva-Bordalo Joana; Freitas-Silva Margarida

    2008-01-01

    Abstract Introduction Langerhans' cell histiocytosis is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. Case presentation The authors report a case of Langerhans' cell histiocytosis in a 48-year-old man with multisystemic disease presentation, including liver involvement. Conclusion Hepatic involvement is an uncommon feature in this rare disease and there is no consensus on the most effective therapeutic approach.

  10. Langerhans cell histiocytosis involving central nervous system: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Won Jin; Park, Dong Woo; Lee, Seung Ro; Hahm, Chang Kok; Ju, Kyung Bin [Hanyang University College of Medicine, Seoul (Korea, Republic of); Kim, Sung Tae [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    1997-01-01

    Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI.

  11. Langerhans cell histiocytosis involving central nervous system: a case report

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI

  12. Langerhans Cell Histiocytosis Causing Cervical Myelopathy in a Child

    OpenAIRE

    Jang, Kun Soo; Jung, Youn Young; Kim, Seok Won

    2010-01-01

    Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumora...

  13. Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

    OpenAIRE

    Juvet, Stephen C; David Hwang; Downey, Gregory P.

    2010-01-01

    Pulmonary Langerhans’ cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert f...

  14. Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

    OpenAIRE

    WEI, PING; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-01-01

    Abstract Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature. A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = ...

  15. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    OpenAIRE

    Mozhgan Rezaei Kanavi; Fatemeh Javadi; Mohammad-Ali Javadi; Amir Faramarzi

    2012-01-01

    Purpose: To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH) simulating a limbal papilloma. Case report: A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of "acute bullous inflammation with granulation tissue" was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral ...

  16. Langerhan′s cell histiocytosis: A single institutional experience

    OpenAIRE

    Singh Tejinder; Satheesh C; Appaji L; Aruna Kumari B; Mamatha H; Giri G; Rao Clementina

    2010-01-01

    Background: Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. Aim: We analyzed our single institutional experience of managing children with LCH. Settings and Design: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated ret...

  17. Langerhan’s cell histiocytosis: A single institutional experience

    OpenAIRE

    Singh, Tejinder; C T Satheesh; Appaji, L.; B S Aruna Kumari; Mamatha, H. S.; G V Giri; Rama Rao, Clementina

    2010-01-01

    Background: Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. Aim: We analyzed our single institutional experience of managing children with LCH. Settings and Design: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated ret...

  18. MR and CT findings of temporal bone langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Jae Ig; Lee, Hee Jung; Kim, Heung Sik [Keimyung Univ. School of Medicine, Dongsan Medical Center, Taegu (Korea, Republic of)

    2001-11-01

    To describe the MRI and CT findings of temperal bone langerhans cell histiocytosis. The MRI (n=8) and CT (n=7) findings of nine lesions of temporal bone Langerhans cell histiocytosis in six children were retrospectively reviewed. Eight lesions were pathologically confirmed and one was clinically diagnosed. The findings were analyzed for bilaterality, location, lesion extent, signal intensity, the attenuation of soft tissue lesions seen at MRI or precontrast CT, enhancement pattern at MRI or CT, and the pattern of bony destruction at CT. Bilateral involvement was present in three of six patients (50%). Lesions were most frequently located in the mastoid (n=8, 89%), followed by the petrous ridge (n=6, 67%), and the squamous portion (n=3, 33%). Seven (78%) lesions extended to the ipsilateral cavernous sinus (n=3), sphenoid bone (n=3), orbit (n=2), or epidural space (n=2). The signals of the soft tissue lesions were isointense in five cases (63%) on T1-weighted images and hyperintense in six (75%) at MRI, and homogeneous in five (71%) at CT. All lesions demonstrated bony destruction without periosteal reaction and five (71%) showed ill-defined destruction, with crossing sutures. Familiarity with findings of predominant mastoid involvement, isointense or isodense soft tissue lesions seen on T1-weighted images or at precontrast CT, with relatively homogeneous enhancement at CT, and irregular bony destruction with crossing sutures may be helpful in narrowing the diagnosis of temporal bone langerhans cell histiocytosis.

  19. MR and CT findings of temporal bone langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    To describe the MRI and CT findings of temperal bone langerhans cell histiocytosis. The MRI (n=8) and CT (n=7) findings of nine lesions of temporal bone Langerhans cell histiocytosis in six children were retrospectively reviewed. Eight lesions were pathologically confirmed and one was clinically diagnosed. The findings were analyzed for bilaterality, location, lesion extent, signal intensity, the attenuation of soft tissue lesions seen at MRI or precontrast CT, enhancement pattern at MRI or CT, and the pattern of bony destruction at CT. Bilateral involvement was present in three of six patients (50%). Lesions were most frequently located in the mastoid (n=8, 89%), followed by the petrous ridge (n=6, 67%), and the squamous portion (n=3, 33%). Seven (78%) lesions extended to the ipsilateral cavernous sinus (n=3), sphenoid bone (n=3), orbit (n=2), or epidural space (n=2). The signals of the soft tissue lesions were isointense in five cases (63%) on T1-weighted images and hyperintense in six (75%) at MRI, and homogeneous in five (71%) at CT. All lesions demonstrated bony destruction without periosteal reaction and five (71%) showed ill-defined destruction, with crossing sutures. Familiarity with findings of predominant mastoid involvement, isointense or isodense soft tissue lesions seen on T1-weighted images or at precontrast CT, with relatively homogeneous enhancement at CT, and irregular bony destruction with crossing sutures may be helpful in narrowing the diagnosis of temporal bone langerhans cell histiocytosis

  20. Langerhans cell histiocytosis of the atlas in an adult.

    Science.gov (United States)

    Zhong, Wo Quan; Jiang, Liang; Ma, Qing Jun; Liu, Zhong Jun; Liu, Xiao Guang; Wei, Feng; Yuan, Hui Shu; Dang, Geng Ting

    2010-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, is a rare disorder (approximately 1:1,500,000 inhabitants) characterized by clonal proliferation and excess accumulation of pathologic Langerhans cells causing local or systemic effects. The exact etiology of LCH is still unknown. LCH could affect patients of any age, although most present when they are children. The most frequent sites of the bony lesions are the skull, femur, mandible, pelvis and spine. A variety of treatment modalities has been reported, but there was no evidence suggesting that any one treatment was more advantageous than another. We present an adult with LCH of the atlas. A 26-year-old young man presented with a 2-month history of neck pain and stiffness. CT revealed osteolytic lesion in the left lateral mass of atlas with compression fracture. Histopathological diagnosis was Langerhans cell histiocytosis by percutaneous needle biopsy under CT guidance. The patient underwent conservative treatment, including Halo-vest immobilization and radiotherapy. At 7-year follow-up, the patient was asymptomatic except for mild motion restriction of the neck. CT revealed a significant reconstruction of the C1 lateral mass. PMID:19844749

  1. The role of bone scintigraphy in Langerhans' cell histiocytosis; a case report

    International Nuclear Information System (INIS)

    We present one case of bone-Langerhans cell histiocytosis in a three-year-old male child presenting osseous lesions in the skull and the femur, which are very frequent localizations in histiocytosis. Bone scintigraphy is useful for both initial staging and follow-up associated with other imaging modalities. (authors)

  2. A child with pulmonary and liver Langerhans'-cell histiocytosis

    Institute of Scientific and Technical Information of China (English)

    MA Xiao-li; SHEN Kun-ling; WANG Bin

    2012-01-01

    Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease.Pulmonary LCH is rare,which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans' cells to organs.We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation.The patient was treated with chemotherapy which included prednisone,vinblastine,methotrexate and 6-mercaptopurine for 52 weeks and follow up all along.The patient has a favorable clinical outcome.

  3. High expression of markers of apoptosis in Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Petersen, Bodil Laub; Lundegaard, Pia Rengtved; Bank, M I;

    2003-01-01

    53 and the number of cells in apoptosis detected with TUNEL. Langerhans cell histiocytosis cells showed strong expression of p53 and in some cases co-expression of Fas and Fas-L. The expression of Fas-L was significantly higher in infiltrates from patients with single-system disease. The actual...... number of pathological Langerhans cells in apoptosis as estimated by TUNEL was low. CONCLUSIONS: The low number of TUNEL-reactive cells can be explained by the rapid turnover of apoptotic cells in the tissue, not leaving the apoptotic cells long enough in the tissue to be detected. The co......-expression of Fas and Fas-L in some Langerhans cells can lead to an autocrine apoptotic shortcut, mediating the death of the double-positive cells. Our findings suggest that apoptosis mediated through the Fas/Fas-L pathway may contribute to the spontaneous regression of lesions in single-system disease. A delicate...

  4. Vulvar ulcer as a presentation of systemic langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Nina A Madnani

    2011-01-01

    Full Text Available We report a 38-year-old housewife with systemic Langerhans cell histiocytosis (LCH presenting as a chronic vulvar and peri-anal ulcer. She had systemic involvement in the form of diabetes insipidus and bone "hot-spots". She responded favorably to etoposide, 6-mercaptopurine, and systemic steroids, and has been in remission since 10 years. Chronic vulvar ulcers not responding to routine therapy should not be neglected and need to be biopsied repeatedly to come to a specific diagnosis. The vulvar ulcer in our case provided a vital clue to a systemic LCH, with a successful outcome.

  5. Extra-osseous involvement of Langerhans' cell histiocytosis in children

    International Nuclear Information System (INIS)

    The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis. (orig.)

  6. Pulmonary Langerhans' Cell Histiocytosis: Report of Six Cases

    OpenAIRE

    SEZGİ, Cengizhan; Abakay, Abdurrahman; DALLI, Ayşe; EREN, Şevval

    2013-01-01

    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare pulmonary interstitial disease. In this report between January 2004 and December 2009 treated by the Dicle University Faculty of Medicine PLCH patients records were retrospectively studied. We found six PLCH patients. Two of six were female and four male. Mean age was 28,2± 7,3. There were only lung invoment in three, lungs and the pituitary involvement in two, lung,and skin involvement in one. Diagnosis of PLCH was established by vide...

  7. Langerhans cell histiocytosis: An uncommon presentation, successfully treated by thalidomide

    Directory of Open Access Journals (Sweden)

    Mohammad Shahidi-Dadras

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease and generally affects children under 15 years of age. Adult onset form and cutaneous features at presentation are uncommon. There are some options for treatment of the skin lesions of LCH such as topical and intralesional corticosteroid, nitrogen mustard, etc., which are not completely curative. Herein, we report a case of perianal LCH in a 20-year-old man with one-year history of recalcitrant well-demarcated, erythematous, and ulcerated plaque surrounding the anal orifice, with pain and difficulty in defecation that was successfully treated with thalidomide.

  8. A rare occurrence of Langerhans cell histiocytosis in an adult

    Science.gov (United States)

    Shevale, Vruturaj V; Ekta, K; Snehal, T; Geetanjal, M

    2014-01-01

    Langerhans Cell Histiocytosis (LCH) is a disease process characterized by accumulation and infiltration of cells, showing ultrastructural and immunohistochemical similarities to Langerhans’ cell, in the affected tissues. It exhibits extreme clinical heterogeneity. LCH was historically divided into 3 clinical entities based on extent of tissue involvement and severity of presentation. These 3 entities were eosinophilic granuloma, Hand-Schuler-Christian disease, Letterer-Siwe disease. Owing to similarities of their histologic appearance, they were grouped together under the term histiocytosis X. It was recently changed to LCH, emphasizing the primary cell involved in the disease process. LCH is a rare disease with an incidenceestimated to be 4.0 to 5.4 per million population. Males are affected twice as frequently as females. The disease may occur at any age with peak incidence in children aged 1 to 3 years. We describe an unusual case of a 65-year-old man who presented with painless swelling in anterior region of mandible. PMID:25948998

  9. Clinical and radiological evolution in patients with pulmonary Langerhans' cell histiocytosis

    NARCIS (Netherlands)

    Westerlaan, HE; van der Valk, PDLPM

    2002-01-01

    Background: Pulmonary Langerhans' cell histiocytosis (LCH) is a diffuse, smoking-related lung disease characterised pathologically by proliferation of abnormal Langerhans' cells, cyst formation and vascular abnormalities, and physiologically by a decreased diffusing capacity. The aim of this study w

  10. Multifocal Langerhans cell histiocytosis of bone: Indications for radiotherapy

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100, 000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology. (authors)

  11. Langerhans Cell Histiocytosis of the Clavicle in a 13-Year-Old Boy

    OpenAIRE

    Parikh, Shital N.; Desai, Vishal R.; Anita Gupta; Anton, Christopher G.

    2014-01-01

    Langerhans Cell Histiocytosis (LCH) is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient’s lesion was treated by curettage, bone grafting, and internal fixa...

  12. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    OpenAIRE

    Routy, B.; J. Hoang; Gruber, J

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette ...

  13. Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anil Gungadeen

    2013-01-01

    Full Text Available Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment.

  14. Imaging findings in Langerhans' cell histiocytosis of the liver and the spleen in an adult

    International Nuclear Information System (INIS)

    We present a case of Langerhans' cell histiocytosis (LCH) of the liver and spleen in an adult. The imaging features are different from those in the few previously reported cases of individual organ involvement by LCH. (orig.) (orig.)

  15. Langerhans Cell Histiocytosis Followed by Hodgkin Lymphoma: A Case Report

    Science.gov (United States)

    Safaei, Akbar; Bagheri, Mandana; Shahryari, Jahanbanoo; Noori, Sadat; Esmailzade, Elmira

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare neoplasm defined as the proliferation of bone marrow langerhans cells, which is a kind of dendritic cells. The major pathological features of LCH are expression of CD1a and S100 as well as Birbeck granules. Its presentation can differ from a mild bone lesion to a multi-systemic evolved malignant neoplasm; however, the latter outcome is almost rare. Thus, LCH is mostly known as a benign neoplasm. In this study, we present a case of LCH followed by Hodgkin lymphoma (HL). Accompaniment of this disease with malignant lymphoma is rare and considered as case report. Several cases in which malignant lymphoma occurred prior to LCH are reported; however, few cases can be found with LCH followed by malignant lymphomas. PMID:25999631

  16. Langerhans cell histiocytosis revisited: Case report with review

    Science.gov (United States)

    Kumar, Y. Pavan; Agrawal, Jayshree; Mohanlakshmi, J.; Kumar, P. Suresh

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist. PMID:26321851

  17. Langerhans cell histiocytosis revisited: Case report with review

    Directory of Open Access Journals (Sweden)

    Y Pavan Kumar

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist.

  18. A Retrospective Analysis of Oral Langerhans Cell Histiocytosis in an Iranian Population: a 20-year Evaluation

    Science.gov (United States)

    Atarbashi Moghadam, Saede; Lotfi, Ali; Piroozhashemi, Batool; Mokhtari, Sepideh

    2015-01-01

    Statement of the Problem Langerhans cell histiocytosis is a rare disease with unknown pathogenesis and is characterized by local or disseminated proliferation of Langerhans cells. There is no previous investigation on prevalence of oral Langerhans cell histiocytosis in Iranian population. Purpose The purpose of this study was to assess the relative frequency of oral Langerhans cell histiocytosis in an Iranian population and to compare the data with previous reports. Materials and Method Pathology files of Oral and Maxillofacial Pathology Department of Dental School of Shahid Beheshti University of Medical Sciences from 1992 to 2012 were searched for cases recorded as oral Langerhans cell histiocytosis. A total number of 20 cases were found and the clinical information of patients was recorded. Results The relative frequency of oral Langerhans cell histiocytosis was 0.34% and the most common location was the posterior mandible. In addition, the mean age of patients was 27 years and there was a definite male predominance. Most lesions were localized and tooth mobility was the most common oral presentation. Conclusion In Iranian population as in many other countries, the relative frequency of oral Langerhans cell histiocytosis is low. Moreover, tooth mobility and periodontal lesions are the frequent early signs of disease. Therefore, in patients with periodontal problems, good oral health, and no response to the treatment; Langerhans cell histiocytosis must be considered. Additionally, although most cases of oral Langerhans cell histiocytosis are localized, systemic involvement must also be considered and dental professionals have an important role in early detection of the disease. PMID:26535408

  19. Langerhans cell histiocytosis : genetic and immunologic fingerprinting

    NARCIS (Netherlands)

    Quispel, W.T.

    2016-01-01

    LCH lesions are characterized by accumulating LCH-cells, which are related to Langerhans and/or Dendritic cells, and the presence of other elements of the immune system. A significant proportion of LCH-cells display somatic mutations in proteins that drive the constitutive activation of the MAPK-pat

  20. Langerhans cell histiocytosis in monozygotic twins with central diabetes insipidus and hypophyseal masses

    Science.gov (United States)

    Wei, Sung-Tai; Chen, Der-Cherng; Cho, Der-Yang; Lin, Hung-Lin

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a systemic disease mainly affecting children and young adults. It can manifest as single system disorder or multi-system involvement. When the central nervous system is involved, the hypothalamic–pituitary axis is the most common location affected. Herein we report a rare case of Langerhans cell histiocytosis in monozygotic twins both with central diabetes and hypophyseal masses. This is the first report about LCH in monozygotic twins with hypophyseal lesions. PMID:25972939

  1. Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying B | Division of Cancer Prevention

    Science.gov (United States)

    DESCRIPTION (provided by applicant): Redefining Langerhans Cell Histiocytosis as a Myeloid Dysplasia and Identifying Biomarkers for Early Detection and Risk Assessment. This application addresses Program Announcement PA-09-197: Biomarkers for Early Detection of Hematopoietic Malignancies (R01). The overall aim of this project is to identify novel biomarkers that may be used to diagnose and treat patients with Langerhans Cell Histiocytosis (LCH). LCH occurs with similar frequency as other rare malignancies including Hodgkin's lymphoma and AML. |

  2. Otic Langerhans' Cell Histiocytosis in an Adult: A Case Report and Review of the Literature

    OpenAIRE

    Anil Gungadeen; Peter Kullar; Philip Yates

    2013-01-01

    Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis...

  3. Pulmonary Langerhans cell histiocytosis causing spontaneous bilateral pneumothorax in a child

    OpenAIRE

    Anupam Patra; Sujit K Bhattacharyay; Arnab Maji; Abhijit Mandal

    2015-01-01

    Bilateral pneumothorax is very rare in childhood. Moreover, if it is due to pulmonary involvement of Langerhans cell histiocytosis, it is even rarer in childhood. In our case, a nonsmoker 12-year-old boy presented with bilateral pneumothorax, whose high-resolution computed tomography scan was highly suggestive of pulmonary Langerhans cell histiocytosis. Excision biopsy of a clinically palpable cervical lymph node and histopathological examination and immunohistochemistry positivity for CD1a i...

  4. Langerhans cell histiocytosis in monozygotic twins with central diabetes insipidus and hypophyseal masses

    OpenAIRE

    Wei, Sung-Tai; Chen, Der-Cherng; Cho, Der-Yang; Lin, Hung-Lin

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a systemic disease mainly affecting children and young adults. It can manifest as single system disorder or multi-system involvement. When the central nervous system is involved, the hypothalamic–pituitary axis is the most common location affected. Herein we report a rare case of Langerhans cell histiocytosis in monozygotic twins both with central diabetes and hypophyseal masses. This is the first report about LCH in monozygotic twins with hypophyseal le...

  5. Unusual location of central nervous system langerhans cell histiocytosis: case report

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis

  6. Pulmonary histiocytosis

    Directory of Open Access Journals (Sweden)

    Milenković Branislava

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, and bone marrow. Pulmonary histiocytosis. Adult pulmonary Langerhans cell histiocytosis is a rare disorder of unknown etiology. It occurs predominantly in male smokers, with an incidence peak between 20 and 40 years of age. High-resolution computed tomography of the chest can show nodules, cavitated nodules, and thickand thin-walled cysts. The definite diagnosis of pulmonary Langerhans cell histiocytosis requires identification of Langerhans’ cell granulomas infiltrating and destroying distal bronchioles, which is usually achieved by lung biopsy at a site selected by chest high-resolution computed tomography. Treatment. Treatment options for adults have never been clarified by a clinical trial. The published literature provides minimal data on the comparative efficacy of various treatment options which include surgery/curettage, steroids, radiation, and various chemotherapy regimens. The improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans cell histiocytosis should help in the development of specific therapeutic strategies and effective treatment.

  7. EXPRESSION OF CELLULAR ADHESION MOLECULES IN LANGERHANS CELL HISTIOCYTOSIS AND NORMAL LANGERHANS CELLS

    NARCIS (Netherlands)

    DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA; TIMENS, W

    1995-01-01

    Langerhans cell histiocytosis (LCH) is characterized by lesions with an accumulation and/or proliferation of Langerhans cells (LCs). Little is known of the etiology and pathogenesis of LCH. Although the relation between the LCH cell and normal LCs is currently uncertain, the localizations of the LCH

  8. Gene expression analysis of dendritic/Langerhans cells and Langerhans cell histiocytosis

    NARCIS (Netherlands)

    Rust, Renata; Kluiver, J.; Visser, Lydia; Harms, G.; Blokzijl, T.; Kamps, W.A.; Poppema, Sibrand; van den Berg, Anke

    2006-01-01

    Langerhans cell histiocytosis (LCH) is a neoplastic disorder that results in clonal proliferation of cells with a Langerhans cell (LQ phenotype. The pathogenesis of LCH is still poorly understood. In the present study, serial analysis of gene expression (SAGE) was applied to LCs generated from umbil

  9. Langerhans cell histiocytosis with presentation as orbital disease

    Directory of Open Access Journals (Sweden)

    Ashok B Bhanage

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration with acute inflammation (1-week duration giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009 but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission.

  10. Langerhans cell histiocytosis with presentation as orbital disease

    Science.gov (United States)

    Bhanage, Ashok B.; Katkar, Anand D.; Ghate, Prajakta S.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is an uncommon multisystem disease with an abnormal polyclonal proliferation of Langerhans cells that invade various organs. In rare instances, the affection of the orbit is the only and the first symptom. We report an unusual case of an 18-month-old male who presented with orbital disease as the first symptom, in the form of chronic presentation of periorbital swelling (2 months duration) with acute inflammation (1-week duration) giving a suspicion of orbital cellulitis. Histopathology after radical excision confirmed the diagnosis of LCH and was advised initial therapy as per Histiocyte Society Evaluation and Treatment Guidelines (2009) but was lost to follow-up only reappearing with progression (multisystem LCH with risk organ involvement) and developed progressive active disease on treatment after 5 weeks. He was treated with salvage therapy for risk patients achieving complete remission. PMID:26167225

  11. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    Science.gov (United States)

    Araujo, Bruno; Costa, Francisco; Lopes, Joanne; Castro, Ricardo

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment. PMID:25977828

  12. Adult Langerhans Cell Histiocytosis with Hepatic and Pulmonary Involvement

    Directory of Open Access Journals (Sweden)

    Bruno Araujo

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment.

  13. Cytological diagnosis of Langerhans cell histiocytosis with cutaneous involvement

    Directory of Open Access Journals (Sweden)

    Sushama A Chandekar

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease affecting predominantly children. The course of the disease varies, from spontaneous resolution to a progressive multisystem disorder with organ dysfunction and potential life-threatening complications. Diagnosis of LCH is often difficult and may be delayed because of its rarity and especially so if it occurs with unusual presentation. Fine needle aspiration cytology of a 4 year old male child, a case of LCH is presented with a purpose of highlighting the characteristic cytological features. A high index of suspicion, awareness of characteristic cytological features of LCH and its differential diagnoses is necessary. This can obviate the need of biopsy and electron microscopy. Immunohistochemistry if available can be performed on cytology smear and cell block.

  14. Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

    Directory of Open Access Journals (Sweden)

    Stephen C Juvet

    2010-01-01

    Full Text Available Pulmonary Langerhans’ cell histiocytosis (PLCH is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.

  15. Multisystem Langerhans cell histiocytosis presenting as an oral lesion

    Directory of Open Access Journals (Sweden)

    Kallarakkal Thomas George

    2013-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare proliferative disorder in which the pathologic Langerhans cells infiltrate and destroy the tissues. Patients with LCH present varied clinical manifestations. Cutaneous lesions in LCH manifest as vesiculopapular eruptions that often mimic various infectious diseases particularly in infants. We present a case of a female infant with an ulcerative lesion intraorally. The baby was asymptomatic otherwise. A detailed history revealed the presence of cutaneous lesions that was overlooked by her parents. Conclusion: This report tries to briefly discuss the current concepts regarding the etiology of LCH. An attempt has been made to emphasis the need for a through systemic examination. The protocol of investigative procedures to be adopted in LCH is also discussed.

  16. Intracranial manifestations of Langerhans cell histiocytosis. MR imaging

    International Nuclear Information System (INIS)

    We report on 3 patients with intracranial manifestations on Langerhans cell histiocytosis (LH). The results are correlated with histological, clinical and radiological reports on some 70 patients described in the literature as suffering from intracranial LH. Two different morphological pictures can be differentiated. First, typical infiltrates can be seen by microscopy; these can be located in every part of the brain, but are seen mainly in the region of te hypothalamus. These infiltrations are shown as space-occupying lesions with Gd-DTPA enhancement. The other manifestation is a demyelinized lesion with a sparse infiltration of Langerhans cells. These lesions, which are located mainly in the region of the nucleus dentatus of the cerebellum and the brain stem, show increased signal intensity in the T2 sequence and no Gd-DTPA enhancement on MRI. (orig.)

  17. Adult langerhans cell histiocytosis with hepatic and pulmonary involvement.

    Science.gov (United States)

    Araujo, Bruno; Costa, Francisco; Lopes, Joanne; Castro, Ricardo

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of Langerhans cells of unknown etiology. It can involve multiple organ systems with different clinical presentation, which complicates the diagnosis. It can range from isolated to multisystem disease with different prognosis. Although common among children, liver involvement is relatively rare in adults and frequently overlooked. Natural history of liver LCH fits into two stages: an early stage with infiltration by histiocytes and a late stage with sclerosis of the biliary tree. Pulmonary findings are more common and include multiple nodules in different stages of cavitation, predominantly in the upper lobes. We present a case of adult LCH with pulmonary and biopsy proven liver involvement with resolution of the hepatic findings after treatment. PMID:25977828

  18. Congenital histiocytosis X

    International Nuclear Information System (INIS)

    Congenital histiocytosis X involving multiple organs is a rare disease that causes rapid mortality in intrauterine and neanatal life. The diagnosis of histiocytosis X (Litterer-Siwe disease should be considered in a neonate with vesiculated crusting skin lesions. We present clinical, radiographic and histopathological findings in a neonate with congenital histiocytosis who died of respiratory failure due to diffuse infilteration of lungs with histiocytic cells. Congenital histiocytosis X is a rare form of Langerhans cell histiocytosis. We report on an infant with congenital histiocytosis X who died within 10 days of birth due to diffuse infiltration of multiple organ systems with Langerhans histiocytic cells. To our knowledge, this is the first case of the radiographic illustration of progressive lung involvement in an infant with histiocytosis X. (orig.)

  19. A patient with lichen sclerosus, Langerhans cell histiocytosis, and invasive squamous cell carcinoma of the vulva

    NARCIS (Netherlands)

    Simons, M.; Nieuwenhof, H.P. van de; Avoort, I.A.M. van der; Bulten, J.; Hullu, J.A. de

    2010-01-01

    We report a patient with vulvar lichen sclerosus, Langerhans cell histiocytosis (LCH), and later vulvar cancer. In LCH, high amounts of non functional Langerhans cells are present in the affected tissue, making it possible that LCH may have contributed to vulvar cancer development in this patient.

  20. Current status of gene transfer into haemopoietic progenitor cells: application to Langerhans cell histiocytosis.

    OpenAIRE

    M. Brenner

    1994-01-01

    A number of recent studies have shown that it is possible to obtain significant levels of gene transfer and expression in marrow progenitor cells and their progeny by using retroviral vectors. The data obtained from these studies and the possible applications to Langerhans cell histiocytosis (LCH) are reviewed.

  1. General Information about Langerhans Cell Histiocytosis (LCH)

    Science.gov (United States)

    ... such as the abdomen , the drugs mainly affect cancer cells in those areas ( regional chemotherapy ). Chemotherapy agents given by injection or by mouth are used to treat LCH. Chemotherapy agents include vinblastine , cytarabine , cladribine , and methotrexate . Nitrogen mustard is a drug that is put ...

  2. Multisystem Langerhans Cell Histiocytosis in Adults Revealed by Skin Lesions.

    Science.gov (United States)

    Atarguine, Hanane; Hocar, Ouafa; Oussmane, Samia; Mouafik, Sara Batoul; Hamdaoui, Abderrachid; Hafiane, Hanan; Belbaraka, Rhizlane; Akhdari, Nadia; Amal, Said

    2016-01-01

    A 37-year-old woman with no remarkable medical or family history presented with papules and vesicles on an erythematous background involving the neck, sacrum, and folds (postauricular, axillary, inguinal, and under the breasts) (Figure 1). During the previous year, she was treated with local and systemic antifungals without improvement. Her history included a secondary amenorrhea, polydipsia, and polyuria (6 L/d) that started 2 years prior. Physical examination revealed chronic bilateral purulent otorrhea with thick eardrums. Histologic examination of skin biopsy revealed a highly suggestive appearance of multisystem Langerhans cell histiocytosis (LCH) with immunohistochemistry (anti-PS100 and anti-CD1a), which were positive (Figure 2A and 2B). Pituitary magnetic resonance imaging showed a thickening of the pituitary stalk in relation to a location histiocytic (Figure 3). Bone gaps were objectified on two radiographic tibial diaphyseal. Results from computed tomography (CT) scan showed a magma coelio mesenteric, axillary, and inguinal lymph nodes. PMID:27319965

  3. Langerhans' cell histiocytosis of the temporal fossa: A case report

    Science.gov (United States)

    LIANG, CHEN; LIANG, QIANLEI; DU, CHANGWANG; ZHANG, XIAODONG; GUO, SHIWEN

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a rare disease with a wide spectrum of clinical manifestations, varying from an isolated lesion to systemic involvement. The etiology of this disease remains to be elucidated. The present study reports a case of LCH with temporal fossa localization in an 8-year-old male patient, who had exhibited left temporal pain and headache for 1 month. Physical examination revealed slight exophthalmos and conjunctival hemorrhage in the patient's left eye, and non-contrast computed tomography imaging of the head revealed a soft tissue mass with unclear margins located in the left temporal fossa, as well as a wide bony defect. Magnetic resonance imaging revealed a heterogeneously contrast-enhanced mass near the left temporal pole, which eroded into the patient's left orbit and maxillary sinus. The lesion was totally excised and confirmed to be LCH through biopsy. PMID:27073529

  4. Solitary Extragnathic Langerhans Cell Histiocytosis – A Rare Case

    Science.gov (United States)

    Reddy, Eppalapally Sharath Kumar; Bhavani, Sangala Naga; A, Krishna; Sekhar, Mane Srinivas Muni

    2015-01-01

    Langerhans cell histiocytosis (LCH), mainly affects the skull, vertebrae, ribs and mandible in children and the long bones of adults. Symptoms range from none to pain, swelling and tenderness over the site of the lesion. This disease presents oral manifestations which can sometimes be the first expression of the condition. It occurs in three forms namely eosinophilic granuloma in which isolated or multiple bones are involved, But has a good prognosis whereas other variants Hand-Shuller-Christian disease (chronic dessiminated variant) and Letterer-Siwe disease (acute dessiminated form) have poor prognosis. Occasionally only soft tissues are affected without bony involvement. Males are more commonly affected than females. This article describes a rare variant of eosinophilic granuloma of labial mucosa without bony involvement. PMID:25954715

  5. Langerhans cell histiocytosis causing cervical myelopathy in a child.

    Science.gov (United States)

    Jang, Kun Soo; Jung, Youn Young; Kim, Seok Won

    2010-06-01

    Langerhans cell histiocytosis (LCH), a disorder of the phagocytic system, is a rare condition. Moreover, spinal involvement causing myelopathy is even rare and unusual. Here, we report a case of atypical LCH causing myelopathy, which was subsequently treated by corpectemy and fusion. A 5-year-old boy presented with 3 weeks of severe neck pain and limited neck movement accompanying right arm motor weakness. CT scans revealed destruction of C7 body and magnetic resonance imaging showed a tumoral process at C7 with cord compression. Interbody fusion using cervical mesh packed by autologus iliac bone was performed. Pathological examination confirmed the diagnosis of LCH. After the surgery, the boy recovered from radiating pain and motor weakness of right arm. Despite the rarity of the LCH in the cervical spine, it is necessary to maintain our awareness of this condition. When neurologic deficits are present, operative treatment should be considered. PMID:20617093

  6. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    Directory of Open Access Journals (Sweden)

    Mozhgan Rezaei Kanavi

    2012-01-01

    Full Text Available Purpose: To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH simulating a limbal papilloma. Case report: A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of "acute bullous inflammation with granulation tissue" was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral patch grafting. Histopathological studies of the excised lesion led to a final diagnosis of LCH. Conclusion: To the best of our knowledge, this is the second report of a rare presentation of LCH in the limbus which recurred after excision of the primary mass. The recurrent lesion was diagnosed based on histopathology and managed accordingly.

  7. Unifocal Langerhans Cell Histiocytosis Simulating a Limbal Papilloma

    Science.gov (United States)

    Rezaei Kanavi, Mozhgan; Javadi, Fatemeh; Javadi, Mohammad-Ali; Faramarzi, Amir

    2012-01-01

    Purpose To report a rare presentation of unifocal Langerhans cell histiocytosis (LCH) simulating a limbal papilloma. Case report A 24-year-old man presented with a limbal mass in his left eye which had initially been suspected to be a papilloma based on clinical findings. The mass was excised and a histopathological diagnosis of “acute bullous inflammation with granulation tissue” was made. The lesion relapsed 10 months later which necessitated repeat resection along with corneoscleral patch grafting. Histopathological studies of the excised lesion led to a final diagnosis of LCH. Conclusion To the best of our knowledge, this is the second report of a rare presentation of LCH in the limbus which recurred after excision of the primary mass. The recurrent lesion was diagnosed based on histopathology and managed accordingly. PMID:23264867

  8. How I treat Langerhans cell histiocytosis

    Science.gov (United States)

    Allen, Carl E.; Ladisch, Stephan

    2015-01-01

    “Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy. PMID:25827831

  9. Pulmonary histiocytosis

    OpenAIRE

    Milenković Branislava; Bogdanović Andrija; Cvok Tijana; Mitić Javorka; Stojšić Jelena; Dudvarski-Ilić Aleksandra

    2013-01-01

    Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, ...

  10. Langerhans cell histiocytosis of the female genital tract.

    Science.gov (United States)

    Axiotis, C A; Merino, M J; Duray, P H

    1991-03-15

    Langerhans cell histiocytosis (LCH) of the female genital tract is rare. Four new cases are reported, and there is a review of the 38 cases in the literature. This disease may involve the vulva, vagina, cervix, endometrium, and ovary. Four distinct patient groups, segregated on the basis of initial presentation and subsequent anatomic extent of disease, were categorized as follows: (1) "pure" genital LCH, (2) genital LCH with subsequent multi-organ involvement, (3) oral or cutaneous LCH with subsequent genital and multi-organ involvement, and (4) diabetes insipidus with subsequent genital and multi-organ disease. Although involvement of the genital tract can occur at any age, it is most common in young adulthood. Clinically, LCH may mimic either primary neoplasia or various inflammatory lesions; the major pathologic differential diagnosis is venereal and other inflammatory diseases. The pure genital form may have a distinct nosologic position in the spectrum of LCH similar to the "pure," self-limited cutaneous histiocytosis seen in infants. There is no correlation between histologic findings and the outcome of the genital lesions. There is also no correlation between clinical presentation and/or the extent of involvement and outcome of genital lesions; complete regression, partial improvement, persistent lesions, and recurrences were seen in all four groups of patients. The treatment of genital LCH is not well defined and is highly individualized. Therapy has included surgery, radiation, topical corticosteroids, topical nitrogen mustard, systemic chemotherapy, and combination therapy; mixed results were obtained with all treatment modalities. Although no modality has been shown to yield a superior outcome, complete surgical excision is advocated as initial therapy.

  11. A case of Langerhans' cell histiocytosis following Hodgkin's disease

    Science.gov (United States)

    LI, XIN; DENG, QI; LI, YU-MING

    2016-01-01

    Langerhans' cell histiocytosis (LCH) is a group of disorders in various tissues characterized by the proliferation of Langerhans cells. It is rarely observed in adults. Langerhans cells are dendritic cells that express cluster of differentiation 1a (CD1a) and S100 protein, and contain Birbeck granules. Its etiopathogenesis remains to be elucidated. One possible etiological cause is a reactive proliferation of Langerhans cells following chemotherapy or radiotherapy for Hodgkin's disease (HD). A number of cases of LCH associated with malignant lymphoma have been reported previously. It may follow after the malignant lymphoma, or occur with it. However, fewer cases have been reported where the LCH followed after HD. In the present case report, a patient was diagnosed with HD following chemotherapy for LCH. As LCH was diagnosed, the patient was treated with a combination of various chemotherapeutic agents in two cycles of cyclophosphamide, vincristine, and prednisolone (COP), and eight cycles of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP). The patient went into a successful clinical remission. One year later, computed tomographic (CT) scans of the thorax and abdomen revealed augmentation of the tumor mass in the mediastinum. An excisional biopsy of the right inguinal lymph node was performed. The patient was diagnosed with nodular sclerosing Hodgkin's disease. Following four cycles of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (ABVD) chemotherapy, a whole-body positron emission tomographic CT scan revealed a decrease in tumor mass in the mediastinum. At present, the patient remains in treatment, and the prognosis has yet to be fully determined. PMID:27330759

  12. Langerhans Cell Histiocytosis of the Clavicle in a 13-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Shital N. Parikh

    2014-01-01

    Full Text Available Langerhans Cell Histiocytosis (LCH is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient’s lesion was treated by curettage, bone grafting, and internal fixation, due to the presence of pathologic fracture. At the 2-year followup, the patient was asymptomatic, and the lesion showed complete resolution without recurrence. The case report highlights the characteristic features of Langerhans Cell Histiocytosis in an unusual location, the knowledge of which would help avoid delayed or missed diagnosis in the future.

  13. Mimicking Stem Cell Niches to Increase Stem Cell Expansion

    OpenAIRE

    Dellatore, Shara M.; Garcia, A. Sofia; Miller, William M.

    2008-01-01

    Niches regulate lineage-specific stem cell self-renewal vs. differentiation in vivo and are comprised of supportive cells and extracellular matrix components arranged in a 3-dimensional topography of controlled stiffness in the presence of oxygen and growth factor gradients. Mimicking stem cell niches in a defined manner will facilitate production of the large numbers of stem cells needed to realize the promise of regenerative medicine and gene therapy. Progress has been made in mimicking com...

  14. Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective.

    Science.gov (United States)

    Roden, Anja C; Yi, Eunhee S

    2016-03-01

    Context .- Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway-targeted treatment might be useful for therapy-resistant patients. Objective . -To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources .- Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions . -Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases. PMID:26927717

  15. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement

    International Nuclear Information System (INIS)

    Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a ''button sequestrum.'' Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement. (orig.)

  16. Pituitary and pulmonary abnormalities in an adult patient with Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Pulmonary histiocytosis is an uncommon interstitial lung disease, which is infrequent in adults and is part of a spectrum of disorders characterized by monoclonal proliferation and infiltration of organs by Langerhans cells. Histiocytosis X affects only the lungs in more than 85% of the cases. We report a case of a 21-years-old male patient with Diabetes Insipidus (DI) and respiratory symptoms due to a spontaneous pneumothorax with favorable clinical evolution. High-resolution CT demonstrated bilateral nodular and cystic pulmonary lesions. MRI revealed a nodular widening of the pituitary stalk. An endoscopic trans bronchial biopsy was negative for Langerhans cells. After thoracoscopy with surgical biopsy of the lung the diagnosis of Langerhans' cell histiocytosis was confirmed. (author)

  17. LANGERHANS CELL HISTIOCYTOSIS - EXPRESSION OF LEUKOCYTE CELLULAR ADHESION MOLECULES SUGGESTS ABNORMAL HOMING AND DIFFERENTIATION

    NARCIS (Netherlands)

    DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA; TIMENS, W

    1994-01-01

    Langerhans' cell histiocytosis (LCH) is characterized by an accumulation of cells with a Langerhans' cell (LC) phenotype. Most patients present with solitary skin or bone lesions, but multi-organ lesions may appear Twenty-two LCH-tissue sections from 13 children and adolescents, with lesions at diff

  18. Disseminated Langerhans' cell histiocytosis and massive protein-losing enteropathy

    Directory of Open Access Journals (Sweden)

    Santos-Machado T.M.

    1999-01-01

    Full Text Available Symptomatic involvement of the gastrointestinal (GI tract as a prominent symptom in Langerhans' cell histiocytosis (LCH is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%, protein-losing enteropathy (33.3% and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.

  19. Bisphosphonates in Langerhans Cell Histiocytosis: An International Retrospective Case Series

    Science.gov (United States)

    Chellapandian, Deepak; Makras, Polyzois; Kaltsas, Gregory; van den Bos, Cor; Naccache, Lamia; Rampal, Raajit; Carret, Anne-Sophie; Weitzman, Sheila; Egeler, R. Maarten; Abla, Oussama

    2016-01-01

    Background Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH), which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results Ten patients (77%) had a single system bone disease, and 3 (23%) had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years). Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve (92%) achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years). One patient did not respond. No major adverse effects were reported in this series. Conclusion Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted. PMID:27413525

  20. Liver involvement of Langerhans’ cell histiocytosis in children

    Science.gov (United States)

    Yi, Xiaoping; Han, Tong; Zai, Hongyan; Long, Xueying; Wang, Xiaoyi; Li, Wenzheng

    2015-01-01

    Objective: Liver involvement is relatively frequent in children with Langerhans cell histiocytosis (LCH). Its features remain poorly defined. Methods: A retrospective study was carried out on 14 hepatic LCH children in our hospital. The Clinicopathological and radiological features of this disease was discussed. Results: The rate of liver involvement in children LCH patients is 51.9%. Majority of the patients were disseminated cases. Hepatomegaly was clinically confirmed in 11 cases (78.6%). Liver function dysfunction was seen in nine (64.3%) children. The association of multi-modal imaging significantly yielded more diagnostic information. There are some imaging characteristics of this disease, CT and MRI could help to assess the staging, extent of the hepatic lesions. We found that liver involvement had a significant impact on survival. Patients treated with systemic chemotherapy earlier from time of diagnosis had a relatively better outcome. Conclusions: The rate of liver involvement in children LCH patients maybe much higher than that of expected. We suggest that clinical and biological liver evaluation and abdominal imaging must be performed regularly onwards to screen every LCH children patient from the time of the initial diagnosis. Patient should be treated with systemic chemotherapy earlier. PMID:26221247

  1. Langerhans cell histiocytosis case with dense metaphyseal band sign.

    Science.gov (United States)

    Kikkawa, Ichiro; Aihara, Toshinori; Morimoto, Akira; Watanabe, Hideaki; Furukawa, Rieko

    2013-02-01

    Eosinophilic granuloma, a type of Langerhans cell histiocytosis, exhibits a classic vertebral collapse, which is called vertebra plana (Calve's disease) and it manifests as a solitary bony lesion. Vertebra plana can cause severe pain in patients. Bisphosphonates (clodronate, pamidronate and zoledronic acid) have been recently used to treat osteolytic bone lesions of LCH. Zoledronic acid has 100 times relative potency that of pamidronate. We report a case of a 10-year-old girl who had zoledronic acid treatment for severe back pain due to vertebra plana. X-ray photographs of the patient's body showed dense metaphyseal band sign, which can be found in lead poisoning, treated leukemia, healing rickets, recovery from scurvy, vitamin D hypervitaminosis, congenital hypothyroidism and hypoparathyroidism. Increased biological potent zoledronic acid deprived her of severe back pain due to vertebra plana and might cause dense metaphyseal band sign of her skeleton. Conclusion; We have cured the severe back pain of a 10-year-old girl case of eosinophilic granuloma with zoledronic acid. After that treatment, X-ray photographs of the patient's body showed dense metaphyseal band sign. There have been few such cases reported until now. PMID:23409985

  2. Langerhan′s cell histiocytosis: A single institutional experience

    Directory of Open Access Journals (Sweden)

    Singh Tejinder

    2010-01-01

    Full Text Available Background: Langerhans cell histiocytosis (LCH is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. Aim: We analyzed our single institutional experience of managing children with LCH. Settings and Design: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated retrospectively. Materials and Methods: Clinicopathological features, laboratory findings, treatment modalities and long-term outcome were analyzed. Results: Children were aged between 2 months and 12 years, with a mean of 3 years. Majority of the children were below 5 years of age. Group B constituted a bulk of children. Disseminated cases were less (five patients. Liver function dysfunction was seen in four (10% children. Pulmonary interstitial infiltrates were seen in two (5% cases. Diabetes insipidus manifested in three patients. There was one death. Conclusion: A better understanding of the etiology and pathogenesis of LCH will result in more directed and efficacious treatment regimens.

  3. IL-17A is not expressed by CD207+ cells in Langerhans Cell Histiocytosis lesions

    OpenAIRE

    Allen, Carl E.; McClain, Kenneth L.

    2009-01-01

    Interleukin-17 (IL-17A) is a pro-inflammatory cytokine that has recently been implicated in pathogenesis of Langerhans Cell Histiocytosis (LCH), a potentially fatal disease characterized by lesions including CD207+ (langerin +) histiocytes. However, in this study we were unable to identify IL-17A gene expression in Langerhans cell lesions, and plasma levels of IL-17A did not correlate with disease activity. Therefore, this study does not support a central role for IL-17A in LCH pathogenesis.

  4. Langerhans cell histiocytosis of the mandible in a six-year-old child

    Directory of Open Access Journals (Sweden)

    Ramani Pratibha

    2007-01-01

    Full Text Available Histiocytosis is a term applied to a group of rare disorders of the reticuloendothelial system. Eosinophilic granuloma, the most benign and localized of the three Langerhans cell histiocytosis entities, may be solitary or multiple. Eosinophilic granuloma can affect almost any bone, but commonly involves the mandible when the jaws are affected. Conventional treatment of LCH is with surgery, radiotherapy, chemotherapy and steroid injections, alone or in combination. Spontaneous regression of localized disease has also been reported. We report a six and a half-year-old patient with Langerhans cell histiocytosis - solitary eosinophilic granuloma of the mandible that initially regressed but rapidly recurred even after radical treatment and had a fatal outcome.

  5. Unusual location of central nervous system langerhans cell histiocytosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, E. Yup; Lee, Jae Kyu; Kim, Chan Kyo; Lee, Chang Hyun; Kang, Chang Ho; Chung, Phil Wook [Armed Forces Capital Hospital, Seongnam (Korea, Republic of)

    2003-03-01

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis.

  6. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, K.-W. [Department of Pediatrics, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); McLeary, M.S. [Div. of Pediatric Radiology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Zuppan, C.W. [Dept. of Pathology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Won, D.J. [Div. of Pediatric Neurosurgery, Loma Linda University Children' s Hospital, Loma Linda, CA (United States)

    2000-05-01

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  7. 18F-Fluorodeoxyglucose PET/CT in Langerhans cell histiocytosis: spectrum of manifestations.

    Science.gov (United States)

    Agarwal, Krishan Kant; Seth, Rachna; Behra, Abhishek; Jana, Manisha; Kumar, Rakesh

    2016-04-01

    The objective of this article is to provide an illustrative tutorial highlighting the utility of 18F-FDG PET/CT imaging to detect the spectrum of manifestations in patients with Langerhans cell histiocytosis. FDG PET/CT is a powerful tool for making an early diagnosis; it allows higher diagnostic confidence with regard to lesions, measuring the extent of disease (staging) and assessing disease activity, and is consequently useful for evaluating the response to therapy in patients with Langerhans cell histiocytosis. PMID:26759026

  8. [Anesthetic Management of a Patient with Langerhans Cell Histiocytosis during Cesarean section].

    Science.gov (United States)

    Ishii, Erika; Takaenoki, Yumiko; Shizukuishi, Masaaki; Fukuda, Isao; Kazama, Tomiei

    2015-04-01

    Langerhans cell histiocytosis is a rare disease, associated with histiocyte increases, and granuloma, in various organs. About 160 patients are reported in Japan. A pregnant patient with a pulmonary Langerhans cell histiocytosis underwent cesarean section under spinal anesthesia. She had repeated pneumothorax with bilateral pulmonary cysts rapidly becoming worse during pregnancy. She was treated with continuous oxygen after 28 weeks of the pregnancy. On 34 weeks of the pregnancy, spinal anesthesia with 0.5% hyperbaric bupivacaine (2 ml) and fentanyl (25 μg) for cesarean section was performed, and provided excellent analgesia without any side-effects. PMID:26419115

  9. Pulmonary langerhans cell histiocytosis: PET/CT for initial workup and treatment response evaluation.

    Science.gov (United States)

    Hansen, Neil J; Hankins, Jordan H

    2015-02-01

    A 40-year-old man underwent pan-endoscopy owing to abdominal pain. Biopsies of the gastrointestinal tract demonstrated diffuse Langerhans cell histiocytosis. PET/CT was done, with CT demonstrating classic pulmonary manifestations of Langerhans cell histiocytosis that had association with intense FDG uptake on PET. Bowel appeared normal. Treatment was initiated with smoking cessation and 6 cycles of cytarabine. Follow-up PET/CT after initial treatment demonstrated improvement of parenchymal abnormalities seen on CT, with resolution of hypermetabolic activity. Maintenance chemotherapy was initiated. PET/CT is increasingly being used for initial staging and treatment response assessment in this rare disorder. PMID:24999688

  10. Differentiating skin-limited and multisystem Langerhans cell histiocytosis

    Science.gov (United States)

    Simko, Stephen J.; Garmezy, Benjamin; Abhyankar, Harshal; Lupo, Philip J.; Chakraborty, Rikhia; Lim, Karen Phaik Har; Shih, Albert; Hicks, M. John; Wright, Teresa S.; Levy, Moise L.; McClain, Kenneth L.; Allen, Carl E.

    2014-01-01

    Objective To identify features associated with multisystem involvement and therapeutic failure in patients with skin Langerhans cell histiocytosis (LCH). Study design We reviewed medical records of 71 consecutive LCH patients with skin involvement evaluated at Texas Children’s Hospital and analyzed clinical features, laboratory results, and presence of circulating cells with the BRAF-V600E mutation, with respect to initial staging and clinical outcomes. Results Skin disease in patients older than 18 months at diagnosis was associated with presence of multisystem disease (OR 9.65, 95% CI 1.17–79.4). Forty percent of patients referred for presumed skin-limited LCH had underlying multisystem involvement, half of these with risk-organ involvement. Patients with skin-limited LCH had 3-year progression-free survival (PFS) of 89% after initial therapy, and none developed multisystem disease. Patients with skin/multisystem involvement had 3 year PFS of 44% with vinblastine/prednisone therapy, and risk-organ involvement did not correlate with failure to achieve non-active disease. Circulating cells with BRAF-V600E were detected at higher frequency in multisystem patients (8/11 skin/multisystem, 1/13 skin-limited, P=0.002). Conclusions Skin-limited LCH requires infrequent therapeutic intervention and has lower risk of progression relative to skin plus multisystem LCH. The less aggressive clinical course and lack of circulating cells with BRAF-V600E mutation in skin-limited LCH suggest a different mechanism of disease origin compared with multisystem or risk-organ disease. PMID:25441388

  11. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Yoo, Jeong Hyun [Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States); Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Rivera, Andreana [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Naeini, Ramin M.; Yedururi, Sireesha; Megahead, Hatem [Baylor College of Medicine, Radiology Department, Houston, TX (United States); Bayindir, Petek [Ege Universitesi, Radiology Department, Lojmanlari (Turkey); Fuller, Gregory N. [MD Anderson Cancer Center, Pathology Department, Houston, TX (United States); Suh, Jeong Soo [Ewha Womans University, Radiology Department, School of Medicine, Seoul (Korea); Adesina, Adekunle M. [Baylor College of Medicine, Pathology Department, Houston, TX (United States); Texas Children' s Cancer Center, Houston, TX (United States); Hunter, Jill V. [Texas Children' s Cancer Center, Houston, TX (United States); Baylor College of Medicine, Neuroradiology Department, Houston, TX (United States)

    2008-05-15

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  12. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  13. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    OpenAIRE

    S. Alavi; N. Hemmati; M.T. Arzanian; Ashena, Z

    2005-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  14. Adult Langerhans cell histiocytosis presenting as metachronous colonic polyps

    Directory of Open Access Journals (Sweden)

    Aloísio Felipe-Silva

    2013-03-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease characterized by proliferation of Langerhans-type cells that express CD1a, Langerin (CD207 and S100 protein. Birbeck granules are a hallmark by ultrastructural examination. LCH presents with a wide clinical spectrum, ranging from solitary lesions of a single site (usually bone or skin to multiple or disseminated multisystemic lesions, which can lead to severe organ dysfunction. Most cases occur in children. Gastrointestinal tract involvement is rare and has been associated with systemic illness and poor prognosis especially in children under the age of 2 years. Adult gastrointestinal LCH is very rare. We report a case of a previously healthy, nonsmoking 48-year-old male who was referred for routine screening colonoscopy. Two sessile, smooth, firm and yellowish LCH polyps measuring 0.2 cm and 0.3 cm were detected in the sigmoid colon. Fifteen months later a second colonoscopy found two histologically confirmed hyperplastic polyps at the sigmoid colon. No other LCH lesions were seen. A third colonoscopy after 28 months of follow-up found a submucosal 0.5 cm infiltrated and ulcerated LCH polyp in the cecum, close to the ostium of the appendix. The patient had been asymptomatic for all this period. Imaging investigation for systemic or multiorgan disease did not find any sign of extracolonic involvement. On histology all lesions showed typical LCH features and immunohistochemical analysis showed strong and diffuse staining for CD1a and CD207. This case illustrates two distinct clinicopathologic features not previously reported in this particular clinical setting: metachronous colonic involvement and positivity for CD207.

  15. Recurrent NRAS mutations in pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Mourah, Samia; How-Kit, Alexandre; Meignin, Véronique; Gossot, Dominique; Lorillon, Gwenaël; Bugnet, Emmanuelle; Mauger, Florence; Lebbe, Celeste; Chevret, Sylvie; Tost, Jörg; Tazi, Abdellatif

    2016-06-01

    The mitogen-activated protein kinase (MAPK) pathway is constantly activated in Langerhans cell histiocytosis (LCH). Mutations of the downstream kinases BRAF and MAP2K1 mediate this activation in a subset of LCH lesions. In this study, we attempted to identify other mutations which may explain the MAPK activation in nonmutated BRAF and MAP2K1 LCH lesions.We analysed 26 pulmonary and 37 nonpulmonary LCH lesions for the presence of BRAF, MAP2K1, NRAS and KRAS mutations. Grossly normal lung tissue from 10 smoker patients was used as control. Patient spontaneous outcomes were concurrently assessed.BRAF(V600E) mutations were observed in 50% and 38% of the pulmonary and nonpulmonary LCH lesions, respectively. 40% of pulmonary LCH lesions harboured NRAS(Q61K) (/R) mutations, whereas no NRAS mutations were identified in nonpulmonary LCH biopsies or in lung tissue control. In seven out of 11 NRAS(Q61K) (/R)-mutated pulmonary LCH lesions, BRAF(V600) (E) mutations were also present. Separately genotyping each CD1a-positive area from the same pulmonary LCH lesion demonstrated that these concurrent BRAF and NRAS mutations were carried by different cell clones. NRAS(Q61K) (/R) mutations activated both the MAPK and AKT (protein kinase B) pathways. In the univariate analysis, the presence of concurrent BRAF(V600E) and NRAS(Q61K) (/R) mutations was significantly associated with patient outcome.These findings highlight the importance of NRAS genotyping of pulmonary LCH lesions because the use of BRAF inhibitors in this context may lead to paradoxical disease progression. These patients might benefit from MAPK kinase inhibitor-based treatments. PMID:27076591

  16. Langerhans cell histiocytosis - eosinophilic granuloma in the maxillo-facial region

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) includes three entities with unknown etiology, which have common histological features where the affected tissues are infiltrated with Langerhans cells. LCH (previously known as Histiocytosis X) comprises rare diseases: Eosinophilic granuloma, Hand-Schueller-Christian syndrome, Letterer-Siwe syndrome, Hashimoto-Pritzke syndrome ect. where the accumulation of pathological Langerhans cells lead to destruction of tissues. Therefore the clinical manifestation of Langerhans cell histiocytosis varies from monoosal or polyosal disorders with unifocal or multifocal bone alteration to dessiminative forms affecting many organs and systems. Eosinophilic granuloma - the localized form of Langerhans cell histiocytosis may flow with single or multiple changes in the skeleton, without involving the internal organs. Eosinophilic granuloma in maxillo-facial region can affect each of the bones, but most often found in the mandible (approx. 70%). First clinical symptoms of EG in maxillo-facial region usually take place in the oral cavity and the bone changes are twice as frequent as oral soft tissue's changes. Sometimes oral manifestation - single or multiple bone and oral soft tissue nonspecific lesions could be the first or the only sign of this disease. That's way early symptoms of Eosinophilic granuloma in maxillo-facial region manifested in jaw area can be first recognized by dental professionals. (authors)

  17. BISPHOSPHONATES IN LANGERHANS CELL HISTIOCYTOSIS: AN INTERNATIONAL RETROSPECTIVE CASE SERIES

    Directory of Open Access Journals (Sweden)

    Deepak Chellapandian

    2016-07-01

    Full Text Available Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH, which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77% had a single system bone disease, and 3 (23% had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years. Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve  (92% achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years. One patient did not respond. No major adverse effects were reported in this series.  Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.

  18. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis.

    Directory of Open Access Journals (Sweden)

    Elena Sieni

    Full Text Available Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy.We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI (group 1 or specific risk factors for (diabetes insipidus, craniofacial bone lesions, but no evidence of, neurodegenerative MRI changes (group 2. All patients underwent clinical, neurophysiological and MRI studies.Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs, and five had abnormal brainstem auditory evoked potentials (BAEPs. MR spectroscopy (MRS showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV and negative predictive value (NPV for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%, 100% (69.2%-100%, 100% (73.5%-100%, and 66.7% (38.4%-88.2%, respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient.A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future therapeutic trials.

  19. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis

    Science.gov (United States)

    Mortilla, Marzia; Savelli, Sara; Grisotto, Laura; Di Giacomo, Gianpiero; Romano, Katiuscia; Fonda, Claudio; Biggeri, Annibale; Guerrini, Renzo; Aricò, Maurizio

    2015-01-01

    Background Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH) is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy. Methods We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI) (group 1) or specific risk factors for (diabetes insipidus, craniofacial bone lesions), but no evidence of, neurodegenerative MRI changes (group 2). All patients underwent clinical, neurophysiological and MRI studies. Results Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs), and five had abnormal brainstem auditory evoked potentials (BAEPs). MR spectroscopy (MRS) showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%), 100% (69.2%-100%), 100% (73.5%-100%), and 66.7% (38.4%-88.2%), respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient. Conclusion A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future

  20. Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

    Science.gov (United States)

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-01-01

    Abstract Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature. A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH. Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking. The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  1. Optimal therapy for adults with Langerhans cell histiocytosis bone lesions.

    Directory of Open Access Journals (Sweden)

    Maria A Cantu

    Full Text Available BACKGROUND: There is little data on treatment of Langerhans cell histiocytosis (LCH in adults. Available data is on small numbers of patients with short follow-up times and no comparison of results from different treatment regimens. We analyzed the responses of adult LCH patients with bone lesions to three primary chemotherapy treatments to define the optimal one. METHODS AND FINDINGS: Fifty-eight adult patients with bone lesions, either as a solitary site or as a component of multisystem disease, were analyzed for disease location and response to surgery, curettage, steroids, radiation, vinblastine/prednisone, 2-Chlorodeoxyadenosine (2-CdA, or cytosine arabinoside (ARA-C. The mean age of patients was 32 years, with equal gender distribution. Twenty-nine patients had 1 lesion; 16, 2 lesions; 5, 3 lesions; and 8 had 4 or more. Most bone lesions were in the skull, spine, or jaw. Chemotherapy, surgery, curettage, or radiation, but not steroids alone, achieved improvement or resolution of lesions in a majority of patients. Comparison of the three chemotherapy regimens revealed 84% of patients treated with vinblastine/prednisone either did not respond or relapsed within a year, whereas 59% of patients treated with 2-CdA and 21% treated with ARA-C failed. Toxicity was worse with the vinblastine/prednisone group as 75% had grade 3-4 neuropathy. Grade 3-4 cytopenias occurred in 37% of the 2-CdA -treated patients and 20% of the ARA-C-treated patients. The major limitation of this study is it is retrospective and not a clinical trial. CONCLUSIONS: ARA-C is an effective and minimally toxic treatment for LCH bone lesions in adults. In contrast, vinblastine/prednisone results in poor overall responses and excessive toxicity.

  2. Pulmonary langerhans cell histiocytosis: case series and literature review.

    Science.gov (United States)

    Wei, Ping; Lu, Hai-Wen; Jiang, Sen; Fan, Li-Chao; Li, Hui-Ping; Xu, Jin-Fu

    2014-11-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature.A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH.Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking.The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis. PMID:25415669

  3. The cognitive spectrum in neurodegenerative Langerhans cell histiocytosis.

    Science.gov (United States)

    Le Guennec, Loïc; Decaix, Caroline; Donadieu, Jean; Santiago-Ribeiro, Maria; Martin-Duverneuil, Nadine; Levy, Richard; Delgadillo, Daniel; Kas, Aurélie; Drier, Aurélie; Magy, Laurent; Bayen, Eleonore; Hoang-Xuan, Khe; Idbaih, Ahmed

    2014-08-01

    Clinical spectrum of cognitive troubles complicating neurodegenerative Langerhans cell histiocytosis (ND-LCH) is poorly known. The aim of this study is to evaluate cognitive functions in ND-LCH. The cognitive functions of a series of eight adult patients (7 males and 1 female; mean age 26 years IQ 25-75; range 20-33) suffering from clinical and/or radiological ND-LCH were evaluated using the following tests: (1) forward/backward digit and spatial span tasks of the WAIS-R scale and the Corsi block task, (2) the French version of the free and cued selective reminding test, (3) verbal fluency tests, (4) the Frontal Assessment Battery (FAB), (5) backward measurement of the verbal and visuospatial memories of the WAIS-R scale, (6) the Rey complex figure test, (7) the trail making tests A and B, (8) digit symbol and symbol search of the WAIS-IV scale, and (9) the Stroop test. Episodic (i.e. autobiographical or personal) memory free recall, categorical verbal fluency, phonological verbal fluency, visuospatial processing skills, attention, speed of processing, and sensitivity to interference were impaired in ND-LCH patients. In contrast, verbal and visuospatial short-term memories (i.e. immediate memories or forward span tasks) were preserved in all patients. Adult ND-LCH patients suffer from a severe but dissociated dysexecutive syndrome, mostly affecting executive strategies and relatively sparing short-term memory. Our study supports the need of assessing executive functions using comprehensive cognitive evaluation in ND-LCH patients for early diagnosis.

  4. Orbital manifestations of Langerhans Cell Histiocytosis: A report of three cases

    Directory of Open Access Journals (Sweden)

    Jayanta K Das

    2009-01-01

    Full Text Available Langerhans Cell Histiocytosis (LCH is a spectrum of disorders characterized by accumulation of histiocytes in various tissues. It is rarely encountered in ophthalmic practice and has an affinity for the orbit. We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

  5. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings

    Energy Technology Data Exchange (ETDEWEB)

    Kilborn, T.N.; Teh, J.; Goodman, T.R

    2003-04-01

    Langerhans cell histiocytosis is a rare disease in children. However, its ability to present in many ways, to mimic other conditions, and to manifest itself in many organs makes it a fascinating disease for radiologists. This article reviews the history of the disease, the features that are most useful in determining prognosis, and the various radiological findings seen in paediatric patients.

  6. Imaging findings in Langerhans` cell histiocytosis of the liver and the spleen in an adult

    Energy Technology Data Exchange (ETDEWEB)

    Mampaey, S. [Dept. of Radiology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium)]|[Dept. of Radiology, University of Antwerp (Belgium); Warson, F. [Dept. of Anatomopathology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium); Hedent, E. van [Dept. of Radiology, Aalsters Stedelijk Ziekenhuis, Aalst (Belgium); Schepper, A.M. de [Dept. of Radiology, University of Antwerp (Belgium)

    1999-02-01

    We present a case of Langerhans` cell histiocytosis (LCH) of the liver and spleen in an adult. The imaging features are different from those in the few previously reported cases of individual organ involvement by LCH. (orig.) (orig.) With 2 figs., 5 refs.

  7. Recurrent adult-onset hypophyseal Langerhans cell histiocytosis after radiotherapy: a case report

    Directory of Open Access Journals (Sweden)

    Funk Ryan K

    2012-10-01

    Full Text Available Abstract Introduction Langerhans cell histiocytosis is a rare disease within the adult population, with very few cases reported as solitary hypophyseal lesions in adults. Of the reported cases, most have been treated successfully with surgery, radiotherapy, and/or chemotherapy. Radiotherapy has been thought to be curative at the relatively low dose of 20Gy. Here we report a case of recurrent hypophyseal Langerhans cell histiocytosis 9 months after radiotherapy with an interval period of symptomatic and radiographic response to therapy. Case presentation A 50-year-old Caucasian woman who had headaches, memory difficulties, and diabetes insipidus was found to have a 2.5cm suprasellar mass. Langerhans cell histiocytosis was diagnosed following stereotactic brain biopsy. Further workup revealed no other lesions. Initial radiation treatment succeeded in shrinking the tumor and relieving clinical symptoms temporarily; however, growth and recurrence of clinical symptoms was noted at 9 months. Re-irradiation was well tolerated and the patient had no acute side effects. Conclusion Isolated hypophyseal involvement by Langerhans cell histiocytosis in adults is a unique presentation of a rare disease. Although radiotherapy doses as low as 20Gy have been reported to offer control, this case demonstrates that higher doses may be warranted to ensure tumor control. With modern imaging and radiotherapy techniques higher doses should offer little increased more durable risk to surrounding critical structures.

  8. Central diabetes insipidus: Is it Langerhans cell histiocytosis of the pituitary stalk? A diagnostic pitfall.

    NARCIS (Netherlands)

    Prosch, H.; Grois, N.; Bökkerink, J.P.M.; Prayer, D.; Leuschner, I.; Minkov, M.; Gadner, H.

    2006-01-01

    Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases. In pediatric and adolescent patients the most common causes for CDI are Langerhans cell histiocytosis (LCH) and germinoma. To avoid a potentially hazardous biopsy of the hypothalamic pituitary region it

  9. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location

    Energy Technology Data Exchange (ETDEWEB)

    Geusens, E.; Brys, P.; Ghekiere, J.; Baert, A.L. [Department of Radiology, University Hospital Gasthuisberg KU Leuven (Belgium); Samson, I. [Department of Orthopedic Surgery, University Hospitals, Leuven (Belgium); Sciot, R. [Department of Pathology II, University Hospitals, Leuven (Belgium); Brock, P. [Department of Pediatrics, University Hospitals, Leuven (Belgium)

    1998-09-01

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. (orig.) With 3 figs., 7 refs.

  10. Diagnosis of Primary Langerhans Cell Histiocytosis of the Vulva in a Postmenopausal Woman

    OpenAIRE

    Sefa Kurt; Mehmet Tunc Canda; Aycan Kopuz; Dudu Solakoglu Kahraman; Abdullah Tasyurt

    2013-01-01

    Langerhans cell histiocytosis (LCH) is a very rare disease of female genital tract, most commonly seen in vulva and unusual in postmenopausal period. Herein, we report the 8th case of pure vulvar LCH in a postmenopausal woman. We pay attention to the differential diagnosis in postmenopausal state, features of pathologic diagnosis, and treatment options.

  11. Langerhans cell histiocytosis forming an asymptomatic solitary nodule in the spleen.

    OpenAIRE

    Lam, K Y; Chan, A C; Wat, M. S.

    1996-01-01

    A case of solitary Langerhans cell histiocytosis (LCH) in the spleen of a 29 year old Chinese man, discovered incidentally at necropsy, is reported. This is the first documented case of LCH confined to the spleen and suggests that LCH should be included in the differential diagnosis of space occupying lesions in the spleen.

  12. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location

    International Nuclear Information System (INIS)

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. (orig.)

  13. Orbital manifestations of Langerhans Cell Histiocytosis: A report of three cases

    OpenAIRE

    Das, Jayanta K.; Ronel Soibam; B K Tiwary; Magdalene, D.; S B Paul; Cida Bhuyan

    2009-01-01

    Langerhans Cell Histiocytosis (LCH) is a spectrum of disorders characterized by accumulation of histiocytes in various tissues. It is rarely encountered in ophthalmic practice and has an affinity for the orbit. We report three patients with LCH involving the lateral orbital wall, each with a different form of the condition.

  14. Paediatric manifestations of Langerhans cell histiocytosis: a review of the clinical and radiological findings

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a rare disease in children. However, its ability to present in many ways, to mimic other conditions, and to manifest itself in many organs makes it a fascinating disease for radiologists. This article reviews the history of the disease, the features that are most useful in determining prognosis, and the various radiological findings seen in paediatric patients

  15. Langerhans cell histiocytosis of the cervical spine: case report of an unusual location.

    Science.gov (United States)

    Geusens, E; Brys, P; Ghekiere, J; Samson, I; Sciot, R; Brock, P; Baert, A L

    1998-01-01

    An unusual location for Langerhans cell histiocytosis of the cervical spine is presented. The osteolytic lesion, instead of being located in the vertebral body, was visualised in the left lateral mass of the fifth cervical vertebra, extending into the vertebral body and through the interapophyseal joint into the lateral mass of the fourth cervical vertebra. PMID:9724427

  16. Persistent pulmonary interstitial emphysema in a case of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Pooja Abbey

    2014-01-01

    Full Text Available We present the case of a 10-month-old boy with multisystem Langerhans cell histiocytosis showing thin-walled lung cysts along with computed tomography (CT evidence of persistent pulmonary interstitial emphysema (PPIE, in the absence of pneumothorax or pneumomediastinum. Follow-up CT performed after 6 months demonstrated complete resolution of interstitial emphysema.

  17. Persistent pulmonary interstitial emphysema in a case of Langerhans cell histiocytosis

    OpenAIRE

    Pooja Abbey; Mahender K Narula; Rama Anand; Jagdish Chandra

    2014-01-01

    We present the case of a 10-month-old boy with multisystem Langerhans cell histiocytosis showing thin-walled lung cysts along with computed tomography (CT) evidence of persistent pulmonary interstitial emphysema (PPIE), in the absence of pneumothorax or pneumomediastinum. Follow-up CT performed after 6 months demonstrated complete resolution of interstitial emphysema.

  18. Role of Fine Needle Aspiration in the Diagnosis of the Rare Disease of Langerhans Cell Histiocytosis in a Child

    OpenAIRE

    Seema Lale; Daniel Soto; Patricia G. Wasserman

    2014-01-01

    Langerhan’s cell histiocytosis (LCH) results from the proliferation of immunophenotypically and functionally immature, morphologically rounded Langerhan’s cells along with eosinophils, macrophages, lymphocytes, and, commonly, multinucleated giant cells. Here we report a case in a 6-year-old boy of differential diagnoses including dermatopathic lymphadenitis (DL), parasitic infection, Kimura’s disease, hypersensitivity reactions, cat-scratch disease, sinus histiocytosis with massive lymphad...

  19. Langerhans Cell Sarcoma Arising from Langerhans Cell Histiocytosis: A Case Report

    OpenAIRE

    Lee, Jong-Sil; Ko, Gyung Hyuck; Kim, Ho Cheol; Jang, In Seok; Jeon, Kyung-Nyeo; Lee, Jeong-Hee

    2006-01-01

    Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomograph...

  20. Immunocytochemical characterization of pulmonary histiocytosis X cells in lung biopsies.

    OpenAIRE

    Soler, P; S. Chollet(Ecole Polytechnique, IN2P3-CNRS); Jacque, C.; Y. Fukuda(Miyagi University of Education); Ferrans, V J; Basset, F.

    1985-01-01

    Morphologic and immunohistochemical studies were made of open lung biopsies from 9 patients with pulmonary histiocytosis X (HX) and 12 patients with other conditions, and of skin biopsies from patients with cutaneous sarcoidosis, Chester-Erdheim disease, and eruptive histiocytoma. The monoclonal antibody OKT6 was detected with the use of goat anti-mouse IgG labeled with fluorescein (FITC) for light microscopy, and sheep antimouse Fab'2 fragment of IgG labeled with horseradish peroxidase (HRP)...

  1. A rare case of "switch on and off" multi-system Langerhans cell histiocytosis in an adult patient

    OpenAIRE

    Froudarakis Marios E; Koutsopoulos Anastasios; Karpathiou Georgia

    2011-01-01

    Abstract Introduction We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a "switch on and off" mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. Case presentation The patient had first presented at the age of 20 years with a solitary lesion of the humerus which spontaneously regressed. At that time, no therapy ...

  2. Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma?

    Science.gov (United States)

    Caltabiano, Rosario; Magro, Gaetano; Vecchio, Giada Maria; Lanzafame, Salvatore

    2010-02-01

    We first report a case of granular cell histiocytosis occurring as a solitary polypoid lesion of the nipple in a 15-year-old girl. Histologically, the lesion was composed of a dermal population of medium- to large-sized, short spindle- to round- to epithelioid-shaped cells with eosinophilic cytoplasm containing numerous and small diastase-resistant periodic acid-Schiff (PAS) positive granules. No associated inflammatory cells were observed. Immunohistochemical studies, revealing immunoreactivity exclusively to vimentin and CD68, were consistent with their histiocytic profile. Based on clinical, morphological and immunohistochemical features, the diagnosis of 'solitary cutaneous histiocytosis with granular cell changes' was proposed. The absence of an inflammatory cell component, such as lymphocytes and leucocytes, along with no history of a previous trauma or medical treatment, suggest that the present lesion could fit into the morphological spectrum of the so-called solitary epithelioid histiocytoma, also known as reticulohistiocytoma. Alternatively, the possibility of a histiocytic reaction to unknown stimuli cannot be completely ruled out. Nevertheless, awareness of solitary cutaneous histiocytosis with granular cell changes is useful to avoid confusion with other dermal tumors, especially 'granular cell tumor' and 'dermal non-neural granular cell tumor'.

  3. B-RAF mutant alleles associated with Langerhans cell histiocytosis, a granulomatous pediatric disease.

    Directory of Open Access Journals (Sweden)

    Takeshi Satoh

    Full Text Available BACKGROUND: Langerhans cell histiocytosis (LCH features inflammatory granuloma characterised by the presence of CD1a+ dendritic cells or 'LCH cells'. Badalian-Very et al. recently reported the presence of a canonical (V600EB-RAF mutation in 57% of paraffin-embedded biopsies from LCH granuloma. Here we confirm their findings and report the identification of two novel B-RAF mutations detected in LCH patients. METHODS AND RESULTS: Mutations of B-RAF were observed in granuloma samples from 11 out of 16 patients using 'next generation' pyrosequencing. In 9 cases the mutation identified was (V600EB-RAF. In 2 cases novel polymorphisms were identified. A somatic (600DLATB-RAF insertion mimicked the structural and functional consequences of the (V600EB-RAF mutant. It destabilized the inactive conformation of the B-RAF kinase and resulted in increased ERK activation in 293 T cells. The (600DLATB-RAF and (V600EB-RAF mutations were found enriched in DNA and mRNA from the CD1a+ fraction of granuloma. They were absent from the blood and monocytes of 58 LCH patients, with a lower threshold of sequencing sensitivity of 1%-2% relative mutation abundance. A novel germ line (T599AB-RAF mutant allele was detected in one patient, at a relative mutation abundance close to 50% in the LCH granuloma, blood monocytes and lymphocytes. However, (T599AB-RAF did not destabilize the inactive conformation of the B-RAF kinase, and did not induce increased ERK phosphorylation or C-RAF transactivation. CONCLUSIONS: Our data confirmed presence of the (V600EB-RAF mutation in LCH granuloma of some patients, and identify two novel B-RAF mutations. They indicate that (V600EB-RAF and (600DLATB-RAF mutations are somatic mutants enriched in LCH CD1a(+ cells and absent from the patient blood. Further studies are needed to assess the functional consequences of the germ-line (T599AB-RAF allele.

  4. "Recurrent Bilateral Spontaneous Pneumothorax in Early Infancy: A Case of Langerhans Cell Histiocytosis"

    Directory of Open Access Journals (Sweden)

    S. Alavi

    2005-07-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax.

  5. Biliary wall calcification in Langerhans cell histiocytosis: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Caruso, Settimo; Miraglia, Roberto; Maruzzelli, Luigi; Luca, Angelo; Gridelli, Bruno [Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IsMeTT), Department of Diagnostic and Interventional Radiology, Palermo (Italy)

    2008-07-15

    Langerhans cell histiocytosis (LCH) is a disorder of unknown pathogenesis affecting one or more organs (unifocal or disseminated form) due to clonal proliferation of Langerhans cells. Liver involvement is more frequent in the disseminated form and the radiological findings of end-stage liver disease due to LCH are similar to those of sclerosing cholangitis. We present the multidetector CT findings in two children with LCH liver involvement and the unique finding of calcification of the biliary wall. (orig.)

  6. Biliary wall calcification in Langerhans cell histiocytosis: report of two cases

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a disorder of unknown pathogenesis affecting one or more organs (unifocal or disseminated form) due to clonal proliferation of Langerhans cells. Liver involvement is more frequent in the disseminated form and the radiological findings of end-stage liver disease due to LCH are similar to those of sclerosing cholangitis. We present the multidetector CT findings in two children with LCH liver involvement and the unique finding of calcification of the biliary wall. (orig.)

  7. Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

    OpenAIRE

    Lovrenski Aleksandra; Đurić Mirna; Klem Ištvan; Eri Živka; Panjković Milana; Tegeltija Dragana; Považan Đorđe

    2013-01-01

    Introduction. Langerhans cell histiocytosis (LCH) is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal le...

  8. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    OpenAIRE

    Adam May; Garvan Kane; Eunhee Yi; Robert Frantz; Robert Vassallo

    2015-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement...

  9. Unusual cutaneous histiocytosis expressing an intermediate immunophenotype between Langerhans' cells and dermal macrophages.

    Science.gov (United States)

    Berti, E; Gianotti, R; Alessi, E

    1988-08-01

    Cutaneous histiocytosis was discovered in a 40-year-old man with a slow-growing nodule located on his right arm. Histologic findings showed an epidermotropic infiltrate of histiocytes with folded, irregular nuclei. Immunologically, the cells presented an intermediate phenotype between Langerhans' cells and dermal macrophages. After surgical removal of the lesion, neither a relapse nor visceral involvement was observed during two years of follow-up.

  10. B-RAF Mutant Alleles Associated with Langerhans Cell Histiocytosis, a Granulomatous Pediatric Disease

    Science.gov (United States)

    Lu, Hui-chun; Mian, Sophie; Trouillet, Celine; Mufti, Ghulam; Emile, Jean-Francois; Fraternali, Franca; Donadieu, Jean; Geissmann, Frederic

    2012-01-01

    Background Langerhans cell histiocytosis (LCH) features inflammatory granuloma characterised by the presence of CD1a+ dendritic cells or ‘LCH cells’. Badalian-Very et al. recently reported the presence of a canonical V600EB-RAF mutation in 57% of paraffin-embedded biopsies from LCH granuloma. Here we confirm their findings and report the identification of two novel B-RAF mutations detected in LCH patients. Methods and Results Mutations of B-RAF were observed in granuloma samples from 11 out of 16 patients using ‘next generation’ pyrosequencing. In 9 cases the mutation identified was V600EB-RAF. In 2 cases novel polymorphisms were identified. A somatic 600DLATB-RAF insertion mimicked the structural and functional consequences of the V600EB-RAF mutant. It destabilized the inactive conformation of the B-RAF kinase and resulted in increased ERK activation in 293 T cells. The 600DLATB-RAF and V600EB-RAF mutations were found enriched in DNA and mRNA from the CD1a+ fraction of granuloma. They were absent from the blood and monocytes of 58 LCH patients, with a lower threshold of sequencing sensitivity of 1%–2% relative mutation abundance. A novel germ line T599AB-RAF mutant allele was detected in one patient, at a relative mutation abundance close to 50% in the LCH granuloma, blood monocytes and lymphocytes. However, T599AB-RAF did not destabilize the inactive conformation of the B-RAF kinase, and did not induce increased ERK phosphorylation or C-RAF transactivation. Conclusions Our data confirmed presence of the V600EB-RAF mutation in LCH granuloma of some patients, and identify two novel B-RAF mutations. They indicate that V600EB-RAF and 600DLATB-RAF mutations are somatic mutants enriched in LCH CD1a+ cells and absent from the patient blood. Further studies are needed to assess the functional consequences of the germ-line T599AB-RAF allele. PMID:22506009

  11. CONGENITAL SELF HEALING CUTANEOUS LANGERHANS CELL HISTIOCYTOSIS WITH MULTIPLE HYPOPIGMENTED MACULES AFTER RECOVERY IN A NEONATE

    Directory of Open Access Journals (Sweden)

    A. Ramyar

    2008-04-01

    Full Text Available Langerhans cell histiocytosis (LCH is the most common type of childhood histiocytic disorder with an incidence of 0.2 to 1 per 100,000 children under the age of 15 years (1. The clinical picture of LCH varies from single system (S-Sbone or skin disease to multi-system disease (M-S (2. We report a neonate with disseminated papulonodular eruption containing mononuclear CD1a and S100 positive histiocytic cells infiltration at epidermis and underlying dermis. The diagnosis of a congenital self healing Langerhans cell histiocytosis (CSHLCH was made and follow up showed a complete recovery of the eruptions , leaving hypopigmented macules in the sites corresponding to the initial findings.

  12. Langerhans cell histiocytosis in adults: a case report and review of the literature

    Science.gov (United States)

    2016-01-01

    Background Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children. Immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective Langerhans cell histiocytosis (LCH) is easy to be misdiagnosed because of its various clinic features and laboratory results. This research focused on the clinicopathological, histopathological, immunohistochemical and other features of LCH and aimed to analyze LCH clinical features for improving diagnosis and decreasing misdiagnosis rate. Case report A case of rare adult LCH was reported and the clinicopathological features were summarized by literature review. The multifocal form of this case includes diabetes insipidus, exophthalmos and mucocutaneous lesions in axillae and anogenital regions, such as infiltrated nodules, extensive coalescing, scaling, crusted papules and ulcerated plaques. The Langerhans cells diffusely infiltrated in the dermis and the tumor cells were positive for CD1a and S-100 expression. The diagnosis was Langerhans cell histiocytosis based on the pathological and immunohistochemical changes. Conclusion LCH has high rate of misdiagnosis and definitive diagnosis depends on pathological biopsy and X-ray examination. The prognosis is related to the onset age and the quantity of affected organs. Although specific therapeutic approach hasn't been well established, combined chemotherapy for multisystem lesions and surgical operation or radiotherapy for unifocal lesions may improve the therapy. PMID:26942568

  13. Cutaneous histiocytosis syndromes.

    Science.gov (United States)

    Roper, S S; Spraker, M K

    1985-11-01

    Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.

  14. Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion

    Energy Technology Data Exchange (ETDEWEB)

    Rajaram, Smitha; Shackley, Fiona; Raghavan, Ashok [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); Wharton, Stephen B. [University of Sheffield, Department of Neurosciences, Sheffield (United Kingdom); Connolly, Daniel J.A. [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); University of Sheffield, Academic Radiology, Sheffield (United Kingdom)

    2010-12-15

    Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic biopsy was performed and histological examination showed diffuse infiltrate of macrophages with foamy cytoplasm. Four months later there was recurrence of seizure activity despite anti-epileptic medication and a repeat MR scan showed a persistent enhancing lesion in the left frontal lobe. Histological examination of the resection specimen resembled juvenile xanthogranuloma (JXG) involving the central nervous system. In the absence of skin lesions a diagnosis of non-Langerhans cell histiocytosis was made. The child made a full recovery following surgery with resolution of his symptoms. (orig.)

  15. Magnetic resonance imaging of thickened pituitary stalk proceeding to langerhans cell histiocytosis in a child

    International Nuclear Information System (INIS)

    Magnetic resonance imaging has shown isolated pituitary stalk thickening in certain cases of idiopathic or secondary central diabetes insipidus (Dl) due to infiltrative processes. We present a 4-year-old boy who was initially diagnosed as having central Dl. The MRI showed isolated pituitary stalk thickening with prominent homogeneous contrast enhancement. The remaining findings on MRI were within normal limits. The patient's personal and family history and laboratory and clinical findings were unremarkable; therefore, he was initially diagnosed as having idiopathic Dl. Since central Dl and isolated pituitary stalk thickening may be considered to be the first manifestations of Langerhans cell histiocytosis, we decided to follow up the patient. After 5 months, following the initial diagnosis, on skeletal X-ray survey, the patient did indeed develop multiple lytic skull lesions which, on biopsy, were histologically typical bone lesions of Langerhans cell histiocytosis. Copyright (2006) Blackwell Science Pty Ltd

  16. Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion

    International Nuclear Information System (INIS)

    Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic biopsy was performed and histological examination showed diffuse infiltrate of macrophages with foamy cytoplasm. Four months later there was recurrence of seizure activity despite anti-epileptic medication and a repeat MR scan showed a persistent enhancing lesion in the left frontal lobe. Histological examination of the resection specimen resembled juvenile xanthogranuloma (JXG) involving the central nervous system. In the absence of skin lesions a diagnosis of non-Langerhans cell histiocytosis was made. The child made a full recovery following surgery with resolution of his symptoms. (orig.)

  17. Polyclonal T-Cells Express CD1a in Langerhans Cell Histiocytosis (LCH) Lesions

    Science.gov (United States)

    West, Jennifer A.; Olsen, Sharon L.; Mitchell, Jenée M.; Priddle, Ross E.; Luke, Jennifer M.; Åkefeldt, Selma Olsson; Henter, Jan-Inge; Turville, Christopher; Kannourakis, George

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a+/CD3+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs). We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH. PMID:25343480

  18. Polyclonal T-cells express CD1a in Langerhans cell histiocytosis (LCH lesions.

    Directory of Open Access Journals (Sweden)

    Jennifer A West

    Full Text Available Langerhans cell histiocytosis (LCH is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a(+/CD3(+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs. We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a(+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH.

  19. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    Directory of Open Access Journals (Sweden)

    Hesam Jahandideh

    2016-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

  20. Congenital solid neck mass: a unique presentation of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Tantiwongkosi, Bundhit [Northeastern Ohio Universities College of Medicine and Canton Affiliated Hospitals, Department of Radiology, Canton, OH (United States); Goske, Marilyn J. [Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Steele, Mark [Akron Children' s Hospital, Department of Pathology, Akron, OH (United States)

    2008-05-15

    Langerhans cell histiocytosis (LCH) presenting in the neonatal period is very rare. In most cases, a self-limited cutaneous disease is the exclusive manifestation. We report an unusual case of neonatal LCH presenting with a large congenital solid neck mass without skin lesions. LCH should be considered in the differential diagnosis of solid masses in neonates and prompt physicians to search for visceral organ involvement. (orig.)

  1. Langerhans cell histiocytosis of long bones: MR imaging and complete follow up study

    Directory of Open Access Journals (Sweden)

    Mohammad A Hashmi

    2012-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a relatively rare disease affecting the reticuloendothelial system in the pediatric age group. It can affect bones, lung, liver, spleen, lymph nodes and skin. MR imaging is particularly informative in diagnosis and management of bone LCH. In this report, we present the initial and 23 months post-treatment MR images of a femoral LCH lesion in a 12-year-old child to describe the role of MRI in bone LCH.

  2. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Ernemann, U.; Skalej, M.; Voigt, K. [Department of Neuroradiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Hermisson, M.; Platten, M. [Department of Neurology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Jaffe, R. [Pathology Department, Children' s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213 (United States)

    2002-09-01

    We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity. (orig.)

  3. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    Science.gov (United States)

    Jahandideh, Hesam; Nasoori, Yasser; Rostami, Sara; Safdarian, Mahdi

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease. PMID:27127670

  4. Anesthesia Technique In A Patient With Langerhans Cell Histiocytosis For Diagnostic Biopsy

    Directory of Open Access Journals (Sweden)

    Abdi S

    2005-07-01

    Full Text Available Histiocytosis under general anesthesia. In these patients, depend on organs involvement and dysfunction, anesthesia technique and specific drugs should be selected.of langerhans cells consist of a range of clinical manifestations, including bone eosimophilic granuloma, Hand-Schuller-Chiristian syndrome and Letterer-Siwe disease. Definitive diagnosis is based on biopsy of involved tissues. This report describes anesthesia technique in a 2 years old girl for diagnostic biopsy

  5. Simultaneous Occurrence of Hodgkin's Lymphoma and Langerhans Cell Histiocytosis of the Spine : A Rare Combination

    OpenAIRE

    Hyun, Seung-Jae; Rhim, Seung-Chul; Huh, Jooryung

    2010-01-01

    Hodgkin's disease presenting with spinal cord compression owing to extradural and bone involvement is extremely unusual. A 48-year-old man presented with progressive lower extremity weakness resulting from spinal cord compression attributable to an epidural mass in the thoracic vertebrae. The patient underwent decompressive surgery, and was then treated with chemotherapy for Langerhans cell histiocytosis. However, the disease progressed, and we performed second decompressive surgery with stab...

  6. CT and MRI preliminary study of langerhans' cell histiocytosis in temporal bone

    International Nuclear Information System (INIS)

    Objective: To study the CT and MRI features of Langerhans' cell histiocytosis affecting the temporal bone. Methods: Ten cases of Langerhans' cell histiocytosis of the temporal bone proved by clinical materials and pathology were retrospectively analyzed. CT was performed in all the 10 cases. MRI was also performed in 5 cases. Results: Temporal bone involvement presented as an isolated manifestation in 5 cases and was associated with lesions of other organs in the remaining 5 cases. External auditory canal and mastoid process were involved in 10 cases, squamous and petrous parts in 8 cases, and middle ear in 7 cases on CT scans. Destruction of ossicles and bony labyrinth were detected in 2 cases, respectively. CT showed massive lytic destruction in the affected region, associated with large soft tissue masses. The lesions had irregular border and well-defined margin without osteosclerosis in 8 cases. On MR imaging, external auditory canal and mastoid process were involved in 5 cases, squamous and petrous parts in 4 cases, middle ear in 3 cases, and membranous labyrinth in 1 case. The lesions were isointense or hypointense compared to brain on T1WI and hyperintense on T2WI in 5 cases. Postcontrast CT and MR imaging scans demonstrated marked enhancement. Conclusion: CT may clearly depict the bony lesions of the temporal bone in Langerhans' cell histiocytosis, and enhance the diagnostic accuracy to a great degree. MRI may effectively define the extent in the temporal bone, especially the intracranial invasion, and aid the selection of the optimal treatment procedure. Together with the two imaging modalities, the diagnosis, treatment, and follow-up would be greatly improved in patients with the temporal bone Langerhans' cell histiocytosis

  7. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

    International Nuclear Information System (INIS)

    We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity. (orig.)

  8. Congenital solid neck mass: a unique presentation of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) presenting in the neonatal period is very rare. In most cases, a self-limited cutaneous disease is the exclusive manifestation. We report an unusual case of neonatal LCH presenting with a large congenital solid neck mass without skin lesions. LCH should be considered in the differential diagnosis of solid masses in neonates and prompt physicians to search for visceral organ involvement. (orig.)

  9. Laryngeal Langerhans Cell Histiocytosis Presenting with Neck Mass in an Adult Woman

    OpenAIRE

    Hesam Jahandideh; Yasser Nasoori; Sara Rostami; Mahdi Safdarian

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a very rare condition that commonly affects the head and neck region. There are very few cases of isolated laryngeal involvement by LCH, mostly reported in pediatric patients. Here, we report a case of laryngeal LCH in a 62-year-old woman presenting with a neck mass several weeks ago. The clinical and histopathological findings are reported with a brief discussion about the disease.

  10. Langerhans cell histiocytosis in adults: Contribution of PET-CT with 18F-FDG

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a rare disease whose clinical presentation is highly variable, and with unpredictable outcome. Once the diagnosis is established, evaluation of the extent of the disease is required for therapeutic purposes and prognosis. PET-CT with 18F-FDG can detect multi systemic involvement, demonstrating metabolically active lesions. We present a case report showing the utility of PET-CT in staging and therapy response evaluation

  11. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

    OpenAIRE

    M. Vaziri; A. Pazooki L. Zahedi

    2008-01-01

    Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was ...

  12. Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis.

    OpenAIRE

    Grobost V.; Khouatra C.; Lazor R; Cordier J.F.; Cottin V

    2014-01-01

    BackgroundPulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte proli...

  13. Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan

    OpenAIRE

    Castoldi, M. C.; A. Verrioli; De Juli, E.; Vanzulli, A.

    2014-01-01

    Objectives Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. Methods This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are i...

  14. Intriguing bronchoalveolar lavage proteome in a case of pulmonary langerhans cell histiocytosis

    OpenAIRE

    Ghafouri, Bijar; Persson, H Lennart; Tagesson, Christer

    2013-01-01

    Background: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease associated with tobacco smoke exposure. New insights into its pathogenesis and how it differs from that of chronic obstructive pulmonary disease (COPD) may be provided by proteomic studies on bronchoalveolar lavage fluid (BALF). Case Report: We present the BALF proteome in a biopsy-proven case of PLCH and compare it with typical proteomes of COPD and of the healthy lung. The BALF proteins were separ...

  15. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    OpenAIRE

    Earlam, K.; Souza, C.A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are biz...

  16. Pulmonary Langerhans Cell Histiocytosis X Presented with Bilateral Pneumothorax: A Case Report

    OpenAIRE

    Majed Al-Mourgi

    2014-01-01

    Background: The report describes the case of a 20-year-old Saudi male patient with Pulmonary Langerhans cell histiocytosis X (PLCH). He presented with bilateral chest pain of few hours duration starting first on the right side. The patient had also dyspnea and non productive cough. The patient was heavy smoker for 6 years. General examination revealed that the patient had tachypnea but he was hemodynamically stable. Chest examination showed bilateral distant breath sounds but no hyperresonanc...

  17. Extrathoracic investigation in adult patients with isolated pulmonary langerhans cell histiocytosis

    OpenAIRE

    Tazi, Abdellatif; de Margerie-Mellon, Constance; Vercellino, Laetitia; Naccache, Jean Marc; Fry, Stéphanie; Dominique, Stéphane; Jouneau, Stéphane; Lorillon, Gwenaël; Bugnet, Emmanuelle; Chiron, Raphael; Wallaert, Benoit; Valeyre, Dominique; Chevret, Sylvie

    2016-01-01

    Background An important objective on diagnosis of patients with Langerhans cell histiocytosis (LCH) is to determine the extent of disease. However, whether systematic extrathoracic investigation is needed in adult patients with clinically isolated pulmonary LCH (PLCH) has not been evaluated. Methods In this prospective, multicentre study, 54 consecutive patients with newly diagnosed clinically isolated PLCH were systematically evaluated at inclusion by bone imaging and blood laboratory testin...

  18. Presentation of pulmonary Langerhans cell histiocytosis before the development of lung cysts

    OpenAIRE

    Martin, Irene; Ballester, Marta; Ruiz, Yolanda; Llatjós, Roger; Alarza, Fernando; Molina, Maria

    2013-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) affects mainly young, predominantly smoking adults with a peak at 20–40 years of age. Patients with PLCH often present with a nonproductive cough and/or dyspnea. High-resolution CT (HRCT) is the most important diagnostic modality in PLCH. The typical HRCT pattern combines small poorly limited nodules, cavitated nodules, and finally thick- and thin-walled cysts. In rare cases, HRCT enables PLCH to be diagnosed prior to the development of cysts.

  19. Modern concepts of pathogenesis and morphogenesis of pulmonary Langerhans’ cell histiocytosis of adults

    OpenAIRE

    Liskina, I. V.

    2013-01-01

    The article presents detailed information about modern pathogenetic mechanisms of pulmonary Langerhans’ cell histiocytosis of adults, as an isolated no neoplastic process, which  in many moments of its development is uncontrolled abnormal immune response to a number of antigens not fully qualified by nature. Morphogenesis of pathology is described in details, as well as all stages of the disease, with emphasis on diagnostic morphological signs. Characteristic diagnostic biopsy microphotograph...

  20. Clinical and Radiological Presentation of Pulmonary Langerhans Cell Histiocytosis: A Case Series of 20 Patients

    OpenAIRE

    A. Fakharian; H. Jamaati; M.O. Tahbaz; S. Khalilzadeh; Pour Abdollah, M.; M. Behkam Shadmehr

    2008-01-01

    Introduction & Objective: Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon (5% ILD) interstitial tissue disorder with significant importance. It occurs predominantly in adult smokers. The organs involved in LCH include skin, bone, pituitary gland, thyroid, lymph node, and lungs.Materials & Methods: In this descriptive study all patients admitted to the Masih Daneshvari Hospital in Tehran from 1996 to 2007 diagnosed with LCH (recognized by clinical features, laboratory investigati...

  1. Effectiveness of cladribine therapy in patients with pulmonary Langerhans cell histiocytosis

    OpenAIRE

    Grobost, Vincent; Khouatra, Chahera; Lazor, Romain; Cordier, Jean-François

    2014-01-01

    BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterised by granulomatous proliferation of CD1a-positive histiocytes forming granulomas within lung parenchyma, in strong association with tobacco smoking, and which may result in chronic respiratory failure. Smoking cessation is considered to be critical in management, but has variable effects on outcome. No drug therapy has been validated. Cladribine (chlorodeoxyadenosine, 2-CDA) down-regulates histiocyte pro...

  2. The natural history of adult pulmonary Langerhans cell histiocytosis: a prospective multicentre study

    OpenAIRE

    Tazi, Abdellatif; de Margerie, Constance; Naccache, Jean Marc; Fry, Stéphanie; Dominique, Stéphane; Jouneau, Stéphane; Lorillon, Gwenaël; Bugnet, Emmanuelle; Chiron, Raphael; Wallaert, Benoit; Valeyre, Dominique; Chevret, Sylvie

    2015-01-01

    Background The natural history of pulmonary Langerhans cell histiocytosis (PLCH) has been unclear due to the absence of prospective studies. The rate of patients who experience an early progression of their disease is unknown. Additionally, conflicting effects of smoking cessation on the outcome of PLCH have been reported. Methods In this prospective, multicentre study, 58 consecutive patients with newly diagnosed PLCH were comprehensively evaluated over a two-year period. Our objectives were...

  3. HISTIOCYTOSIS X: CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    E. Y. Ponomareva

    2014-07-01

    Full Text Available Two clinical cases of pulmonary Langerhans cell histiocytosis X have been analyzed demonstrating lung and other inner organ pathology, common clinical and X-ray features but different life prognosis.

  4. HISTIOCYTOSIS X: CLINICAL OBSERVATIONS

    OpenAIRE

    E. Y. Ponomareva; A. P. Rebrov; E. E. Archangelskaja; A. A. Roshchina; R. N. Steshenko; A. S. Bukia; N. V. Dvornikova; N. N. Moskaleva

    2014-01-01

    Two clinical cases of pulmonary Langerhans cell histiocytosis X have been analyzed demonstrating lung and other inner organ pathology, common clinical and X-ray features but different life prognosis.

  5. Button sequestrum in a case of localized Langerhans' cell histiocytosis of the ilium: case report

    International Nuclear Information System (INIS)

    Langerhans' cell histiocytosis (LCH) is characterized by a proliferation of cells exhibiting the same immunohistochemical and ultra-structural characteristics as Langerhans' cells of the epidermis. Eosinophilic granuloma, chronic polyostotic disease (Hand-Schuller-Christian disease) and multisystemic disease (Letterer-Siwe disease) all belong to the spectrum LCH. Osseous lesions are the most common findings. the radiological appearance of skeletal LCH depends on the site and on disease activity. Button sequestrum has been described as uncommon in LCH of the skull and exceedingly rare in LCH at other sites. We report a case of localized LCH of the ilium with a button sequestrum. (author)

  6. Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b)

    Energy Technology Data Exchange (ETDEWEB)

    Herman, M.; Demaerel, P.; Wilms, G. [University Hospitals Leuven, Department of Radiology, Leuven (Belgium); Gool, S. van [University Hospitals Leuven, Department of Pediactrics, Leuven (Belgium); Casteels, I. [University Hospitals Leuven, Department of Ophthalmology, Leuven (Belgium)

    2007-12-15

    We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before. (orig.)

  7. Langerhans Cell Histiocytosis Arising from the Mandible as Diagnosed by US-guided Core Biopsy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Soo Jin [Center of Thyroid Cancer, National Cancer Center, Goyang (Korea, Republic of); Kim, Eun Kyung [Research Institute of Radiological Science, Yonsei University Heath System, Seoul (Korea, Republic of); Lee, Min Kyung [Eulji University College of Medicine, Eulji University Hospital, Daejeon (Korea, Republic of)

    2010-09-15

    Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells. Although LCH is not considered a malignant disease, its appearance on radiographs may be similar to that of a malignant tumor. The diagnosis of LCH is usually made by a soft tissue biopsy, or by bone marrow aspiration or curettage. We present a patient with a mandibular mass confirmed to be LCH by US-guided core needle biopsy, and present a strategy for diagnosing localized LCH of the bone based on the usefulness and reliability of the percutaneous biopsy

  8. Lacrimal gland and perioptic nerve lesions due to Langerhans cell histiocytosis (2007: 9b)

    International Nuclear Information System (INIS)

    We report a patient presenting with bilateral lacrimal gland involvement and perioptic nerve sheath lesions due to Langerhans cell histiocytosis (LCH) invasion. LCH is a rare multisystemic disease characterized by a clonal proliferation of Langerhans cells. All organs may be involved with a clinical spectrum ranging from a solitary bone lesion to a severe life-threatening multisystem disease. Osteolytic orbital bone lesions with extension into the adjacent orbital soft tissues have been described. To our knowledge, lacrimal gland involvement has probably been described only once before. Perioptic nerve lesions are also very rare, having been described only three times before. (orig.)

  9. Langerhans Cell Histiocytosis Arising from the Mandible as Diagnosed by US-guided Core Biopsy

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a clonal proliferative disorder of Langerhans cells. Although LCH is not considered a malignant disease, its appearance on radiographs may be similar to that of a malignant tumor. The diagnosis of LCH is usually made by a soft tissue biopsy, or by bone marrow aspiration or curettage. We present a patient with a mandibular mass confirmed to be LCH by US-guided core needle biopsy, and present a strategy for diagnosing localized LCH of the bone based on the usefulness and reliability of the percutaneous biopsy

  10. [The Langerhans cell histiocytosis with thymic localization as initial and exclusive place].

    Science.gov (United States)

    Hernández Pérez, J M; Franquet Casas, T; Rodríguez, S; Giménez, A

    2007-10-01

    The Langerhans' cell histiocytosis (LCH), also known as Histiocitosis X it is an illness not very frequent granulomatosus etiology not clarified yet, that it can have different manifestations and localizations, however the thymic localization as initial and exclusive place gives presentation HCL it is quite unusual. The present case is presented a patient that debuted with a clinical unspecific, where the tests give image they put she gives apparent a mass in previous mediastinum and that after the pathologic and immunohistochemical analysis they evidenced a proliferation Langerhans s cells and eosinophils it being positive for CD1a and S-100 confirming the diagnosis of the LCH.

  11. Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

    Directory of Open Access Journals (Sweden)

    R. Saxena

    2009-09-01

    Full Text Available We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

  12. Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

    OpenAIRE

    R. Saxena; Pati, H. P.; Dutta, S.; Kar, R.; Sharma, P.

    2009-01-01

    We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

  13. Radiotherapy of Langerhans' cell histiocytosis. Results and Implications of a national patterns-of-care study

    International Nuclear Information System (INIS)

    Purpose: This patterns-of-care study was performed to define the current clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany and to define open questions resulting from this study. Material and Methods: A standardized questionnaire was sent to 198 German radiotherapy institutions. Data about patient characteristics, stage of disease, practice and fractionation of radiotherapy, outcome of therapy, etc. were systematically evaluated. 123 of 198 institutions answered the complete questionnaire (62.1%). Results: Only 23 of the 123 institutions (18.7%) reported experience with radiotherapy of Langerhans' cell histiocytosis of adults. 18 institutions with 98 patients were evaluable. The majority of patients (72 of 98) was treated on a linear accelerator. The median single dose of radiotherapy was 2 Gy, while the median total dose was 24 Gy. 81 of 89 evaluable patients (91%) reached a local control of the treated lesion(s), 69 of those had a complete remission. Eight of 89 patients (9%) developed an in-field recurrence. 87.8% of patients experienced no acute and 97% of patients no late side effects of radiotherapy. Conclusion: Clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany is still very limited. Nevertheless, the clinical results - with high remission and local control rates - confirm the effectiveness of radiotherapy in the multidisciplinary treatment of this disease. Due to the small number of patients in this study despite higher incidence rates, the knowledge of this disease has to be multiplied in Germany. Future patients should be systematically included into a prospective radiotherapy registry. (orig.)

  14. Langerhans` cell histiocytosis of the spine: use of MRI in guiding biopsy

    Energy Technology Data Exchange (ETDEWEB)

    Kaplan, G.R.; Saifuddin, A. [Department of Diagnostic Imaging, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom); Pringle, J.A.S. [Department of Morbid Anatomy, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom); Noordeen, M.H.; Mehta, M.H. [Department of Spinal Deformities, Royal National Orthopaedic Hospital Trust, Brockley Hill (United Kingdom)

    1998-12-01

    The MRI features of two cases of spinal Langerhans` cell histiocytosis with multilevel involvement are presented in which MRI was of help in differentiating active from inactive healing lesions by the demonstration of signal changes in the vertebral body marrow of the active lesion, manifest as low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. This distinction could not be made by plain radiography or bone scintigraphy. In cases where biopsy is required for diagnosis, MRI is recommended to guide the biopsy towards levels suggestive of active involvement. (orig.) With 7 figs., 13 refs.

  15. Langerhans' cell histiocytosis of the clivus: case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Hurley, M.E.; Hayes, R. [Dept. of Radiology, Our Lady' s Hospital for Sick Children, Crumlin, Dublin (Ireland); O' Meara, A. [Dept. of Oncology, Our Lady' s Hospital for Sick Children, Dublin (Ireland); Fogarty, E. [Dept. of Orthopaedics, Our Lady' s Hospital for Sick Children, Dublin (Ireland)

    2004-03-01

    This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus. (orig.)

  16. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    Directory of Open Access Journals (Sweden)

    Sung Hyun Kim

    2016-01-01

    Full Text Available Single-site, single-system Langerhans cell histiocytosis (LCH of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed.

  17. The rapid evolution of CT findings in pulmonary langerhans cell histiocytosis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Tae Wook; Lee, Kyung Soo; Cho, Eun Yoon [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2007-07-15

    Imaging findings of pulmonary Langerhans cell histiocytosis (PLCH) demonstrate evolving changes over time, and the radiological transitions shown by imaging tools may allow a prediction of histopathological activity in PLCH. However, there are no reports describing how rapidly CT findings change with time. We describe a case of PLCH that showed a rapid evolutional change of the pulmonary lesions in a 48-year-old man, in which the nodular lesions showed cystic changes within two-month follow-up periods on chest CT scans.

  18. An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Furmanczyk, Paul S. [University of Washington Medical Center, Department of Pathology, 1959 NE Pacific, Room BB220, P.O. Box 356100, Seattle, WA (United States); Bruckner, James D. [University of Washington Medical Center, Department of Orthopaedics and Sports Medicine, Seattle, WA (United States); Gillespy, Thurman [Harborview Medical Center, Department of Radiology, Seattle, WA (United States); Rubin, Brian P. [The Cleveland Clinic, Department of Anatomic Pathology, Cleveland, OH (United States)

    2007-09-15

    Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are both exceedingly rare histiocytic proliferations that can involve the skeletal system. We report on a case of ECD with some features suggestive of LCH. Radiographs demonstrated a large lytic lesion in the left femur, with multiple lesions of sclerosis involving both distal femurs and tibias. Both the lytic lesion and a sclerotic lesion were biopsied and demonstrated distinctive histologic features characteristic of ECD in the tibia and features of LCH in the femur. The clinical/radiologic and pathologic features that distinguish ECD and LCH as distinct entities are reviewed, and the underlying biological connection between them is discussed. (orig.)

  19. MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Koenig, C.W.; Pfannenberg, C.; Truebenbach, J.; Remy, C.; Claussen, C.D. [Tuebingen Univ. (Germany). Abt. fuer Radiologische Diagnostik; Boehmer, G.M. [Dept. of Gastroenterology, Univ. of Tuebingen (Germany); Ruck, P. [Inst. of Pathology, Univ. of Tuebingen (Germany)

    2001-12-01

    Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients. (orig.)

  20. BRAF V600E-Positive Multisite Langerhans Cell Histiocytosis in a Preterm Neonate

    Directory of Open Access Journals (Sweden)

    Sara V. Bates

    2013-10-01

    Full Text Available Hemorrhagic pustules with a “blueberry muffin” appearance accompanied by respiratory failure in a neonate present a challenging differential diagnosis that includes infections and neoplasms. We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH, positive for the oncogenic BRAF V600E mutation, in a preterm neonate. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. This case is unusual in that the newborn presented with severe multiorgan involvement. Due to the rare incidence, wide spectrum of clinical manifestations, and high mortality rate, clinicians must maintain a high index of suspicion for LCH.

  1. Langerhans cell histiocytosis following Hodgkin lymphoma: A case report from Iran

    Directory of Open Access Journals (Sweden)

    Nahid Reisi Dehkordi

    2010-01-01

    Full Text Available The occurrence of Langerhans Cell Histiocytosis (LCH in a patient with lymphoma is an indication of a probable relationship between them. The two conditions have similarities both clinically and histopathologically. Occurrence of these two conditions in the same patient, particularly not simultaneously, is rare. According to different management and treatment of these conditions, exact histopathologic evaluation and even using immunohistochemistery (IHC can prevent misdiagnosis. In this report, a 10 year old boy presented who afflicted with LCH 3 years after diagnosis and treatment of mixed cellularity Hodgkin lymphoma.

  2. A rare case of Langerhans cell histiocytosis of the skull in an adult: a systematic review

    Directory of Open Access Journals (Sweden)

    Corinna Chiong

    2013-07-01

    Full Text Available We report a 41-year old male who presented to the Emergency Department after falling while water-skiing. He had a previous medical history included chronic headaches, which had persisted for the last 2-3 months prior to presentation. Computed tomography of the head showed a small hypersensitivity with a small extra axial collection with a maximum thickness of 1 mm. Differential diagnoses included an arachnoid cyst, haemangioma, meningioma or a secondary lesion. A diagnosis of Langerhans Cell Histiocytosis was made based on the histopathology examination and the immunoperoxidase staining.

  3. Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

    Science.gov (United States)

    Kim, Sung Hyun; Choi, Moon Young

    2016-01-01

    Single-site, single-system Langerhans cell histiocytosis (LCH) of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed. PMID:26933424

  4. Extra-osseous involvement of Langerhans' cell histiocytosis in children

    Energy Technology Data Exchange (ETDEWEB)

    Schmidt, Sabine; Gudinchet, Francois [Departments of Radiology and Interventional Radiology, University Hospital Centre - CHUV, Lausanne (Switzerland); Eich, Georg [Department of Paediatric Radiology, Children' s Hospital, Zuerich (Switzerland); Hanquinet, Sylviane [Department of Paediatric Radiology, Hopital Cantonal, Geneva (Switzerland); Tschaeppeler, Heinz [Department of Paediatric Radiology, Children' s Hospital, Bern (Switzerland); Waibel, Peter [Department of Paediatric Radiology, Children' s Hospital, St. Gallen (Switzerland)

    2004-04-01

    The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis. (orig.)

  5. Langerhans' cell histiocytosis of the spine: use of MRI in guiding biopsy

    International Nuclear Information System (INIS)

    The MRI features of two cases of spinal Langerhans' cell histiocytosis with multilevel involvement are presented in which MRI was of help in differentiating active from inactive healing lesions by the demonstration of signal changes in the vertebral body marrow of the active lesion, manifest as low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences. This distinction could not be made by plain radiography or bone scintigraphy. In cases where biopsy is required for diagnosis, MRI is recommended to guide the biopsy towards levels suggestive of active involvement. (orig.)

  6. The rapid evolution of CT findings in pulmonary langerhans cell histiocytosis: a case report

    International Nuclear Information System (INIS)

    Imaging findings of pulmonary Langerhans cell histiocytosis (PLCH) demonstrate evolving changes over time, and the radiological transitions shown by imaging tools may allow a prediction of histopathological activity in PLCH. However, there are no reports describing how rapidly CT findings change with time. We describe a case of PLCH that showed a rapid evolutional change of the pulmonary lesions in a 48-year-old man, in which the nodular lesions showed cystic changes within two-month follow-up periods on chest CT scans

  7. MR cholangiography in the diagnosis of sclerosing cholangitis in Langerhans' cell histiocytosis

    International Nuclear Information System (INIS)

    Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients. (orig.)

  8. An unusual case of Erdheim-Chester disease with features of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) are both exceedingly rare histiocytic proliferations that can involve the skeletal system. We report on a case of ECD with some features suggestive of LCH. Radiographs demonstrated a large lytic lesion in the left femur, with multiple lesions of sclerosis involving both distal femurs and tibias. Both the lytic lesion and a sclerotic lesion were biopsied and demonstrated distinctive histologic features characteristic of ECD in the tibia and features of LCH in the femur. The clinical/radiologic and pathologic features that distinguish ECD and LCH as distinct entities are reviewed, and the underlying biological connection between them is discussed. (orig.)

  9. [Symptomatic Langerhans-cell-histiocytosis of the cervical spine in a child: case report].

    Science.gov (United States)

    Schroers, C; Donauer, E; Laudan, M; Herbst, E W; Barz, H

    2000-01-01

    We report on a six year old female presented with a painful torticollis and a hemidysaesthesia caused by destruction of the third cervical vertebra and a paravertebral soft-tissue mass. At diagnostic routine finally a biopsy gives the diagnosis of Langerhans cell histiocytosis. In a second open approach the destructed vertebral body was replaced by a precisely adjusted autologous bone interponate and the patient was maintained in halo vest immobilisation. The outcome is described and an overview of the current literature is given. PMID:10916784

  10. Langerhans cell histiocytosis of the cervical spine in an adult: a case report.

    Science.gov (United States)

    Sayhan, Salih; Altinel, Deniz; Erguden, Cenk; Kizmazoglu, Ceren; Guray, Merih; Acar, Umit

    2010-07-01

    We present a case of a 47-year-old-woman with a complaint of cervical pain with paresthetic appearance on her left arm. She was treated with analgetics. Further radiological evaluation because of the persistent pain revealed an osteolytic destruction of the fourth cervical vertebra. The patient underwent anterior cervical corpectomy with total excision of the tumor. Stabilization of the cervical spine was performed. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH) of the cervical spine. This case report presents the histopathological evaluation, diagnostic work-up and the treatment procedures because of rarity of cervical spinal LCH cases in the literature. PMID:20669118

  11. Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation.

    Science.gov (United States)

    Kinoshita, Yoshiaki; Watanabe, Kentaro; Sakamoto, Atsuhiko; Hidaka, Kouko

    2016-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking. PMID:26935369

  12. Langerhans' cell histiocytosis of the clivus: case report and literature review

    International Nuclear Information System (INIS)

    This report describes a 5-year-old girl with Langerhans' cell histiocytosis (LCH) of the clivus. To date only five patients, including our patient, have been described with LCH at this site. Our patient differs from those previously reported by her atypical clinical presentation with torticollis, but without a sixth nerve palsy. In addition, she is the first patient to present with concomitant disease elsewhere at the time of diagnosis, i.e. both femoral necks and left proximal humerus. Our patient thus presents unique features and underlines the importance of including LCH in the differential diagnosis of erosive lesions of the clivus. (orig.)

  13. Combined Cutaneous Rosai-Dorfman Disease and Localized Cutaneous Langerhans Cell Histiocytosis Within a Single Subcutaneous Nodule

    Science.gov (United States)

    Litzner, Brandon R.; Subtil, Antonio

    2015-01-01

    Abstract: Rosai-Dorfman disease (RDD) is a reactive multisystem histiocytosis that typically presents with cervical lymphadenopathy and systemic symptoms. Cutaneous involvement occurs in approximately 10% of cases, and 3% of cases are limited to the skin without nodal or other extranodal involvement. Langerhans cell histiocytosis (LCH) is a clonal histiocytosis with a wide spectrum of presentations ranging from isolated skin or bone disease to multisystem involvement. Rare case reports have identified concomitant presentation of RDD and LCH; however, most of these reports have involved LCH and RDD occurring concurrently but at separate sites. We present a rare case of concurrent RDD and LCH presenting within a single skin nodule. The patient did not have any evidence of systemic involvement and has remained stable without additional treatment. We also review the literature on this unusual co-presentation and suggest possible underlying mechanisms. Finally, we recommend baseline laboratory and imaging studies and discuss treatment options based on the available evidence. PMID:26588339

  14. Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

    Directory of Open Access Journals (Sweden)

    Lovrenski Aleksandra

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis. In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.

  15. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma

    Science.gov (United States)

    Ceyran, A. Bahar; Şenol, Serkan; Bayraktar, Barış; Özkanlı, Şeyma; Cinel, Z. Leyla; Aydın, Abdullah

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac arrest with respiratory causes in the early postoperative period. Langerhans cell histiocytosis is a rare primary condition which involves abnormal clonal proliferation of Langerhans cells in various tissues and organs. Thyroid involvement is infrequently seen. Although the etiology is unknown, genetic components may be linked to the disease. It is also associated with a family history of thyroid disease. Papillary thyroid carcinoma is the most common malignant epithelial tumor of the thyroid gland. Langerhans cell histiocytosis presenting with papillary thyroid carcinoma is rare. The privilege of our case is langerhans cell histiocytosis of the thyroid with multiple cervical lymph node involvement accompanying cervical lymph node metastatic thyroid papillary carcinoma. PMID:25349760

  16. Langerhans cell histiocytosis of skin: A clinicopathologic analysis of five cases

    Directory of Open Access Journals (Sweden)

    Punia Rajpal

    2006-01-01

    Full Text Available Background and Aims: Langerhans cell histiocytosis (LCH is a rare proliferative disorder of histiocytes characterized by a proliferation of abnormal and clonal Langerhans cells. We retrospectively studied clinicopathologic features of this disorder in five cases. Methods: Clinical and histopathological findings of five cases of cutaneous LCH were reviewed based on the hospital records. Results: The age of patients ranged from 28 days to 5 years and M: F ratio was 1:1.5. Clinically, the diagnoses suggested were histiocytosis, varicella, transient neonatal pustular melanosis, keloid, sarcoidosis, seborrheic keratosis and LCH. The most common type of skin lesion was a generalized papular lesion. Histologically, all cases showed aggregates of large mononuclear histiocytes (Langerhans cells with reniform, irregular, cleaved nuclei and abundant eosinophilic cytoplasm. There was multi-systemic involvement in two patients and single-system involvement in three patients. Conclusion: Cutaneous lesions may be the sole presenting feature of LCH. Diagnosis is based on demonstration of S-100 positive histiocytes.

  17. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    Directory of Open Access Journals (Sweden)

    B. Routy

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8 vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented.

  18. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    Science.gov (United States)

    Routy, B.; Hoang, J.; Gruber, J.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  19. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation.

    Science.gov (United States)

    Routy, B; Hoang, J; Gruber, J

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. PMID:25789184

  20. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Shi, Yingyan; Qiao, Zhongwei; Gong, Ying; Yang, Haowei; Li, Guoping; Pa, Mier [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China); Xia, Chunmei [Shanghai Medical College of Fudan University, Physiology and Pathophysiology Department, Shanghai (China)

    2014-06-15

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  1. Blood Patch Pleurodesis in the Treatment of Persistant Air Leakage in Langerhans Cell Histiocytosis; A Case Report

    Directory of Open Access Journals (Sweden)

    Ayse Baccioglu

    2013-08-01

    Full Text Available Pulmonary langerhans cell histiocytosis is a rare reactive disorder with unclear pathogenesis. 16-year-old male patient complained about shortness of breath induced with exercise, non-productive cough, and intermittent chest pain for one year. Pulmonary function tests were in restrictive nature. There were multiple air cysts in lung parenchyma smaller than one cm and minimal pneumothorax on the left hemithorax in high resolution computed tomography of thorax. There was no hypoxemia in arterial blood gas analysis, and no pulmonary hypertension in echocardiography. Pulmonary langerhans cell histiocytosis was diagnosed with clinical features and typical radiographic appearance. Existence of multisystem langerhans cell histiocytosis was excluded thorough a detailed history, comprehensive physical examination, and baseline radiographic, blood and urine tests. He was recommended to quit smoking and close follow-up was planned. A tube thoracostomy was performed for left sided total pneumothorax one month later, and %u201Cblood patch%u201D pleurodesis was done due to persistent air leakage with a successful outcome. As far as we know this is the first case report of pulmonary langerhans cell histiocytosis who was treated with autologous blood pleurodesis in the literature.

  2. A rare case of "switch on and off" multi-system Langerhans cell histiocytosis in an adult patient

    Directory of Open Access Journals (Sweden)

    Froudarakis Marios E

    2011-07-01

    Full Text Available Abstract Introduction We report the case of a 24-year-old Greek woman with histologically proven osseous and pulmonary Langerhans cell histiocytosis whose lesions had progressively regressed with a "switch on and off" mode. This is the first report in the literature of this mode of presentation of Langerhans cell histiocytosis. Case presentation The patient had first presented at the age of 20 years with a solitary lesion of the humerus which spontaneously regressed. At that time, no therapy or smoking cessation was indicated. Four years later she presented with bilateral pneumothorax and pulmonary lesions of Langerhans cell histiocytosis. She had pleurodesis for this disease-related complication, and no further systemic treatment was applied, except with regard to smoking cessation. During the follow-up period, her pulmonary lesions regressed without recurrence six years after the initial lung involvement. Conclusion This uncommon case of remission of multi-system Langerhans cell histiocytosis indicates the unpredictable evolution of the disease, raising the question of conservative management in such a patient.

  3. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  4. High-resolution computed tomography findings in pulmonary Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Rodrigues, Rosana Souza [Universidade Federal do Rio de Janeiro (HUCFF/UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho. Unit of Radiology; Capone, Domenico; Ferreira Neto, Armando Leao [Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ (Brazil)

    2011-07-15

    Objective: The present study was aimed at characterizing main lung changes observed in pulmonary Langerhans cell histiocytosis by means of high-resolution computed tomography. Materials and Methods: High-resolution computed tomography findings in eight patients with proven disease diagnosed by open lung biopsy, immunohistochemistry studies and/or extrapulmonary manifestations were retrospectively evaluated. Results: Small rounded, thin-walled cystic lesions were observed in the lung of all the patients. Nodules with predominantly peripheral distribution over the lung parenchyma were observed in 75% of the patients. The lesions were diffusely distributed, predominantly in the upper and middle lung fields in all of the cases, but involvement of costophrenic angles was observed in 25% of the patients. Conclusion: Comparative analysis of high-resolution computed tomography and chest radiography findings demonstrated that thinwalled cysts and small nodules cannot be satisfactorily evaluated by conventional radiography. Because of its capacity to detect and characterize lung cysts and nodules, high-resolution computed tomography increases the probability of diagnosing pulmonary Langerhans cell histiocytosis. (author)

  5. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases

    Energy Technology Data Exchange (ETDEWEB)

    Hindman, B.W.; Thomas, R.D.; Young, Lionel W. [Loma Linda University Medical Center, 11234 Anderson Street, Loma Linda, CA 92354 (United States); Yu, Leisure [Department of Orthopedic Surgery, Loma Linda University Medical Center, Loma Linda, CA 92354 (United States)

    1998-04-01

    Objective. Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. Design and patients. Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. Results. Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial `button` sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. Conclusions. Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis. (orig.) With 7 figs., 10 refs.

  6. High-resolution computed tomography findings in pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: The present study was aimed at characterizing main lung changes observed in pulmonary Langerhans cell histiocytosis by means of high-resolution computed tomography. Materials and Methods: High-resolution computed tomography findings in eight patients with proven disease diagnosed by open lung biopsy, immunohistochemistry studies and/or extrapulmonary manifestations were retrospectively evaluated. Results: Small rounded, thin-walled cystic lesions were observed in the lung of all the patients. Nodules with predominantly peripheral distribution over the lung parenchyma were observed in 75% of the patients. The lesions were diffusely distributed, predominantly in the upper and middle lung fields in all of the cases, but involvement of costophrenic angles was observed in 25% of the patients. Conclusion: Comparative analysis of high-resolution computed tomography and chest radiography findings demonstrated that thinwalled cysts and small nodules cannot be satisfactorily evaluated by conventional radiography. Because of its capacity to detect and characterize lung cysts and nodules, high-resolution computed tomography increases the probability of diagnosing pulmonary Langerhans cell histiocytosis. (author)

  7. Langerhans cell histiocytosis: unusual skeletal manifestations observed in thirty-four cases

    International Nuclear Information System (INIS)

    Objective. Unusual manifestations are occasionally encountered in Langerhans cell histiocytosis and may be a source of confusion. The objective of this study was to determine the frequency of occurrence of the unusual manifestations in our case material. Design and patients. Thirty-four children, average age 4.4 years (range 3 months to 16 years) with 262 skeletal lesions of biopsy-proven Langerhans cell histiocytosis (LCH) were retrospectively reviewed to determine the frequency of occurrence of unusual manifestations defined either as an atypical location of a skeletal lesion or an atypical radiographic appearance of the lesion. Results. Twenty-four unusual lesions were found in this retrospective review. Among these were epiphyseal lesions, transphyseal lesions, extracranial 'button' sequestra, posterior vertebral arch lesions, dural extension of vertebral lesions, and fluid-fluid levels. The finding of fluid-fluid levels has not previously been described in the radiologic literature. Involvement of unusual sites included clavicles and small bones of the hands and feet. Conclusions. Radiographic, computed tomographic, and magnetic resonance imaging of LCH yield a variety of unusual manifestations. Recognition of these varied appearances of LCH may prevent confusion of such appearances with other pathologic processes. When the unusual manifestation occurs as the initial finding of the disease, LCH should be included in the differential diagnosis. (orig.)

  8. Radiotherapy for management of Langerhans' cell histiocytosis. Two case reports and a literature review

    International Nuclear Information System (INIS)

    The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7x1.8 Gy and 7x2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required. (orig.)

  9. Haematopoietic Stem Cell Transplantation for Refractory Langerhans Cell Histiocytosis: Outcome by Intensity of Conditioning

    Science.gov (United States)

    Veys, Paul A.; Nanduri, Vasanta; Baker, K. Scott; He, Wensheng; Bandini, Giuseppe; Biondi, Andrea; Dalissier, Arnaud; Davis, Jeffrey H.; Eames, Gretchen M.; Egeler, R. Maarten; Filipovich, Alexandra H.; Fischer, Alain; Jürgens, Herbert; Krance, Robert; Lanino, Edoardo; Leung, Wing H.; Matthes, Susanne; Michel, Gérard; Orchard, Paul J.; Pieczonka, Anna; Ringdén, Olle; Schlegel, Paul G.; Sirvent, Anne; Vettenranta, Kim; Eapen, Mary

    2015-01-01

    Summary Patients with Langerhans cell histiocytosis (LCH) refractory to conventional chemotherapy have a poor outcome. There are currently two promising treatment strategies for high-risk patients: the first involves the combination of 2-chlorodeoxyadenosine and cytarbine; the other approach is allogeneic haematopoietic stem cell transplantation (HSCT). Here we evaluated 87 patients with high-risk LCH who were transplanted between 1990–2013. Prior to the year 2000, most patients underwent HSCT following myeloablative conditioning (MAC): only 5 of 20 patients (25%) survived with a high rate (55%) of transplant-related mortality (TRM). After the year 2000 an increasing number of patients underwent HSCT with reduced intensity conditioning (RIC): 49/67 (73%) patients survived, however, the improved survival was not overtly achieved by the introduction of RIC regimens with similar 3-year probability of survival after MAC (77%) and RIC transplantation (71%). There was no significant difference in TRM by conditioning regimen intensity but relapse rates were higher after RIC compared to MAC regimens (28% vs. 8%, p=0.02), although most patients relapsing after RIC transplantation could be salvaged with further chemotherapy. HSCT may be a curative approach in 3 out of 4 patients with high risk LCH refractory to chemotherapy: the optimal choice of HSCT conditioning remains uncertain. PMID:25817915

  10. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT)

    Energy Technology Data Exchange (ETDEWEB)

    Turpin, Sophie [CHU Sainte-Justine, Nuclear Medicine, Montreal (Canada); Carret, Anne-Sophie [CHU Sainte-Justine, Hemato-Oncology, Montreal (Canada); Dubois, Josee [CHU Sainte-Justine, Radiology, Montreal (Canada); Buteau, Chantal [CHU Sainte-Justine, Infectious Diseases, Montreal (Canada); Patey, Natalie [CHU Sainte-Justine, Pathology, Montreal (Canada)

    2015-11-15

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis. (orig.)

  11. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT)

    International Nuclear Information System (INIS)

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis. (orig.)

  12. Langerhans cell histiocytosis: A case report of a three year old boy with primary oral manifestations

    Directory of Open Access Journals (Sweden)

    Seraj B

    2006-06-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare disease with formation of granulomatous infiltrations consisting of Langerhans cells, histiocytes, lymphocytes and eosinophilic granulocytes. The ethiopathogenesis of the disease has not been fully clarified yet. It can occur as focal or disseminated form - acute or chronic. Oral manifestations may be the first signs. This article reports a case of a 3 year old boy with LCH suffering from severe dental mobility and foul breathe. Radiographically, the lesions appeared as well-defined radiolucent defects in maxilla and mandible. The microscopic findings consisted of sheet like arrangements of histiocytes with a mixture of eosinophils and other inflammatory cells. Biochemical tests were within normal limits.

  13. Pulmonary Langerhans Cell Histiocytosis in an Adult Diagnosed with Solitary Inguinal Lymphadenopathy.

    Science.gov (United States)

    Luo, Wei; Li, Ya-lun; Jia, Lian; Liu, Chun-tao

    2015-01-01

    We herein report an extremely rare case of pulmonary Langerhans cell histiocytosis with a solitary enlarged inguinal lymph node. A 19-year-old man presented with a non-productive cough lasting for over a five-month period and an enlarged left inguinal lymph node that had persisted for four months. A histopathological study of the lymph node specimens found Langerhans cells coupled with eosinophils. Positive immunohistochemical staining for langerin, Cluster of Differentiation 1a, S100 in the Langerhans cells confirmed the diagnosis, and a mildly impaired ventilation function in addition to multiple peripheral pulmonary cystic lesions were detected. The patient was managed with prednisone (0.5 mg/kg daily), with slow tapering over several months. PMID:26134198

  14. A case of coexisting Warthin tumor and langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction in intraparotid lymph nodes

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    Fredrik Petersson

    2011-04-01

    Full Text Available We present a patient (50-year-old male with coexisting Warthin tumor and involvement of two intraparotid lymph nodes by Langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction. This is the second documented case of this unusual combination of histological changes in nodal Langerhans cell histiocytosis and the first case involving intraparotid lymph nodes occurring together with an ipsilateral Warthin tumor.

  15. A case of coexisting Warthin tumor and langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction in intraparotid lymph nodes

    OpenAIRE

    Fredrik Petersson; Gangaraju Changal Raju; Char Loo Tan

    2011-01-01

    We present a patient (50-year-old male) with coexisting Warthin tumor and involvement of two intraparotid lymph nodes by Langerhans cell histiocytosis associated with necrosis, eosinophilic abscesses and a granulomatous reaction. This is the second documented case of this unusual combination of histological changes in nodal Langerhans cell histiocytosis and the first case involving intraparotid lymph nodes occurring together with an ipsilateral Warthin tumor.

  16. Increased expression of growth factor genes for macrophages and fibroblasts in bronchoalveolar lavage cells of a patient with pulmonary histiocytosis X.

    OpenAIRE

    Barth, J.; Kreipe, H; Radzun, H. J.; Heidorn, K.; Petermann, W; Bewig, B; Parwaresch, M. R.

    1991-01-01

    Pulmonary histiocytosis X is the local manifestation of a systemic disorder of unknown cause characterised by infiltration of Langerhans cell like histiocytes and parenchymal fibrosis. In a male smoker with histologically proved histiocytosis X and functional impairment bronchoalveolar lavage showed an increase in CD-1/OKT-6 antigen positive histiocytes to 8%. Northern blot analysis of RNA from bronchoalveolar lavage cells showed an exaggerated expression of the M-CSF gene and of the c-fms ge...

  17. FNAC of Bacillus- Calmette- Guerin lymphadenitis masquerading as Langerhans cell histiocytosis.

    Directory of Open Access Journals (Sweden)

    Gupta Nalini

    2004-12-01

    Full Text Available Abstract Bacillus Calmette Guerin (BCG lymphadenitis is a well known entity. Disseminated BCG infection usually presents as generalized lymphadenopathy, skin rash and hepatosplenomegaly and at times, can pose a diagnostic challenge to clinicians. There are only a few published studies on the cytological findings of BCG lymphadenitis. In this letter we report the fine needle aspiration cytology (FNAC of BCG lymphadenitis clinically masquerading as Langerhans cell histiocytosis (LCH. FNA smears showed sheets of foamy macrophages and many polymorphs in a dirty necrotic background with many macrophages as well as polymorphs showing negatively stained rod like structures within their cytoplasm. Zeihl Neelson stain revealed that these cells were heavily loaded with acid fast bacilli (AFB. In the index case, AFB were also seen within the cytoplasm of polymorphs, which has not been documented earlier in the literature.

  18. Langerhans cell histiocytosis with multisystem involvement in an infant: A case report

    Science.gov (United States)

    BI, LINTAO; SUN, BUTONG; LU, ZHENXIA; SHI, ZHANGZHEN; WANG, DAN; ZHU, ZHENXING

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells, with a wide range of clinical presentations that vary from a solitary lesion to more severe multifocal or disseminated lesions. The disease can affect any age group; however, the peak incidence rate is in infants aged between 1 and 3 years-old. Diagnosis of LCH should be based on the synthetical analysis of clinical presentations, in addition to features of imaging and histopathology. Although certain cases regress spontaneously, other patients require systemic chemotherapy together with the administration of steroids. The present study reports the case of an infant with LDH with multisystem involvement, including that of the bone, skin, orbit, spleen and lungs. The patient received chemotherapy and obtained rapid improvement in the involved systems. A total of 2.5 years after completion of the therapy, the patient still remains in follow-up and no evidence of active disease has been noted. PMID:26136948

  19. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    Science.gov (United States)

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2014-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  20. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI

    Science.gov (United States)

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis. PMID:26962425

  1. Case of Langerhans Cell Histiocytosis That Mimics Meningioma in CT and MRI.

    Science.gov (United States)

    Zhu, Ming; Yu, Bing-Bing; Zhai, Ji-Liang; Sun, Gang

    2016-03-01

    Langerhans cell histiocytosis (LCH) is a rare disorder histologically characterized by the proliferation of Langerhans cells. Here we present the case of a 13-year-old girl with LCH wherein CT and MRI results led us to an initially incorrect diagnosis of meningioma. The diagnosis was corrected to LCH based on pathology findings. An intracranial mass was found mainly in the dura mater, with thickening of the surrounding dura. It appeared to be growing downward from the calvaria, pressing on underlying brain tissue, and had infiltrated the inner skull, causing a bone defect. The lesion was calcified with the typical dural tail sign. The dural origin of the lesion was verified upon surgical dissection. There are no previous reports in the literature describing LCH of dural origin presenting in young patients with typical dural tail signs and meningioma-like imaging findings. The current case report underscores the need for thorough histological and immunocytochemical examinations in LCH differential diagnosis. PMID:26962425

  2. Langerhans cell histiocytosis of the cervical spine: a single institution experience in four patients.

    Science.gov (United States)

    Tan, Grace; Samson, Ignace; De Wever, Ivo; Goffin, Jan; Demaerel, Philippe; Van Gool, Stefaan W

    2004-03-01

    When Langerhans cell histiocytosis (LCH) occurs at critical sites, such as in the cervical spine, there is a substantial risk for morbidity. Therefore, reports on clinical experiences with those patients remain important. We summarize the history of four patients with unifocal LCH at the cervical spine. All four patients received a biopsy to prove the histopathological diagnosis of LCH by demonstration of CD1a+cells. They were treated with oral prednisolone. All patients recovered completely and kept a normal function of the cervical spine. No reactivation of the disease occurred with an observation time of 3.4-7.3 years. This report contributes to the clinical experience for the treatment of LCH at critical sites. PMID:15076592

  3. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy

    Directory of Open Access Journals (Sweden)

    Adam May

    2015-01-01

    Full Text Available Pulmonary Langerhans cell histiocytosis (PLCH is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.

  4. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.

    Science.gov (United States)

    May, Adam; Kane, Garvan; Yi, Eunhee; Frantz, Robert; Vassallo, Robert

    2015-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH. PMID:26029568

  5. Solitary Langerhans cell histiocytosis of the hard palate: a diagnostic pitfall

    Science.gov (United States)

    Varsha, Dalal; Kaur, Manveen; Chaudhary, Neena; Siraj, Fouzia

    2016-01-01

    Langerhans cell histiocytosis (LCH) is a relatively rare and unique disease characterized by an abnormal proliferation of immature dendritic cells. It is predominantly seen in children with adults showing less than ten times the incidence compared to childhood. The clinical presentation and organ involvement is highly variable. Oral manifestations generally consist of mucosal ulceration associated with lesions of the underlying bone. Lesions limited to the oral mucosa are rare. We present a case of a 45-year-old male who presented with an ulcer on the hard palate showing histopathologic features of LCH. The present case is a reminder of the possibility of occurrence of this unusual entity in the oral cavity. Appropriate use of immunohistochemistry is advocated to avoid diagnostic pitfalls. PMID:27703428

  6. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female

    OpenAIRE

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the u...

  7. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker.

    Science.gov (United States)

    Earlam, K; Souza, C A; Glikstein, R; Gomes, M M; Pakhalé, S

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement. PMID:27445532

  8. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    Directory of Open Access Journals (Sweden)

    K. Earlam

    2016-01-01

    Full Text Available Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

  9. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    Science.gov (United States)

    Earlam, K.; Souza, C. A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement. PMID:27445532

  10. PULMONARY LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS SIMULTANEOUS BILATERAL SPONTANEOUS PNEUMOTHORAX IN A NON-SMOKER PATIENT

    Directory of Open Access Journals (Sweden)

    M. Vaziri

    2008-06-01

    Full Text Available Pulmonary Langerhans Cell Histiocytosis (PLCH is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

  11. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    Science.gov (United States)

    Nabi, Shahzaib; Arshad, Adeel; Jain, Tarun; Virk, Fawad; Gulati, Rohit; Awdish, Rana

    2015-01-01

    A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient's skin biopsy was consistent with Langerhans cell histiocytosis. PMID:26579323

  12. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    Directory of Open Access Journals (Sweden)

    Shahzaib Nabi

    2015-01-01

    Full Text Available A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

  13. Localized Langerhans` cell histiocytosis of bone: treatment and follow-up in children

    Energy Technology Data Exchange (ETDEWEB)

    Libicher, M. [Dept. of Diagnostic Radiology, Univ. Heidelberg (Germany); Roeren, T. [Dept. of Diagnostic Radiology, Univ. Heidelberg (Germany); Troeger, J. [Dept. of Pediatric Radiology, Univ. Heidelberg (Germany)

    1995-11-01

    We report a case of Langerhans` cell histiocytosis (LCH) involving the right scapula in a 4-year-old child. Because of progressive shoulder pain and immobility methylprednisolone was injected directly into the lesion under computed tomography (CT) guidance. Follow-up studies with magnetic resonance imaging (MRI) and ultrasound (US) revealed continuous ossification of the osteolytic lesion with healing after 6 months. Plain films and CT confirmed the healing process. We consider intralesional corticoid instillation a safe method when performed under CT guidance. For initial evaluation and follow-up, MRI and US yielded reliable results in comparison to plain films and CT, thus helping to reduce the radiation dosage in children. (orig.)

  14. Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

    International Nuclear Information System (INIS)

    Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.

  15. The value of bone scintigraphy in the diagnosis and follow-up of Langerhans' cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: To investigate the clinical value of bone scintigraphy in the diagnosis and follow-up of Langerhans' cell histiocytosis (LCH). Methods: Whole-body bone scintigraphy was performed on 23 LCH patients. Results: Nineteen of 23 patients (82.6%) showed positive in their bone scan. Eight cases were with cranial abnormal uptake, clavicle, rib and pelvis involvements were seen and each was of 3 cases, respectively, upper limb, lower limb and spinal abnormal uptakes were seen in 2, 4 and 5 patients, respectively, and 1 child had photon deficient area in the lower part of the sternum. Among 19 positive scintigrams, solitary bone lesion was shown in 9 patients (47.4%). Conclusion: Characters of bone scintigraphy in LCH are useful in the diagnosis and follow-up of this disease. (authors)

  16. Localized Langerhans' cell histiocytosis of bone: treatment and follow-up in children

    International Nuclear Information System (INIS)

    We report a case of Langerhans' cell histiocytosis (LCH) involving the right scapula in a 4-year-old child. Because of progressive shoulder pain and immobility methylprednisolone was injected directly into the lesion under computed tomography (CT) guidance. Follow-up studies with magnetic resonance imaging (MRI) and ultrasound (US) revealed continuous ossification of the osteolytic lesion with healing after 6 months. Plain films and CT confirmed the healing process. We consider intralesional corticoid instillation a safe method when performed under CT guidance. For initial evaluation and follow-up, MRI and US yielded reliable results in comparison to plain films and CT, thus helping to reduce the radiation dosage in children. (orig.)

  17. Brain MR in patients with Langerhans cell histiocytosis: Findings and enhancement with Gd-DTPA

    International Nuclear Information System (INIS)

    The brains of eight patients with Langerhans Cell Histiocytosis (LCH) were studied with magnetic Resonance Imaging (MRI). One scan was normal and a spectrum of abnormalities was seen in the others. Five patients had absence of the posterior pituitary bright spot, and four with evidence of pituitary dysfunction had a lesion in the region of the hypothalamus. An orbital extraconal mass was noted in one child with exopthalmus. This decreased in a follow-up study after Vinblastine therapy. The cerebellum was abnormal in two patients; the area of the dentate nuclei exhibited abnormal signal in one asymptomatic child and the MRI of another boy with a ten year history of progressive cerebellar dysfunction showed cerebral and cerebellar atrophy. Three of the five patients with deficiency of antidiuretic hormone (ADH) were studied both before and after the administration of intravenous gadolinium DTPA. In all three, after administration of the contrast agent, there was enhancement of involved areas. (orig.)

  18. Pulmonary Langerhans Cell Histiocytosis and Diabetes Insipidus in a Young Smoker

    Science.gov (United States)

    Earlam, K.; Souza, C. A.; Glikstein, R.; Gomes, M. M.; Pakhalé, S.

    2016-01-01

    Langerhans cell histiocytosis is characterized by the abnormal nodular proliferation of histiocytes in various organ systems. Pulmonary involvement seen in young adults is nearly always seen in the context of past or current cigarette smoking. Although it tends to be a single-system disease, extrapulmonary manifestations involving the skin, bone, and hypothalamic-pituitary-axis are possible. High resolution CT (HRCT) of the thorax findings includes centrilobular nodules and cysts that are bizarre in shape, variable in size, and thin-walled. Often the diagnosis can be made based on the appropriate clinical presentation and typical imaging findings. Treatment includes smoking cessation and the potential use of glucocorticoids or cytotoxic agents depending on the severity of disease and multisystem involvement.

  19. Rare interstitial lung disease: Pulmonary Langerhans Cell Histiocytosis in a young non smoking Indian female.

    Science.gov (United States)

    Fernandes, Lalita; Vadala, Rohit; Mesquita, Anthony Menezes; Vaideeswar, Pradeep

    2015-01-01

    Adult Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare interstitial lung disease which occurs almost exclusively in smokers. A marked male predominance was initially reported, but recent studies show both men and women are equally affected due to the increasing smoking habits in women. The natural history is variable with 25% of patients having asymptomatic disease while 10-20% progress rapidly to respiratory insufficiency and death. The diagnosis is not easily recognized by clinicians or pathologists. Awareness of the clinical presentation and classical HRCT findings helps in early diagnosis and management of this disease. We report a rare case of severe PLCH in a young non smoking female with a short history who progressed rapidly to respiratory failure and died. PMID:25857566

  20. A case of pulmonary Langerhans cell histiocytosis in a young woman with coeliac disease.

    Science.gov (United States)

    Mastruzzo, Claudio; Vancheri, Carlo; Li Mura, Dario; Poletti, Venerino; Failla, Marco; Crimi, Nunzio

    2007-06-01

    Pulmonary Langerhans cells histiocytosis (pulmonary LCH) is an idiopathic unusual lung disease and its association with other systemic diseases has been rarely observed. Here, we describe a young non-smoking woman with concomitant pulmonary LCH and coeliac disease that, despite therapy, suddenly deteriorated. To the best of the authors' knowledge, this is the first report in the medical literature describing an association of coeliac disease with pulmonary LCH. Considering the concomitant occurrence of both diseases in our patient and the severe course of pulmonary LCH observed, we hypothesise that coeliac disease and pulmonary LCH might be related by a common disturbance in immunity and the onset and/or the course of pulmonary LCH could be influenced or markedly worsened by the presence of coeliac disease. PMID:17629812

  1. Relapsing nodular lesions in the course of adult pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Tazi, A; Montcelly, L; Bergeron, A; Valeyre, D; Battesti, J P; Hance, A J

    1998-06-01

    In most patients with pulmonary Langerhans cell histiocytosis (LCH), clinical and radiological abnormalities initially either stabilize or regress, often without treatment. Little information is available, however, concerning the subsequent evolution of disease in patients who initially follow a benign course. We describe four patients with biopsy-confirmed pulmonary LCH whose initial course was characterized by regression of parenchymal nodular lesions, but who subsequently developed one or more episodes of active disease 7 mo to 7.5 yr after their initial presentation. In each case, the subsequent episodes of active disease were characterized by the reappearance or marked increase in nodular radiographic abnormalities, whose presence was confirmed by high-resolution computed tomography (HRCT). Thus, initial regression of nodular lesions in pulmonary LCH does not preclude the reappearance of one or more episodes of active disease, and may have important consequences on the long-term prognosis of these patients. PMID:9620939

  2. Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

    Energy Technology Data Exchange (ETDEWEB)

    Bano, Shahina, E-mail: dr_shahinaindia@yahoo.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Chaudhary, Vikas, E-mail: dr_vikaschaudhary@yahoo.com [Department of Radiodiagnosis, Employees’ State Insurance Corporation (ESIC) Model Hospital, Gurgaon 122001, Haryana (India); Narula, Mahender Kaur, E-mail: narulamk@gmail.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Anand, Rama, E-mail: rama_home@yahoo.co.in [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Venkatesan, Bhuvaneswari, E-mail: venkatesanbhuvana@yahoo.com [Department of Radiodiagnosis, Lady Hardinge Medical College and Associated Hospitals, New Delhi 110001 (India); Mandal, Shramana, E-mail: shramana@hotmail.co.in [Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002 (India); Majumdar, Kaushik, E-mail: drkaushik.m@gmail.com [Department of Pathology, Govind Ballabh Pant Hospital, New Delhi 110002 (India)

    2014-01-15

    Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.

  3. Results of Treatment with 2-Chlorodeoxyadenosine (2-CDA in Multiple Reactivations or Refractory Langerhans Cell Histiocytosis

    Directory of Open Access Journals (Sweden)

    Ghasem Miri-Aliabad

    2014-01-01

    Full Text Available Background: Langerhans cell histiocytosis (LCH is the most common type of histiocytosis and characterized by abnormal proliferation and excess accumulation of inflammatory and langerhans cells at various tissue sites. Clinical manifestations are variable, ranging from spontaneously regressing single bone lesion to multisystem disease, life-threatening and refractory to treatment. Conventional chemotherapy has been shown to be effective in treatment of majority of patients with LCH. However, treatment of refractory disease or multiple reactivations is difficult. The aim of this study is to assess the efficacy of 2-CDA in relapsed or refractory LCH. Materials and methods: Four patients with relapsed or refractory LCH that were treated with 2-chlorodeoxyadenosin (2-CDA enrolled in this study. All patients had received at least one prior chemotherapy regimen. The dose and schedule of 2-CDA was 6 mg/m²/day for 5 days every 3-4 weeks. Results: Median age at the time of treatment with 2-CDA was 9.7 years. Three patients had multisystem disease and one had multifocal bone lesions. All patients had multifocal bone lesions. None of them had risk organ involvement. Mean course of treatment with 2-CDA was 9.5. Radiologic evaluations revealed complete resolution of bone lesions in two (50% patients. In one (25% patient lesions regressed (partial response and in another (25% the disease remained stable. Drug related side effects were minimal. At the present time all patients are alive. Conclusion: Our study demonstrates that 2-CDA as a single agent is efficacious in treatment of multiple reactivations or refractory LCH and well-tolerated in children.

  4. Radiotherapy in langerhans cell histiocytosis - a rare indication in a rare disease

    International Nuclear Information System (INIS)

    Langerhans Cell Histiocytosis (LCH) represents a rare benign disorder, previously designated as “Histiocytosis X”, “Type II Histiocytosis” or “Langerhans Cell Granulomatosis”. Clinical presentation includes osteolysis, ulcerations of skin and soft tissues but also involvement of the CNS is described. Because treatment concepts are not well defined the German Cooperative Group on Radiotherapy for Benign Diseases performed a retrospective analysis. Eight closely cooperating centres collected patients’ data of the past 45 years. As study endpoints disease free survival, recurrent disease, death and therapy related side effects were defined. A total of 80 patients with histologically proven LCH were irradiated within the past 45 years. According to the LCH classification of Greenberger et al. 37 patients had stage Ia, 21 patients stage Ib, 13 patients stage II and 9 patients stage IIIb and the median age was 29 years. The median Follow up was 54 months (range 9–134 months). A total of 39 patients had a surgical intervention and 23 patients a chemotherapy regimen. Radiation treatment was carried out with a median total dose of 15 Gy (range 3–50.4 Gy). The median single fraction was 2 Gy (range 1.8-3 Gy). Overall, 77% patients achieved a complete remission and 12.5% achieved a partial remission. The long-term control rate reached 80%. Within an actuarial overall 5-year survival of 90% no radiogenic side and late effects ≥EORTC/RTOG II° were observed. In the present study a large collective of irradiated patients was analysed. Radiotherapy (RT) is a very effective and safe treatment option and even low RT doses show sufficient local control

  5. Clinical characteristics and recent progress in the diagnosis and treatment for langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Objective: To discuss the clinical characteristics, reactivation, diagnosis, treatment and prognosis of Langerhans cell histiocytosis (LCH) based on the new progress. Methods: Fifty-five such patients treated from Jan. 1974 to May 1998 were retrospectively analyzed. The patients aged from 2 to 67 years at the time of diagnosis, with a median age of 31 years, male 47, female 8. Forty patients had single system disease and the other 15 with multisystem disease. Partial or total excision and/or local radiotherapy were the main treatments. The involved irradiation field was given, with a median dose of 30 Gy. Results: The soft tissue of head and neck was the most frequently involved (63.6%), followed by the bone (23.6%). Patients ≤15 years had more bony involvement (66.7%) compared to those aged > 15 years (11.6%) ( P 15 years were 58.3% and 74.4%, respectively, (P=0.830); whereas it was 75.0% for single system disease and 60.0% for multisystem disease (P=0.130). The total reactivation for all patients was 43.6%. Two thirds of these patients developed recurrence within 5 years after treatment with 75% developing new foci. At the time of the last follow-up, 74.5% of the patients have survived without disease, while 27.6% of survivors still had active disease. Conclusions: Langerhans cell histiocytosis, not being confined to children, involves the head and neck area most commonly. The frequency of bony invasion is associated with age. Reactivation is very frequent. Patients without risk organs involvement usually carry a favorable prognosis. (authors)

  6. Angioimmunoblastic T Cell Lymphoma Mimicking Chronic Urticaria

    Directory of Open Access Journals (Sweden)

    Mohleen Kang

    2016-01-01

    Full Text Available Angioimmunoblastic T cell lymphoma (AITL is a rare but distinct type of T cell lymphoma with an aggressive course and high mortality. Most patients are diagnosed late in the disease and usually present with generalized lymphadenopathy. A minority have skin lesions at the time of diagnosis, more commonly in the form of nonspecific maculopapular rash with or without pruritus. We report a rare case of AITL presenting with chronic, recurrent angioedema and urticaria-like lesions and no palpable peripheral adenopathy. Primary Care physicians, dermatologists, and allergists must maintain a high index of suspicion for cutaneous manifestations of lymphoma, especially if the skin lesions are refractory to standard treatment. Timely diagnosis is essential to improve survival.

  7. Dual anaplastic large cell lymphoma mimicking meningioma: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Keun Ho; Kim, Ki Hwan; Lee, Ghi Jai; Lee, Hye Kyung; Shim, Jae Chan; Lee, Kyoung Eun; Suh, Jung Ho [Seoul Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Lee, Chae Heuck [Dept. of Neurosurgery, Ilsan Paik Hospital, Inje University College of Medicine, Goyang (Korea, Republic of)

    2014-01-01

    Anaplastic large cell lymphoma (ALCL) is a rare T cell lymphoma composed of CD30-positive lymphoid cells. Most ALCLs present as nodal disease, with skin, bone, soft tissue, lung, and liver as common extranodal sites. ALCL rarely occurs in the central nervous system and is even more infrequent in the dura of the brain. We report a case of dural-based ALCL secondary to systemic disease in a 17-year-old male that mimicked meningioma on magnetic resonance imaging and angiography.

  8. Granuloma inguinale mimicking as squamous cell carcinoma of penis

    Directory of Open Access Journals (Sweden)

    Abhishek Pilani

    2014-01-01

    Full Text Available Granuloma inguinale (GI is an acquired chronic, slowly progressive, mildly contagious disease of venereal origin, characterized by granulomatous ulceration of the genitalia and neighboring sites, with little or no tendency to spontaneous healing caused by Klebsiella (Calymmatobacterium granulomatis. A 55-year-old male presented with fissured, foul smelling, fungating growth over prepuce with phimosis mimicking squamous cell carcinoma (SCC without lymphadenopathy. It started with painless papulonodular showed pseudoepitheliomatous hyperplasia, infiltration in dermis, acanthosis and vacuolated macrophages suggestive of GI and not showing any histopathological features of SCC. Patient was successfully treated by giving cotrimoxazole twice a day for 21 days. Here, we presented a case of GI mimicking SCC of penis, which was diagnosed on basis of histopathology and treated with excision followed by medical therapy with cotrimoxazole.

  9. Lyme carditis mimicking giant cell arteritis

    Directory of Open Access Journals (Sweden)

    Krati Chauhan

    2015-10-01

    Full Text Available Presenting an interesting case of a patient who complained of myalgias, fatigue, headache, jaw claudication and scalp tenderness. Patient’s physical examination was unremarkable. Laboratory findings showed elevated erythrocyte sedimentation rate and C-reactive protein, bilateral temporal artery biopsy results were negative and first degree atrioventricular block was seen on electrocardiogram. Serology for Borrelia burgdorferi was positive; patient was diagnosed with Lyme carditis and treated with doxycycline. Lyme is a tick-borne, multi-system disease and occasionally its presentation may mimic giant cell arteritis. On follow-up there was complete resolution of symptoms and electrocardiogram findings.

  10. Langerhans Cell Histiocytosis in Childhood: Review, Symptoms in the Oral Cavity, Differential Diagnosis and Report of One Case

    Directory of Open Access Journals (Sweden)

    Mohammad Shooriabi

    2016-08-01

    Full Text Available Background Langerhans cell histiocytosis (LCH is a rare disease in which monoclonal migration and proliferation of specific dendritic cells is seen. The disease primarily affects the bones and skin, but there is a possibility that involves other organs or appears as a multi-systemic disease. Case Report In oral examination of a nine-month girl, two deep wounds with a yellow membrane with approximate size of 1 × 1 cm on both sides of mandibular alveolar ridge were seen. The edges of the wounds were swollen and proliferated and redder than the surrounding mucosa. At the touch the edges of the wound were not indurated. The wound were created from the third-month and the size of wounds had become slightly larger within 6 months. According to the chronic wound and being non-responsive to various systemic and local treatments, incisional biopsy was taken from the wounds. Langerhans cell histiocytosis was confirmedhistologically and immune histochemically. Conclusion Mouth ulcers may be the only symptoms of Langerhans cell histiocytosis. Therefore, the role of dentist could be important in diagnosis of this disease.

  11. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child

    Science.gov (United States)

    Srivastava, Vinay Kumar; Bansal, Rajesh; Gupta, Vineeta; Bansal, Manish; Patne, Shashikant

    2014-01-01

    ABSTRACT% Langerhans cell histiocytosis (LCH), previously known as histio-cytosis X, is a rare idiopathic disorder of reticulo-endothelial system with abnormal proliferation of bone marrow derived Langerhans cells along with a variable number of leukocytes, such as eosinophils, neutrophils, lymphocytes and plasma cells. Three years old male child presented with multifocal osteolytic lesions and papulosquamous skin lesions. Clinical and radio-graphic features, such as severe alveolar bone loss, mobility of teeth, precocious eruption of teeth, foating appearance of teeth in orthopantomogram (OPG), osteolytic lesion in skull and cutaneous lesions were highly suggestive of LCH disease. Skin biopsy confirmed a diagnosis of LCH. Induction chemotherapy with oral prednisolone and intravenous vinblastine was started. Child responded well to chemotherapy. The clinical significance of the presented case is to diagnose the case of LCH on the basis of the manifestation of severe periodontal disease as this can be first or only manifestation of LCH. A dentist plays a major role in the multidisciplinary treatment of LCH through routine examination and periodic follow-up. How to cite this article: Bansal M, Srivastava VK, Bansal R, Gupta V, Bansal M, Patne S. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child. Int J Clin Pediatr Dent 2014;7(3):217-219. PMID:25709306

  12. Diagnosis of Langerhans Cell Histiocytosis on Fine Needle Aspiration Cytology: A Case Report and Review of the Cytology Literature

    Directory of Open Access Journals (Sweden)

    Neeta Kumar

    2011-01-01

    Full Text Available A case of multifocal Langerhans cell histiocytosis in a two-year-old child is presented where fine needle aspiration was helpful in achieving a rapid and accurate diagnosis in an appropriate clinical and radiological setting. This can avoid unnecessary biopsy and guide the management especially where access to histopathology is limited. The highly characteristic common and rare cytological features are highlighted with focus on differential diagnoses and causes of pitfalls.

  13. Diagnosis and Intralesional Corticotherapy in Oral Ulcers Occurring as the Sole Manifestation of Langerhans Cell Histiocytosis. A Case Report

    Science.gov (United States)

    Gambirazi, Liane; Libório, Tatiana; Nunes, Fábio; Sugaya, Norberto; Migliari, Dante

    2016-01-01

    This article reports a case of oral mucosa lesions as the sole manifestation in Langerhans cell histiocytosis (LCH). This is a very uncommon manifestation of LCH since this disease preferably affects the bones with frequent involvement of the jaws. LCH may also involve other organs, particularly the lungs, liver, lymph nodes, and skin. The highlights of this report are the differential diagnosis, immunohistochemical analysis and, mostly, the therapeutic approach. PMID:27398106

  14. Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

    Science.gov (United States)

    Udaka, Toru; Susa, Michiro; Kikuta, Kazutaka; Nishimoto, Kazumasa; Horiuchi, Keisuke; Sasaki, Aya; Kameyama, Kaori; Nakamura, Masaya; Matsumoto, Morio; Chiba, Kazuhiro; Morioka, Hideo

    2015-01-01

    Langerhans cell histiocytosis (LCH) usually occurs in children under the age of 10 years with a predilection for the skull, spine, rib and humerus. Solitary LCH occurring in an adult clavicle is uncommon with limited reports to date. The lesion in our patient was curetted with the intent to make a diagnosis, which subsequently lead to the remission of the symptom and the disease. At the final follow-up after 1 year, no local recurrence or metastasis is observed. PMID:26600774

  15. Unifocal Granuloma of Femur due to Langerhans' Cell Histiocytosis: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Harpreet Singh

    2010-01-01

    Full Text Available The radiological diagnosis of osteolytic lesions of the long bones in pediatric population constitutes a challenge when the case history and clinical data are uncharacteristic. We believe that the description of few clinically and histologically proven cases to verify the existence of radiological signs useful for diagnosis may be of interest. Here, we describe a case of Langerhans' cell histiocytosis (LCH presenting as unifocal eosinophilic granuloma of femur along with a brief review of the literature.

  16. Measuring diffuse metabolic activity on FDG-PET/CT: new method for evaluating Langerhans cell histiocytosis activity in pulmonary parenchyma

    OpenAIRE

    Szturz Petr; Řehák Zdeněk; Koukalová Renata; Adam Zdeněk; Krejčí Marta; Pour Luděk; Zahradová Lenka; Vaníček Jiří; Nebeský Tomáš; Hájek Roman; Mayer Jiří

    2012-01-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare cause of interstitial lung disease characterized by formation of nodules in the active phase of the disease that evolve into nonactive cystic lesions later on. To evaluate PLCH activity in patients, we developed a new method for measuring diffuse metabolic activity on fluorine-18-fluorodeoxyglucose positron emission tomography/ computed tomography (FDG-PET/CT) using a lung-to-liver activity ratio. Material and Methods: We retrospectivel...

  17. Intraluminal fibrosis and elastic fiber degradation lead to lung remodeling in pulmonary Langerhans cell granulomatosis (histiocytosis X).

    OpenAIRE

    Y. Fukuda(Miyagi University of Education); Basset, F.; Soler, P; Ferrans, V J; Masugi, Y.; Crystal, R G

    1990-01-01

    To evaluate the morphogenesis of lung remodeling in pulmonary Langerhans cell granulomatosis (LCG; previously called histiocytosis X or eosinophilic granuloma), lung tissues obtained by open biopsy from 62 patients with pulmonary LCG were studied by light and electron microscopy. Tissues from 20 patients were also studied by immunohistochemical methods for the detection of fibronectin, elastin, and S-100 protein, and samples from six patients were studied using OKT6 monoclonal antibody. In ea...

  18. Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis

    OpenAIRE

    Abdul Aziz, Dayang Anita; Abdul Rahman, Nur Afdzillah; Tang, Swee Fong; Abdul Latif, Hasniah; Mohd Zaki, Faizah; Mohd Annuar, Zulfiqar; Alias, Hamidah; Abdul Latiff, Zarina

    2011-01-01

    Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleuro...

  19. Blood Patch Pleurodesis in the Treatment of Persistant Air Leakage in Langerhans Cell Histiocytosis; A Case Report

    OpenAIRE

    Ayse Baccioglu

    2013-01-01

    Pulmonary langerhans cell histiocytosis is a rare reactive disorder with unclear pathogenesis. 16-year-old male patient complained about shortness of breath induced with exercise, non-productive cough, and intermittent chest pain for one year. Pulmonary function tests were in restrictive nature. There were multiple air cysts in lung parenchyma smaller than one cm and minimal pneumothorax on the left hemithorax in high resolution computed tomography of thorax. There was no hypoxemia in arteria...

  20. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    OpenAIRE

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae; Kim, Sang-Ha

    2015-01-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipi...

  1. Langerhans Cell Histiocytosis of the Thyroid with Multiple Cervical Lymph Node Involvement Accompanying Metastatic Thyroid Papillary Carcinoma

    OpenAIRE

    A. Bahar Ceyran; Serkan Şenol; Barış Bayraktar; Şeyma Özkanlı; Z. Leyla Cinel; Abdullah Aydın

    2014-01-01

    A 37-year-old male case was admitted with goiter. Ultrasonography of thyroid showed a 5 cm cystic nodule in the left lobe with a 1.5 cm solid component. Fine needle aspiration biopsy revealed atypia of undetermined significance or follicular lesion. The patient was operated on. The pathological diagnosis was reported as papillary thyroid carcinoma. The immunohistochemical examination showed multiple foci of Langerhans cell histiocytosis involving both lobes. The patient died due to cardiac ar...

  2. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report

    Science.gov (United States)

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae

    2015-01-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  3. Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report.

    Science.gov (United States)

    Choi, Yeun Seoung; Lim, Jung Soo; Kwon, Woocheol; Jung, Soon-Hee; Park, Il Hwan; Lee, Myoung Kyu; Lee, Won Yeon; Yong, Suk Joong; Lee, Seok Jeong; Jung, Ye-Ryung; Choi, Jiwon; Choi, Ji Sun; Jeong, Joon Taek; Yoo, Jin Sae; Kim, Sang-Ha

    2015-10-01

    Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus. PMID:26508947

  4. A Case of Pulmonary Langerhans' Cell Histiocytosis Mimicking Hematogenous Pulmonary Metastases

    OpenAIRE

    Chon, Suyeon; Kyung, Sun Young; Lee, Sang Pyo; Park, Jeong-Woong; Jeong, Sung Hwan; Choi, Soo Jin; Ha, Seung Yeon

    2009-01-01

    A 31-year-old man presented with a dry cough and exertional dyspnea. The chest X-ray showed multiple nodular opacities throughout the entire lung field. Chest computed tomography (CT) revealed variable-sized nodules with a peribronchiolar or centrilobular distribution, some of which revealed thick-walled cavitary change. Based on the chest CT findings, it was initially assumed that metastatic lung nodules with hematogenous spread were present; therefore, we performed an open lung biopsy. On m...

  5. Mesothelial cell inclusions mimicking adenocarcinoma in cervical lymph nodes in association with chylous effusion

    Directory of Open Access Journals (Sweden)

    Goyal Manu

    2010-01-01

    Full Text Available Mesothelial cell inclusions in lymph nodes are of rare occurrence and can be mistaken as metastatic adenocarcinomas, mesothelioma or sinus histiocytosis. These are usually found in mediastinal and abdominal lymph nodes and are associated with effusions. We report a case of benign mesothelial cell inclusions in cervical lymph nodes, which was associated with chylous effusion, and immunohistochemistry revealed unusual weak cytoplasmic epithelial membrane antigen positivity in the cells.

  6. A rare case of langerhans cell histiocytosis of the gastrointestinal tract

    Institute of Scientific and Technical Information of China (English)

    Uday Shankar; Monika Prasad; Om P Chaurasia

    2012-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-year-old woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD-1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increasing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.

  7. Langerhans cell histiocytosis in children diagnosed by fine-needle aspiration

    Science.gov (United States)

    Handa, Uma; Kundu, Reetu; Punia, Rajpal Singh; Mohan, Harsh

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare intricate pediatric neoplasm with varied clinical manifestations and multiple treatment modalities. Aim: To study the cytological features of LCH and the differential diagnoses on fine-needle aspiration (FNA). Materials and Methods: FNA was performed using a 23-gauge needle fitted to a 10 mL syringe mounted on syringe holder. LCH was diagnosed on FNA smears in seven cases confined to the head and neck region, which included three cases of lymphadenopathy, three cases of scalp swelling, and one case of orbital swelling. Results: The age of the patients ranged from 25 days to 11 years and male-to-female ratio was 1:1.3. Clinically, the diagnoses suggested were tuberculosis, inflammatory lesion, abscess, and malignancy. The cytologic findings included high cellularity, isolated Langerhans cells (LCs) with prominent nuclear indentation, grooves and abundant vacuolated cytoplasm, multinucleated giant cells, eosinophils, and lymphocytes. Areas of necrosis were noted in one case. Histopathology, along with positive S-100 immunohistochemistry, confirmed the diagnosis of LCH. Conclusions: LCH is a rare disease occurring predominantly in children and can be diagnosed with ease on FNA cytology by the presence of characteristic Langerhans cells. The S-100 positivity aids in suggesting a diagnosis of LCH. PMID:26811572

  8. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass

    Science.gov (United States)

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung

    2016-01-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  9. A Case of Langerhans Cell Histiocytosis Manifested as a Suprasellar Mass.

    Science.gov (United States)

    Yoon, Ju Young; Park, Byung-Kiu; Yoo, Heon; Lee, Sang Hyun; Hong, Eun Kyung; Park, Weon Seo; Kwon, Young Joo; Yoon, Jong Hyung; Park, Hyeon Jin

    2016-04-01

    Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis. PMID:27195259

  10. [Possibilities and limits of paraffin-embedded cell markers in diagnosis of primary cutaneous histiocytosis].

    Science.gov (United States)

    Fartasch, M; Goerdt, S; Hornstein, O P

    1995-03-01

    To date, the rare primary histiocytoses of the skin are diagnosed definitively on the basis of the clinical symptoms, H&E-stained sections, and demonstration of CD1 positivity in frozen sections and of Birbeck granules on electron microscopy. The improvement and analysis of antibodies with the ability to react in paraffin tissue allow retrospective evaluation and classification of these disorders. The antibodies for S-100-protein, peanut agglutinin (PNA) and PCNA (proliferating cell nuclear antigen) have been advocated for differentiation of the specific cells of Langerhans cell histiocytosis (LCH) from other histiocytic cell systems. To date the non-Langerhans cell histiocytoses (non-LCH) have no common ultrastructural and immunohistochemical characteristics. The infiltrate is made up of multiple cell populations, which are of significance for the cellular pathobiology (subtypes of monocytes/macrophages and dendritic cells). The number and distribution of the different monocyte/macrophages and dendritic cells and their ability to react with immunohistochemical markers in paraffin tissue can be completely different in different clinical entities. The antibodies against factor XIIIa (shown on xanthoma disseminatum) and the monoclonal antibody Ki-M1P (shown on juvenile xanthogranuloma) seem to be valuable in discrimination between LCH and non-LCH. Both markers show a positive staining pattern with the characteristic large macrophages. In juvenile xanthogranuloma, the foam cells and giant cells express Ki-M1P, KP1 and anti-cathepsin B. Other monocyte/macrophage markers with the ability to react in paraffin tissue, such as Mac387, lysozyme, alpha 1-antitrypsin and Leu-M1 (Anti-CD 15), in contrast, did not show a typical staining pattern with the characteristic large macrophages dominating the histological picture.

  11. Engineered nanoparticles mimicking cell membranes for toxin neutralization.

    Science.gov (United States)

    Fang, Ronnie H; Luk, Brian T; Hu, Che-Ming J; Zhang, Liangfang

    2015-08-01

    Protein toxins secreted from pathogenic bacteria and venomous animals rely on multiple mechanisms to overcome the cell membrane barrier to inflict their virulence effect. A promising therapeutic concept toward developing a broadly applicable anti-toxin platform is to administer cell membrane mimics as decoys to sequester these virulence factors. As such, lipid membrane-based nanoparticulates are an ideal candidate given their structural similarity to cellular membranes. This article reviews the virulence mechanisms employed by toxins at the cell membrane interface and highlights the application of cell-membrane mimicking nanoparticles as toxin decoys for systemic detoxification. In addition, the implication of particle/toxin nanocomplexes in the development of toxoid vaccines is discussed. PMID:25868452

  12. Dentinogenic Ghost Cell Tumor of the Peripheral Variant Mimicking Epulis

    Directory of Open Access Journals (Sweden)

    Uddipan Kumar

    2010-01-01

    Full Text Available Dentinogenic ghost cell tumor (DGCT is an uncommon locally invasive odontogenic tumor regarded by many as a variant of calcifying odontogenic cyst. The peripheral variant of this clinical rarity appears as a well-circumscribed mass mimicking a nonspecific gingival enlargement. Microscopic appearance of odontogenic epithelium admixed with focal areas of dentinoid formation and sheets of ghost cells giving the definitive diagnosis of dentinogenic ghost cell tumor imply that microscopic examination is compulsory for any gingival mass. Van Gieson histochemical stain further confirmed the nature of dentinoid-like material. A complete workup of a case of peripheral dentinogenic ghost cell tumor is presented in this paper and the current concept as well as the appraisal of literature is presented.

  13. Button sequestrum in a case of localized Langerhans' cell histiocytosis of the ilium: case report

    Energy Technology Data Exchange (ETDEWEB)

    Tordeur, M. [Pitie-Salpetriere Teaching Hospital, Radiology Dept., Paris (France); Wybier, M. [Lariboisiere Teaching Hospital, Paris (France); Laporte, J.L. [Pitie-Salpetriere Teaching Hospital, Pathology Dept., Paris (France); Grenier, P. [Pitie-Salpetriere Teaching Hospital, Radiology Dept., Paris (France); Laredo, J.D. [Lariboisiere Teaching Hospital, Paris (France)

    2000-04-15

    Langerhans' cell histiocytosis (LCH) is characterized by a proliferation of cells exhibiting the same immunohistochemical and ultra-structural characteristics as Langerhans' cells of the epidermis. Eosinophilic granuloma, chronic polyostotic disease (Hand-Schuller-Christian disease) and multisystemic disease (Letterer-Siwe disease) all belong to the spectrum LCH. Osseous lesions are the most common findings. the radiological appearance of skeletal LCH depends on the site and on disease activity. Button sequestrum has been described as uncommon in LCH of the skull and exceedingly rare in LCH at other sites. We report a case of localized LCH of the ilium with a button sequestrum. (author)

  14. Langerhans cell histiocytosis: literature review and descriptive analysis of oral manifestations.

    Science.gov (United States)

    Madrigal-Martínez-Pereda, Cristina; Guerrero-Rodríguez, Vanesa; Guisado-Moya, Blanca; Meniz-García, Cristina

    2009-05-01

    Langerhans cell histiocytosis (LCH) is a rare disease, of unknown pathogenesis, characterized by intense and abnormal proliferation of bone marrow-derived histiocytes (Langerhans cells). It can present both local and systemic manifestations involving bone, skin and mucosal tissue, and internal organs. Three basic clinical forms develop: Letterer-Siwe disease (subacute or acute disseminated form), Hand-Schüller-Christian disease (disseminated chronic form) and eosinophilic granuloma (localized chronic form). LCH may manifest orally with single or multiple lesions of the alveolar or basal bone, ulcerated mucosal lesions accompanied by adenopathies and/or periodontal lesions, presenting gingival inflammation, bleeding, recession, necrosis, odontalgia, dental hypermobility and premature loss of teeth. The principal differential diagnoses include advanced periodontal disease or a periapical process of dental or periodontal origin. The odontologist plays a vital role in the diagnosis and multidisciplinary treatment of such patients, by performing routine examinations for periodic follow-up of the disease and its possible oral manifestations, bearing in mind that these may be the first or only signs of LCH.

  15. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E. Michel [University of Miami, Pediatric Radiology Section, Department of Radiology, Miami, FL (United States); Saigal, Gaurav [McGill University, Department of Medical Imaging, Quebec (Canada); Rodriguez, Maria M. [University of Miami, Department of Pathology, Miami, FL (United States); Podda, Antonello [University of Miami, Division of Pediatric Hematology/Oncology, Miami, FL (United States)

    2005-02-01

    Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a ''button sequestrum.'' Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement. (orig.)

  16. A solitary pulmonary ground-glass nodule in adult systemic langerhans’ cell histiocytosis

    Science.gov (United States)

    Zhou, Ying; Li, Yunyuan; Fan, Li; Liu, Shiyuan

    2015-01-01

    During a thoracic computed tomography (CT) scan, a 36-year-old male was diagnosed with a solitary oval pulmonary mixed ground-glass nodule in the right upper lobe of the lung. The edge of the nodule was well-defined, and its largest axial size was approximately 1.1×0.9 cm2. This nodule was slightly lobulated, but not obviously speculated. Solid components, micro-cystic lucency shadow, small high-density rings and tiny vascular branches were all visible in the nodule. During hospitalization, a technetium 99 m methylene diphosphonate (Tc-99 m MDP) bone scan was performed, which showed a skeletal foci with abnormal uptake in the left iliac. A pulmonary lobectomy of the right upper lobe of the lung by video-assisted thoracoscopy was performed. In post-operative pathological photomicrographs, proliferative Langerhans’ cells, eosinophils and lymphocytes were found. Immunohistochemistry showed that the expression of S-100 protein, CD1a, and CD68 antigen all stained positive. Since Langerhans’ cell histiocytosis (LCH) that is also associated with isolated mixed ground-glass nodules is relatively rare, such a multi-systemic LCH case as identified herein, is reported. PMID:26722574

  17. Intracranial manifestations of Langerhans cell histiocytosis. MR imaging; Intrakranielle Manifestationen der Langerhanszell-Histiozytose. Kernspintomographische Befunde

    Energy Technology Data Exchange (ETDEWEB)

    Kujat, C. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Inst. fuer Neuroradiologie; Hermes, M. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Inst. fuer Neuroradiologie; Heide, G. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Neurologische Klinik; Baeumler, S. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Klinik fuer Kinder- und Jugenmedizin; Feiden, W. [Universitaetskliniken des Saarlandes, Homburg/Saar (Germany). Abt. fuer Neuropathologie

    1994-11-01

    We report on 3 patients with intracranial manifestations on Langerhans cell histiocytosis (LH). The results are correlated with histological, clinical and radiological reports on some 70 patients described in the literature as suffering from intracranial LH. Two different morphological pictures can be differentiated. First, typical infiltrates can be seen by microscopy; these can be located in every part of the brain, but are seen mainly in the region of te hypothalamus. These infiltrations are shown as space-occupying lesions with Gd-DTPA enhancement. The other manifestation is a demyelinized lesion with a sparse infiltration of Langerhans cells. These lesions, which are located mainly in the region of the nucleus dentatus of the cerebellum and the brain stem, show increased signal intensity in the T2 sequence and no Gd-DTPA enhancement on MRI. (orig.) [Deutsch] Es werden die Befunde von 3 Patienten mit intrakranieller Manifestation einer Langerhanszell-Histiozytose (LH) dargestellt. Die Korrelation mit den histologischen, radiologischen und/oder klinischen Befunden bei etwa 70 in der Literatur erfassten Patienten mit intrakranieller LH laesst 2 morphologische Bilder bei dieser Krankheit erkennen: In der Regel sind histologisch definierte LH-Infiltrate zu erkennen, die bevorzugt in der Hypothalamusregion - jedoch letztlich in jedem Teil des Gehirns - auftreten koennen und sich im MRT als gadoliniumaffine Raumforderung darstellen. Die andere Manifestationsform dominiert im Bereich des Nucleus dentatus des Kleinhirns und im Hirnstamm. Es handelt sich um Demyelinisierungsherde mit spaerlicher Histiozyteninfiltration, die im MRT als nicht gadoliniumaffine Bezirke zu erkennen sind. (orig.)

  18. Isolated Langerhans cell histiocytosis of the sublingual gland in an adult

    Science.gov (United States)

    Yang, Shaodong; Chen, Xinming; Zhang, Jiali; Fang, Qiong

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder characterized by the proliferation of pathologic Langerhans cells. Its clinical presentation is highly variable, that range from single-system, limited disease to severe, multi-organ disease with high mortality. LCH usually affects children and young adults. The most frequent sites for LCH are the bone, skin, lung, pituitary gland, and lymph nodes. Salivary gland involvement by LCH is extremely rare, and only a few cases of LHC involving the parotid glands have been reported in the English literature. To our knowledge, the involvement of the sublingual gland as a part of single or multisystem LCH has not been previously described. Herein we reported the first case of primary LCH of the sublingual gland. A 40-year-old woman presented with a 2-month history of a painless mass on the right sublingual area. Excision of the lesion including the right sublingual gland was performed. Histopathological diagnosis of LCH was rendered. The patient remains free of symptoms 17 months after surgery. PMID:26722591

  19. Role of multidisciplinary approach in a case of Langerhans cell histiocytosis with initial periodontal manifestations

    Science.gov (United States)

    Cisternino, Angelo; Asa’ad, Farah; Fusco, Nicola; Ferrero, Stefano; Rasperini, Giulio

    2015-01-01

    Introduction: Langerhans cell histiocytosis (LCH) is a rare inflammatory myeloid neoplasia of unknown etiology occurring in both children and adults. This condition is characterized by an abnormal proliferation of Langerhans cells that may virtually affect all sites in the human body. Oral manifestations of LCH could be the first clinical sign of disease and its periodontal localization could be easily mistaken for other more common entities, such as chronic periodontitis, aggressive periodontitis, and necrotizing ulcerative periodontitis. Case presentation: A 32-years old female visited a private dental practice with a chief complaint of sensitivity in the mandibular left first molar. Clinical and radiographic examination revealed deep periodontal pocket, recession, furcation involvement, mobility, severe alveolar bone destruction and a diagnosis of aggressive periodontitis was rendered. Multiple tooth extractions were carried out due to progressive periodontal destruction with impaired healing and development of ulcerative lesions. Multidisciplinary investigation demonstrated that the periodontal involvement was a manifestation of an underlying systemic disease. A biopsy of a bone lesion was therefore performed, revealing the presence of multifocal single system LCH. Conclusion: The identification of periodontal LCH is not trivial given that it may clinically resemble other periodontal disease entities. The dentist can be the first health care personnel to unravel the presence of an underlying systemic LCH. PMID:26722570

  20. Local Langerhans cell histiocytosis (eosinophilic granuloma in a six-month baby: a case report

    Directory of Open Access Journals (Sweden)

    Bahador M

    2008-12-01

    Full Text Available "nBackground: Langerhans cell histiocytosis (LCH is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells and mature eosinophils. The estimated annual incidence ranges from 0.5-2 cases per 100,000 persons per year. The pathogenesis of LCH is unknown. The prevalence of LCH seems to be higher among whites and males. The most common complaints at presentation are those related to bone lesions. Treatment consists of surgery, chemotherapy and radiotherapy alone or in combination. The age of onset varies according to the variety of LCH. Solitary lesions may occur in bones or skin. Cutaneous lesions present with firm, painless papulonodules or vesicles. "nCase report: This six-month-old baby presented with firm papulonodules on her temporal skin, but fortunately her other organs were healthy. She underwent two surgeries, separated by a one-month interval. Due to local recurrence after a short period of time, she underwent a 10-Gy dose of radiation. Her response proved good during follow-up. "nConclusion: Radiotherapy is good for controlling local recurrence in LCH, with few sequelae related to treatment.

  1. A solitary pulmonary ground-glass nodule in adult systemic langerhans' cell histiocytosis.

    Science.gov (United States)

    Zhou, Ying; Li, Yunyuan; Fan, Li; Liu, Shiyuan

    2015-01-01

    During a thoracic computed tomography (CT) scan, a 36-year-old male was diagnosed with a solitary oval pulmonary mixed ground-glass nodule in the right upper lobe of the lung. The edge of the nodule was well-defined, and its largest axial size was approximately 1.1 × 0.9 cm(2). This nodule was slightly lobulated, but not obviously speculated. Solid components, micro-cystic lucency shadow, small high-density rings and tiny vascular branches were all visible in the nodule. During hospitalization, a technetium 99 m methylene diphosphonate (Tc-99 m MDP) bone scan was performed, which showed a skeletal foci with abnormal uptake in the left iliac. A pulmonary lobectomy of the right upper lobe of the lung by video-assisted thoracoscopy was performed. In post-operative pathological photomicrographs, proliferative Langerhans' cells, eosinophils and lymphocytes were found. Immunohistochemistry showed that the expression of S-100 protein, CD1a, and CD68 antigen all stained positive. Since Langerhans' cell histiocytosis (LCH) that is also associated with isolated mixed ground-glass nodules is relatively rare, such a multi-systemic LCH case as identified herein, is reported. PMID:26722574

  2. [Pulmonary Langerhans cell histiocytosis in a non-smoking Japanese woman].

    Science.gov (United States)

    Tadokoro, Akira; Ishii, Tomoya; Bandoh, Shuji; Yokomise, Hiroyasu; Haba, Reiji; Ishida, Toshihiko

    2011-03-01

    A 44-year-old Japanese woman was admitted to our hospital because of dry cough and dyspnea on exertion. She had never smoked. She had been passively exposed to smoking by her husband and co-workers from the age of 21 (1984) to 33 (1996). She had previously developed pneumothorax twice, in 1985. On admission, computed tomography (CT) of the chest showed reticulonodular opacities predominant in bilateral upper lung fields, and pulmonary function tests revealed a decrease in vital capacity. The differential diagnoses were sarcoidosis, idiopathic pulmonary fibrosis and pulmonary Langerhans cell histiocytosis (PLCH). Video-assisted thoracic surgery was performed to make a definitive diagnosis. A histological specimen revealed the presence of CD1a-positive Langerhans cells in bronchiolocentric nodular lesions, leading to a diagnosis of PLCH. She was given 0.5 mg/kg bodyweight/ day oral prednisolone. Her symptoms disappeared with steroid maintenance therapy, and her vital capacity on pulmonary function testing was prevented from further deterioration. Based on the pathogenesis of PLCH, this case suggested that not only active smoking, but also passive smoking, played an important role in the development of PLCH. PMID:21485154

  3. [Xanthomas in a patient with Langerhans cell histiocytosis and liver cirrhosis].

    Science.gov (United States)

    Calzado, Leticia; Postigo, Concepción; Prado Sánchez-Caminero, M; Sanz, Henar; Guerra, Aurora; Vanaclocha, Francisco; Rodríguez-Peralto, José L

    2005-10-01

    Skin involvement in acute forms of Langerhans cell histiocytosis (LCH) is in the form of erythematous papules, although rare forms of xanthomatous lesions have been described. We present the case of a boy with acute disseminated LCH who, at the age of 16 months, began to experience outbreaks of seborrheic dermatitis-like skin lesions and progressive hepatic dysfunction. The symptoms were complicated by partial central diabetes insipidus and specific pulmonary infiltration by Langerhans cells, which led to fibrosis. During the course of the disease, the patient developed liver cirrhosis, alterations in the lipid profile and disseminated xanthomatous skin lesions, concomitant with the lesions specific to the LCH. Despite successive cycles of chemotherapy, the outcome was the death of the patient after five years, due to his liver disease. Xanthomatous lesions in LCH are typical of the late stages of chronic progressive forms, such as Hand-Schüller-Christian disease. When they appear in acute disseminated forms, there is some controversy over whether they correspond to a progression of the disease towards more chronic forms, or whether they are associated independent lesions, such as in this case.

  4. The role of bone scintigraphy in Langerhans' cell histiocytosis; a case report; Interet de la scintigraphie osseuse dans l'histiocytose langerhansienne: a propos d'un cas

    Energy Technology Data Exchange (ETDEWEB)

    Razzouk, M.; Carrier, P.; Darcourt, J. [Centre TEP, Hopital de l' Archet-1, Service Universitaire de Medecine Nucleaire, 06 - Nice (France); Razzouk, M.; Carrier, P.; Darcourt, J. [Federation interhospitaliere, CHU, CAL universitaire de Nice, 06 - Nice (France); Deville, A. [Hopital de l' Archet, Service d' Hemato-Oncologie Pediatrique, 06 - Nice (France); Cadet, G. [Centre Hospitalier intercommunal de Grasse, Service de Pediatrie, 06 - Grasse (France)

    2008-09-15

    We present one case of bone-Langerhans cell histiocytosis in a three-year-old male child presenting osseous lesions in the skull and the femur, which are very frequent localizations in histiocytosis. Bone scintigraphy is useful for both initial staging and follow-up associated with other imaging modalities. (authors)

  5. Efficacy of vinblastine in central nervous system Langerhans cell histiocytosis: a nationwide retrospective study

    Directory of Open Access Journals (Sweden)

    Ng Wing Tin Sophie

    2011-12-01

    Full Text Available Abstract Background Vinblastine (VBL is the standard treatment for systemic Langerhans cell histiocytosis (LCH, but little is known about its efficacy in central nervous system (CNS mass lesions. Methods A retrospective chart review was conducted. Twenty patients from the French LCH Study Group register met the inclusion criteria. In brief, they had CNS mass lesions, had been treated with VBL, and were evaluable for radiologic response. Results The median age at diagnosis of LCH was 11.5 years (range: 1-50. Intravenous VBL 6 mg/m2 was given in a 6-week induction treatment, followed by a maintenance treatment. The median total duration was 12 months (range: 3-30. Eleven patients received steroids concomitantly. Fifteen patients achieved an objective response; five had a complete response (CR: 25%, ten had a partial response (PR: 50%, four had stable disease (SD: 20% and one patient progressed (PD: 5%. Of interest, four out of the six patients who received VBL without concomitant steroids achieved an objective response. With a median follow-up of 6.8 years, the 5-year event-free and overall survival was 61% and 84%, respectively. VBL was well-tolerated and there were no patient withdrawals due to adverse events. Conclusion VBL, with or without steroids, could potentially be a useful therapeutic option in LCH with CNS mass lesions, especially for those with inoperable lesions or multiple lesions. Prospective clinical trials are warranted for the evaluation of VBL in this indication.

  6. Clinicopathological pattern of cranial unifocal Langerhans cell histiocytosis: A study at medical college hospital

    Directory of Open Access Journals (Sweden)

    Salma Bhat

    2015-01-01

    Full Text Available Background: Eosinophilic granuloma (EG of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic or multiple sites of involvement (polyostotic. Materials and Methods: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014. They all had been preoperatively evaluated by skull X-ray and computed tomography. To rule out a multifocal disease scintigraphy was performed in all cases preoperatively. Surgical excision was performed, and EG was diagnosed on histopathology and immunohistochemistry. Results: There was a male predominance. Parietal bone was the most common affected bone. Total excision of the lesion was performed in all cases. No patient received postoperative radiotherapy. The follow-up period ranged from 6 months to 3 years. No tumor recurrence was noted. Conclusion: With an unknown etiology, nonspecific clinical and radiological findings with diagnosis possible only on histopathological examination, EG needs to be considered in the differential diagnosis as a skull mass, especially in children.

  7. Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings

    Energy Technology Data Exchange (ETDEWEB)

    Binkovitz, Larry A.; Adler, Brent H. [Department of Radiology, Columbus Children' s Hospital, OH (United States); Olshefski, Randal S. [Department of Hematology and Oncology, Columbus Children' s Hospital, OH (United States)

    2003-09-01

    Bone involvement in Langerhans' cell histiocytosis (LCH) is common. Both bone scintigraphy and plain films are used to identify osseous lesions, but lack specificity for disease activity and response to therapy. FDG-PET is a sensitive technique for identifying bone lesions when histiocytes are present. To describe the potential of coincidence FDG-PET (cFDG-PET) for identification of active bone lesions in LCH and to determine whether it can provide more specific information regarding lesional response to therapy than bone scintigraphy or radiography. The clinical data and imaging findings of three patients with osseous lesions of LCH were retrospectively reviewed. cFDG-PET identified all active LCH osseous lesions in these patients, differentiated active from healed lesions, and demonstrated normalization of uptake in a treated lesion earlier than bone scintigraphy and radiography. cFDG-PET appears to have greater specificity than bone scintigraphy and radiography for the identification of active osseous lesions in LCH. It also may predict response to treatment earlier than conventional techniques. Its use in the evaluation of LCH warrants further study. (orig.)

  8. Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

    Science.gov (United States)

    Choi, Jung-Eun; Lee, Hae Ri; Ohn, Jung Hun; Moon, Min Kyong; Park, Juri; Lee, Seong Jin; Choi, Moon-Gi; Yoo, Hyung Joon; Kim, Jung Han

    2014-01-01

    We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH) involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT). He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated. PMID:25309800

  9. Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?

    Directory of Open Access Journals (Sweden)

    Andreas Meyer

    2012-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is a rare disease of unknown etiology with different clinical features. A standardised treatment has not been established so far. Case Report. We report a case of a 28-year-old patient who initially presented with hypesthesia of the fifth cranial nerve and pain of the left ear. Diagnosis showed a tumour localised in the cranial base with a maximum diameter of 4.1 cm. The diagnosis of LCH was confirmed histologically by biopsy. Diagnostic workup verified the cranial lesion as the sole manifestation of LCH. A total dose of 9 Gy (single dose 1.8 Gy was delivered. The symptoms dissolved completely within 6 months after radiation; repeated CT and MRI scans revealed a reduction in size of the lesion and a remineralisation of the bone. After a followup of 13 years the patient remains free of symptoms without relapse or any side effects from therapy. Discussion. Due to the indolent course of the disease with a high rate of spontaneous remissions the choice of treatment strongly depends on the individual clinical situation. In the presented case low-dose radiotherapy was sufficient to obtain long-term local control in a region with critical structures and tissues.

  10. Cladribine and cytarabine in refractory multisystem Langerhans cell histiocytosis: results of an international phase 2 study

    Science.gov (United States)

    Bernard, Frederic; van Noesel, Max; Barkaoui, Mohamed; Bardet, Odile; Mura, Rosella; Arico, Maurizio; Piguet, Christophe; Gandemer, Virginie; Armari Alla, Corinne; Clausen, Niels; Jeziorski, Eric; Lambilliote, Anne; Weitzman, Sheila; Henter, Jan Inge; Van Den Bos, Cor

    2015-01-01

    An international phase 2 study combining cladribine and cytarabine (Ara-C) was initiated for patients with refractory, risk-organ–positive Langerhans cell histiocytosis (LCH) in 2005. The protocol, comprising at least two 5-day courses of Ara-C (1 g/m2 per day) plus cladribine (9 mg/m2 per day) followed by maintenance therapy, was administered to 27 patients (median age at diagnosis, 0.7 years; median follow-up, 5.3 years). At inclusion, all patients were refractory after at least 1 course of vinblastine (VBL) plus corticosteroid, all had liver and spleen involvement, and 25 patients had hematologic cytopenia. After 2 courses, disease status was nonactive (n = 2), better (n = 23), or stable (n = 2), with an overall response rate of 92%. Median disease activity scores decreased from 12 at the start of therapy to 3 after 2 courses (P < .0001). During maintenance therapy, 4 patients experienced reactivation in risk organs. There were 4 deaths; 2 were related to therapy toxicity and 2 were related to reactivation. All patients experienced severe toxicity, with World Health Organization grade 4 hematologic toxicity and 6 documented severe infections. The overall 5-year survival rate was 85% (95% confidence interval, 65.2%-94.2%). Thus, the combination of cladribine/Ara-C is effective therapy for refractory multisystem LCH but is associated with high toxicity. PMID:26194764

  11. Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy

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    Goo, Hyun Woo; Yang, Dong Hyun [Asan Medical Center, Department of Radiology, University of Ulsan College of Medicine, Seoul (Korea); Ra, Young Shin [Asan Medical Center, Department of Neurosurgery, University of Ulsan College of Medicine, Seoul (Korea); Song, Joon Sup; Im, Ho Joon; Seo, Jong Jin; Ghim, Thad; Moon, Hyung Nam [Asan Medical Center, Department of Pediatric Oncology, University of Ulsan College of Medicine, Seoul (Korea)

    2006-10-15

    In Langerhans cell histiocytosis (LCH) evaluation of the extent of disease is one of the major predictors of patient outcome. Historically this is undertaken using plain radiography and bone scintigraphy. Recently, whole-body (WB) MRI has been reported to be useful in detecting skeletal and extraskeletal metastases in both adults and children. To evaluate the usefulness of WB MRI in patients with LCH in comparison with plain radiography and bone scintigraphy. In nine children (1-7 years of age; mean 3.3 years) who had a pathological diagnosis of LCH and had either plain radiography or bone scintigraphy for comparison, 43 WB MR examinations were performed. Skeletal and extraskeletal lesions of the disease on WB MRI were compared with those on plain radiography and bone scintigraphy. LCH showed unifocal single-system involvement in one patient, multifocal single-system involvement in three, and multifocal multisystem disease in five. WB MRI identified additional skeletal lesions in three (38%) of eight patients, compared with plain radiography, and in two (25%) of eight, compared with bone scintigraphy. WB MRI detected extraskeletal lesions of the disease in five (56%) of the nine patients exclusively, except for one patient whose lung lesions were also detected on plain radiography. In two patients, treatment was changed according to WB MRI findings. (orig.)

  12. A confusing case report of pulmonary langerhans cell histiocytosis and literature review

    Directory of Open Access Journals (Sweden)

    Li Yang

    2016-06-01

    Full Text Available Pulmonary Langerhans Cell Histiocytosis (PLCH is a rare disease. From the insidious onset and nonspecific manifestations, it is difficult to diagnose PLCH. To help improve the diagnosis and therapy options of adult PLCH, we present this case report and literature review about a confusing case of PLCH. In this report, we present a 37-year-old male PLCH case that was negative for CD1a and S100 expression. Smoking cessation and use of prescribed Spiriva appeared to improve the patient’s symptoms. To the best of our knowledge, this is the first reported case of PLCH in which improved symptoms were seen with the use of Spiriva alone.The mechanism is not clear, but potentially has some relationship with dilating the airway, decreasing the mucous hypersecretion and promoting anti-inflammatory pathways. From this patient’s case, we may be able to find more cases to then find other first line therapies for PLCH patients.

  13. Clinicopathological pattern of cranial unifocal Langerhans cell histiocytosis: A study at medical college hospital

    Science.gov (United States)

    Bhat, Salma; Nazir, Parvez; Bashir, Humaira; Reshi, Ruby; Sheikh, Sheema; Wani, Rohi

    2015-01-01

    Background: Eosinophilic granuloma (EG) of bone refers to a generally benign form of Langerhans cell histiocytosis localized to the bone. Patients may present with a solitary lesion (monostotic) or multiple sites of involvement (polyostotic). Materials and Methods: This study was done to evaluate the clinicopathological pattern of 6 cases of EGs of the skull diagnosed at a tertiary care hospital. All patients of EG were included with the help of medical records over a 5-year period that is, November 2009 to November 2014. They all had been preoperatively evaluated by skull X-ray and computed tomography. To rule out a multifocal disease scintigraphy was performed in all cases preoperatively. Surgical excision was performed, and EG was diagnosed on histopathology and immunohistochemistry. Results: There was a male predominance. Parietal bone was the most common affected bone. Total excision of the lesion was performed in all cases. No patient received postoperative radiotherapy. The follow-up period ranged from 6 months to 3 years. No tumor recurrence was noted. Conclusion: With an unknown etiology, nonspecific clinical and radiological findings with diagnosis possible only on histopathological examination, EG needs to be considered in the differential diagnosis as a skull mass, especially in children. PMID:26855527

  14. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients

    Energy Technology Data Exchange (ETDEWEB)

    Song, You Seon; Lee, In Sook; Kim, Do Kyung [Pusan National University Hospital, Medical Research Institute, Department of Radiology, Busan (Korea, Republic of); Pusan National University, School of Medicine, Department of Radiology, Busan (Korea, Republic of); Yi, Jae Hyuck [Kyungpook National University Hospital, Department of Radiology, Daegu (Korea, Republic of); Cho, Kil Ho [Yeungnam University College of Medicine, Department of Radiology, Daegu (Korea, Republic of); Song, Jong Woon [Inje University Haeundae Paik Hospital, Department of Radiology, Busan (Korea, Republic of)

    2011-11-15

    The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a ''budding'' appearance on MRI; and the presence of sclerotic margins or septations on CT. The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults. (orig.)

  15. A Rare Case of Vulvar Langerhans Cell Histiocytosis and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Didem Didar Balcı

    2009-12-01

    Full Text Available Langerhans cell histiocytosis (LCH of the vulva is rare. A 32 year-old woman presented with a two year history of non-healing vulvar ulceration and a nine month history of ulcerative nodular lesion in the left inguinal region. Biopsy results of the vulva and inguinal lesions were consistent with LCH. Screening of the body systems revealed hepatosplenomegaly, iliac, inguinal, paraaortic lymph nodes and lung involvement. She was treated with systemic steroid, resulting in complete improvement of vulvar lesion. She also underwent a local excision of left inguinal lesion. Three months later, we found a lesion on her left inguinal region that was consistent with a recurrence and enlargement in the lymph nodes. Therefore, high dose systemic steroid therapy and then a 12-session radyotherapy were given. After the treatment, a complete improvement was obtained. A 1-year of systemic methotrexate therapy was recommended to prevent recurrence of the disease. In women with vulvar ulcer, LCH should be considered in the differential diagnosis.

  16. {sup 18}F-FDG PET in neuro-degenerative Langerhans cell histiocytosis

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    Ribeiro, M.Jo. [CEA, DSV, DRM, Serv Hosp Frederic Joliot, F-91406 Orsay (France); Idbaih, A.; Hoang-Xuan, K. [UPMC, Grp Hosp Pitie Salpetriere, ServNeurol Mazarin, AP-HP, Paris (France); Thomas, C. [CHU Nantes, Unite Hematol et Oncol Pediat, F-44035 Nantes 01 (France); Remy, P. [CEA, Serv Hosp Frederic Joliot, CNRS, URA 2210, F-91406 Orsay (France); Remy, P. [CHU Henri Mondor, Fac Med Paris 12, Dept Neurosci, AP-HP, F-94010 Creteil (France)

    2008-07-01

    Introduction: The so called 'neuro-degenerative Langerhans cell histiocytosis' (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease. Objectives: To search for CNS metabolic changes in NDLCH. Methods: Seven patients suffering from ND-LCH were investigated by {sup 18}F-FDG PET in this prospective study and compared with 21 healthy controls. Results: ND-LCH patients demonstrated recurrent abnormalities including bilateral hypo-metabolism in the cerebellum, the basal ganglia (caudate nuclei), frontal cortex and, bilateral, a relatively increased metabolism in the amygdalae (p {<=} 0.001). Functional changes in these anatomical regions may be detected in the absence of any apparent lesion on MRI. Conclusions: ND-LCH demonstrates a recurrent {sup 18}F-FDG PET metabolic signature. Our results suggest that {sup 18}F-FDG PET might be a useful tool for an early diagnosis of ND-LCH before neuro-radiologic abnormalities appear. (authors)

  17. Radiotherapy for bony manifestations of Langerhans Cell Histiocytosis. Review and proposal for an international registry

    Energy Technology Data Exchange (ETDEWEB)

    Olschewski, T.; Seegenschmiedt, M.H. [Dept. of Radiotherapy and Radiation Oncology, Alfried Krupp Krankenhaus, Essen (Germany)

    2006-02-01

    Purpose: to examine the role of radiotherapy (RT) in adult Langerhans Cell Histiocytosis (LCH) for osseous manifestations, to define open questions regarding RT, and to develop recommendations for the clinical decision-making and problem-solving process. Material and methods: a literature review using different medical databases was conducted including the last 3 decades, and resulting questions regarding the use of ionizing radiation were systematically compiled. Results: the literature review revealed a local control rate of 96% (93% complete remissions) in patients with osseous single-system disease and of 92% (76% complete remissions) in patients with bony involvement in multi-system disease. To increase our knowledge, a prospective registry has been developed to allow a differentiated analysis of RT outcome and definition of potential prognostic factors. Conclusion: ionizing radiation can be successfully applied as a single treatment or in combination with other therapies for osseous manifestations of LCH. It leads to high remission and local control rates. Nevertheless, many open questions still exist. A prospective clinical registry is proposed to define the exact role of RT in this disease and to develop future interdisciplinary treatment guidelines. (orig.)

  18. Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone

    Energy Technology Data Exchange (ETDEWEB)

    Nieuwenhuyse, J.P. van [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Clapuyt, P. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Malghem, J. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Everarts, P. [Department of Radiology and Medical Imaging, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Melin, J. [Department of Nuclear Medicine, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Pauwels, S. [Department of Nuclear Medicine, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Brichard, B. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Ninane, J. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Vermylen, C. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium); Cornu, G. [Department of Pediatric Hematology, Cliniques Universitaires St-Luc, University of Louvain Medical School, Brussels (Belgium)

    1996-10-01

    Background. The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. Objective. To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. Materials and methods. Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). Results. Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/RBS+ abnormalities (n = 10) were most frequently insignificant. Conclusion. In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed. (orig.). With 2 figs., 4 tabs.

  19. Langerhans cell histiocytosis: a retrospective analysis in a Korean tertiary hospital from 2003 to 2012.

    Science.gov (United States)

    Kwon, Soon Hyo; Choi, Jae Woo; Kim, Hyo Jin; Youn, Sang Woong

    2013-10-01

    Epidemiological study of Langerhans cell histiocytosis (LCH) has been limited due to its rarity and multisystemic involvement. The aim of this study was to investigate the epidemiological features of LCH via the clinical data warehouse (CDW). Clinical data of 30 LCH patients from the all departments of a tertiary referral hospital between 2003 and 2012 were analyzed retrospectively by searching the CDW. The male-to-female ratio was 2.8:1. The age of onset ranged 7 days to 57 years with a median of 13 years. Of the patients, 36.7% presented initial symptoms before the age of 10 years. The involved organs at diagnosis were: bone (66.7%), skin (16.7%), lungs (13.3%) and lymph node (3.3%). For all of the 30 cases, there were 31 disease sites because of a single case of multisystemic disease involving both skin and bone. Of the 96.7% of patients with single-system disease, 69.0% had bony involvement. This study elucidated the clinical features of LCH from all the departments of a tertiary hospital via the CDW, which suggests a potential role of the CDW as a new epidemiological approach for rare diseases.

  20. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients

    International Nuclear Information System (INIS)

    The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a ''budding'' appearance on MRI; and the presence of sclerotic margins or septations on CT. The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults. (orig.)

  1. Langerhans cell histiocytosis in children: CT manifestation (report of 13 cases)

    International Nuclear Information System (INIS)

    Objective: To study the value and limitation of CT in the damage to multiple organs in children with Langerhans cell histiocytosis (LCH). Methods: Analysis of CT manifestations was performed in 13 cases of LCH proved by clinical features, laboratory data, and pathologic changes. Localized LCH was found in 4 cases, and disseminated LCH in 9 cases. All cases were examined by plain scan in craniocerebral, chest, liver, and spleen, and four cases by contrast-enhanced CT scan. Results: The male outnumbered female in this group. Cranial bones were involved in the sequence of temporal bone (8 cases), parietal bone (3 cases), occipital bone (2 cases), and frontal bone (1 case). CT findings were very sensitive in showing the early destructions of the bones. The characteristic manifestation on CT was multiple lytic lesions of the flat bones at any sites, without reactive sclerosis and soft tissue mass accompanied with the bony lesion. Imaging findings presented later than the clinical manifestations for LCH involving the hypothalamus/pituitary axis. Involvement of the lung at various stages by LCH could be demonstrated by CT scan. Conclusion: Imaging manifestations had no characteristics in LCH involvement. The sites of predilection and related CT manifestations could be suggestive of the diagnosis. In addition, CT findings can be helpful in the classification and evaluation of the therapy and prognosis in LCH

  2. Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone

    International Nuclear Information System (INIS)

    Background. The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. Objective. To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. Materials and methods. Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). Results. Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/RBS+ abnormalities (n = 10) were most frequently insignificant. Conclusion. In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed. (orig.). With 2 figs., 4 tabs

  3. 18F-FDG PET in neuro-degenerative Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Introduction: The so called 'neuro-degenerative Langerhans cell histiocytosis' (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease. Objectives: To search for CNS metabolic changes in NDLCH. Methods: Seven patients suffering from ND-LCH were investigated by 18F-FDG PET in this prospective study and compared with 21 healthy controls. Results: ND-LCH patients demonstrated recurrent abnormalities including bilateral hypo-metabolism in the cerebellum, the basal ganglia (caudate nuclei), frontal cortex and, bilateral, a relatively increased metabolism in the amygdalae (p ≤ 0.001). Functional changes in these anatomical regions may be detected in the absence of any apparent lesion on MRI. Conclusions: ND-LCH demonstrates a recurrent 18F-FDG PET metabolic signature. Our results suggest that 18F-FDG PET might be a useful tool for an early diagnosis of ND-LCH before neuro-radiologic abnormalities appear. (authors)

  4. Radiotherapy for bony manifestations of Langerhans Cell Histiocytosis. Review and proposal for an international registry

    International Nuclear Information System (INIS)

    Purpose: to examine the role of radiotherapy (RT) in adult Langerhans Cell Histiocytosis (LCH) for osseous manifestations, to define open questions regarding RT, and to develop recommendations for the clinical decision-making and problem-solving process. Material and methods: a literature review using different medical databases was conducted including the last 3 decades, and resulting questions regarding the use of ionizing radiation were systematically compiled. Results: the literature review revealed a local control rate of 96% (93% complete remissions) in patients with osseous single-system disease and of 92% (76% complete remissions) in patients with bony involvement in multi-system disease. To increase our knowledge, a prospective registry has been developed to allow a differentiated analysis of RT outcome and definition of potential prognostic factors. Conclusion: ionizing radiation can be successfully applied as a single treatment or in combination with other therapies for osseous manifestations of LCH. It leads to high remission and local control rates. Nevertheless, many open questions still exist. A prospective clinical registry is proposed to define the exact role of RT in this disease and to develop future interdisciplinary treatment guidelines. (orig.)

  5. Whole-body MRI of Langerhans cell histiocytosis: comparison with radiography and bone scintigraphy

    International Nuclear Information System (INIS)

    In Langerhans cell histiocytosis (LCH) evaluation of the extent of disease is one of the major predictors of patient outcome. Historically this is undertaken using plain radiography and bone scintigraphy. Recently, whole-body (WB) MRI has been reported to be useful in detecting skeletal and extraskeletal metastases in both adults and children. To evaluate the usefulness of WB MRI in patients with LCH in comparison with plain radiography and bone scintigraphy. In nine children (1-7 years of age; mean 3.3 years) who had a pathological diagnosis of LCH and had either plain radiography or bone scintigraphy for comparison, 43 WB MR examinations were performed. Skeletal and extraskeletal lesions of the disease on WB MRI were compared with those on plain radiography and bone scintigraphy. LCH showed unifocal single-system involvement in one patient, multifocal single-system involvement in three, and multifocal multisystem disease in five. WB MRI identified additional skeletal lesions in three (38%) of eight patients, compared with plain radiography, and in two (25%) of eight, compared with bone scintigraphy. WB MRI detected extraskeletal lesions of the disease in five (56%) of the nine patients exclusively, except for one patient whose lung lesions were also detected on plain radiography. In two patients, treatment was changed according to WB MRI findings. (orig.)

  6. Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings

    International Nuclear Information System (INIS)

    Bone involvement in Langerhans' cell histiocytosis (LCH) is common. Both bone scintigraphy and plain films are used to identify osseous lesions, but lack specificity for disease activity and response to therapy. FDG-PET is a sensitive technique for identifying bone lesions when histiocytes are present. To describe the potential of coincidence FDG-PET (cFDG-PET) for identification of active bone lesions in LCH and to determine whether it can provide more specific information regarding lesional response to therapy than bone scintigraphy or radiography. The clinical data and imaging findings of three patients with osseous lesions of LCH were retrospectively reviewed. cFDG-PET identified all active LCH osseous lesions in these patients, differentiated active from healed lesions, and demonstrated normalization of uptake in a treated lesion earlier than bone scintigraphy and radiography. cFDG-PET appears to have greater specificity than bone scintigraphy and radiography for the identification of active osseous lesions in LCH. It also may predict response to treatment earlier than conventional techniques. Its use in the evaluation of LCH warrants further study. (orig.)

  7. Clinical and Radiological Presentation of Pulmonary Langerhans Cell Histiocytosis: A Case Series of 20 Patients

    Directory of Open Access Journals (Sweden)

    A. Fakharian

    2008-07-01

    Full Text Available Introduction & Objective: Pulmonary Langerhans Cell Histiocytosis (PLCH is an uncommon (5% ILD interstitial tissue disorder with significant importance. It occurs predominantly in adult smokers. The organs involved in LCH include skin, bone, pituitary gland, thyroid, lymph node, and lungs.Materials & Methods: In this descriptive study all patients admitted to the Masih Daneshvari Hospital in Tehran from 1996 to 2007 diagnosed with LCH (recognized by clinical features, laboratory investigations, radiological manifestations, BAL and biopsy were evaluated.Results: A total of 20 patients were studied; mean age was 27.8 yrs.; 40% were smokers; 80% had pulmonary involvement while in 20% other organs were affected. On spirometry, a mixed pattern (50% was the most common and on chest radiography (CXR 77% of lung cysts were visible. The most frequent finding on CT- scan was diffuse lung cysts (80%.Conclusion: It is notable that ground glass opacity and consolidation occur in the early stages of disease. Since most of the patients refer with features of advanced disease, it is essential for the physicians to consider the early signs during the diagnostic stage. Also bronchoscopy and bronchial lavage are recommended in selected cases.

  8. Pulmonary Langerhans' cell granulomatosis (histiocytosis X): clinical analysis of 8 cases.

    Science.gov (United States)

    Callebaut, W; Demedts, M; Verleden, G

    1998-10-01

    A detailed retrospective analysis of 8 cases has been set up to obtain an update of the clinical, radiological, diagnostic and therapeutical aspects of pulmonary Langerhans' cell granulomatosis (PLCG), previously called Histiocytosis X. This disease represents 2.8% of interstitial lung diseases (ILD) in a registration by pneumologists in Flanders. Seventy five% of our patients were active smokers. Cough, dyspnoe and constitutional symptoms were the main presenting symptoms. There was a 37.5% frequency of pneumothorax during the whole disease evolution and all these patients had to be treated with chemical or surgical pleurodesis. Spirometric pattern was variable but CO-transferfactor (TLCO) was significantly impaired in all our patients. Radiologically nodules and/or cystic lesions were found with preserved volumes. Open lung biopsy led to the diagnosis in 6 of the 8 cases. Transbronchial biopsies (TBB) were found to be not sensitive. Systemic staging in our group showed 50% of the patients to have a second focus of organ involvement. Treatment with corticosteroids and possibly immunosuppressives was without clear effect. Since 25% of our patients ultimately became candidate for lung transplantation extrapulmonary staging is prerogative and the high frequency of pleurodesis procedures in the past medical history of our patients has important implications since bilateral pleurodesis is still a contraindication for lung transplantation in some but not all institutions. PMID:9861760

  9. Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus

    Science.gov (United States)

    2016-01-01

    Etiologies of a thickened stalk include inflammatory, neoplastic, and idiopathic origins, and the underlying diagnosis may remain occult. We report a patient with a thickened pituitary stalk (TPS) and papillary thyroid carcinoma (PTC) whose diagnosis remained obscure until a skin lesion appeared. The patient presented with PTC, status postthyroidectomy, and I131 therapy. PTC molecular testing revealed BRAF mutant (V600E, GTC>GAG). She had a 5-year history of polyuria/polydipsia. Overnight dehydration study confirmed diabetes insipidus (DI). MRI revealed TPS with loss of the posterior pituitary bright spot. Evaluation showed hypogonadotropic hypogonadism and low IGF-1. Chest X-ray and ACE levels were normal. Radiographs to evaluate for extrapituitary sites of Langerhans Cell Histiocytosis (LCH) were unremarkable. Germinoma studies were negative: normal serum and CSF beta-hCG, alpha-fetoprotein, and CEA. Three years later, the patient developed vulvar labial lesions followed by inguinal region skin lesions, biopsy of which revealed LCH. Reanalysis of thyroid pathology was consistent with concurrent LCH, PTC, and Hashimoto's thyroiditis within the thyroid. This case illustrates that one must be vigilant for extrapituitary manifestations of systemic diseases to diagnose the etiology of TPS. An activating mutation of the protooncogene BRAF is a potential unifying etiology of both PTC and LCH. PMID:27656301

  10. Purely cutaneous Langerhans cell histiocytosis presenting as an ulcer on the chin in an elderly man successfully treated with thalidomide

    Science.gov (United States)

    Subramaniyan, Radhakrishnan; Ramachandran, Rajagopal; Rajangam, Gnanasekaran; Donaparthi, Navya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare, clonal proliferative disorder of Langerhans’ cells of unknown etiology. Although the clinical presentation and therapeutic approach to the disease in children have been well established; limited data is available about the disease in adults. Purely cutaneous involvement of LCH in a man older than 70 years has rarely been described. Herein we report the case of a 71-year-old man with cutaneous LCH confined to the perioral region, scalp, and flexures successfully treated with thalidomide. PMID:26753141

  11. Histiocytosis--an introduction.

    OpenAIRE

    Pritchard, J.; Broadbent, V

    1994-01-01

    The Histiocytoses are a group of rare and puzzling multisystem disorders, currently regarded as non-malignant but often treated with 'cancer chemotherapy'. In this article, the origins of histiocytes and of the Histiocyte Society's classification of the Histiocytoses are described with suggested minor modifications to the classification. The current nomenclature for the 2 principal diseases, now named 'Langerhans cell histiocytosis' and 'Haemophagocytic Lymphohistiocytosis', is less confusing...

  12. BRAF and MAP2K1 mutations in Langerhans cell histiocytosis: a study of 50 cases.

    Science.gov (United States)

    Alayed, Khaled; Medeiros, L Jeffrey; Patel, Keyur P; Zuo, Zhuang; Li, Shaoying; Verma, Shalini; Galbincea, John; Cason, R Craig; Luthra, Rajyalakshmi; Yin, C Cameron

    2016-06-01

    Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells, often associated with lymphocytes, eosinophils, macrophages, and giant cells. BRAF mutations, usually V600E, have been reported in 40%-70% of cases, and recently, MAP2K1 mutations have been reported in BRAF-negative cases. We assessed 50 cases of LCH for BRAF mutations and assessed a subset of cases for MAP2K1 mutations. The study group included 28 men and 22 women (median age, 36.5 years; range, 1-78 years). BRAF V600E mutation was detected in 8 (16%) cases including 3 (30%) skin, 2 (11%) bone, 1 (50%) colon, 1 (20%) lung, and 1 (33%) extradural, intracranial mass. MAP2K1 mutations were detected in 6 of 13 (46%) BRAF-negative cases including 2 (100%) lymph node, 2 (50%) bone, 1 (25%) skin, and 1 (100%) orbit. Patients with BRAF mutation were younger than patients with wild-type BRAF (median age, 28 versus 38 years; P = .026). The median age of MAP2K1-mutated patients was 34.5 years, similar to patients without MAP2K1 mutation (41 years; P = .368). In agreement with 2 recent studies, we showed a high frequency of MAP2K1 mutations in BRAF-negative LCH cases. Unlike other studies, the overall frequency of BRAF mutation in this cohort is substantially lower than what has been reported in pediatric patients, perhaps because most patients in this study were adults. Moreover, we showed a high concordance between mutational and immunohistochemical analysis for BRAF mutation. There was no statistically significant association between BRAF or MAP2K1 mutation and anatomic site, unifocal versus multifocal presentation, or clinical outcome. PMID:26980021

  13. Radiotherapy of Langerhans' cell histiocytosis. Results and Implications of a national patterns-of-care study

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    Seegenschmiedt, M.H. [Alfried Krupp Krankenhaus, Essen (Germany). Dept. of Radiation Oncology and Radiotherapy; Olschewski, T.

    2006-11-15

    Purpose: This patterns-of-care study was performed to define the current clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany and to define open questions resulting from this study. Material and Methods: A standardized questionnaire was sent to 198 German radiotherapy institutions. Data about patient characteristics, stage of disease, practice and fractionation of radiotherapy, outcome of therapy, etc. were systematically evaluated. 123 of 198 institutions answered the complete questionnaire (62.1%). Results: Only 23 of the 123 institutions (18.7%) reported experience with radiotherapy of Langerhans' cell histiocytosis of adults. 18 institutions with 98 patients were evaluable. The majority of patients (72 of 98) was treated on a linear accelerator. The median single dose of radiotherapy was 2 Gy, while the median total dose was 24 Gy. 81 of 89 evaluable patients (91%) reached a local control of the treated lesion(s), 69 of those had a complete remission. Eight of 89 patients (9%) developed an in-field recurrence. 87.8% of patients experienced no acute and 97% of patients no late side effects of radiotherapy. Conclusion: Clinical experience with radiotherapy of Langerhans' cell histiocytosis in adults in Germany is still very limited. Nevertheless, the clinical results - with high remission and local control rates - confirm the effectiveness of radiotherapy in the multidisciplinary treatment of this disease. Due to the small number of patients in this study despite higher incidence rates, the knowledge of this disease has to be multiplied in Germany. Future patients should be systematically included into a prospective radiotherapy registry. (orig.)

  14. Primary Langerhans cell histiocytosis of the vulva: Report of a case and brief review of the literature

    Directory of Open Access Journals (Sweden)

    Pan Zenggang

    2009-01-01

    Full Text Available Primary Langerhans cell histiocytosis (LCH of the vulva is rare. Fifteen cases of primary cutaneous vulvar LCH have been reported in English literature. We report an additional case of LCH confined to the vulva. In this article, we describe the clinical presentation, histopathology and immunohistochemistry findings of vulvar LCH that are helpful to both gynecologists and pathologists in the diagnosis of this entity. We briefly discuss the pathogenesis of LCH. The debate whether LCH is a reactive or neoplastic entity is still ongoing.

  15. Long-Term Extracorporeal Membrane Oxygenation as Bridging Strategies to Lung Transplantation in Rapidly Devastating Isolated Langerhans Cell Histiocytosis.

    Science.gov (United States)

    Sacco, Oliviero; Moscatelli, Andrea; Conte, Massimo; Grasso, Chiara; Magnano, Gian Michele; Sementa, Angela Rita; Martelli, Alberto; Rossi, Giovanni A

    2016-05-01

    Isolated pulmonary involvement in pediatric Langerhans cell histiocytosis (LCH) is extremely rare. While the multisystem-LCH course varies from spontaneous remission to rapid deterioration with lethal outcome, single system involvement is generally associated with favorable prognosis. A child with isolated pulmonary LCH had an extremely rapid progression leading to respiratory failure, despite treatment with prednisone and vinblastine. Since lung hyperinflation and cystic degeneration contraindicated conventional mechanical ventilation, extracorporeal membrane oxygenation (ECMO) was chosen for 50 days as a bridge to lung transplantation. The mechanisms involved in disease progression and the usefulness of long-term ECMO are discussed. PMID:26840616

  16. Histiocytosis X in the lung.

    Science.gov (United States)

    Colby, T V; Lombard, C

    1983-10-01

    The histologic and clinical features of pulmonary histiocytosis X are discussed. The majority of patients are in the third and fourth decades of life. They present with nonspecific respiratory complaints and bilateral reticulonodular infiltrates that can be seen on chest radiographs. The histologic lesions of pulmonary histiocytosis X are scattered, discrete nodules that frequently center on small airways. The initially cellular nodules are replaced progressively by fibrous tissue. The cell infiltrates in active lesions contain variable numbers of eosinophils and histiocytes resembling Langerhans cells of the epidermis (Hx cells). In most patients the disease resolves or stabilizes, leaving few, if any, significant residues. A minority of patients develop progressive pulmonary disease that is ultimately fatal. The cause of pulmonary histiocytosis X is unknown, but two recent series suggest a relationship to cigarette smoking. PMID:6352456

  17. Detection of Epstein-Barr Virus DNA in Langerhans Cell Histiocytosis

    Science.gov (United States)

    Khoddami, Maliheh; Nadji, Seyed Alireza; Dehghanian, Paria; Vahdatinia, Mahsa; Shamshiri, Ahmad Reza

    2015-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare histiocytic proliferation of unknown etiology. It is characterized by granuloma-like proliferation of Langerhans-type dendritic cells and mainly affects young children. Although multiple investigators have suggested the possible role of viruses, such as Epstein-Barr virus (EBV), human herpesvirus-6 (HHV-6), Herpes simplex virus (HSV) types 1 and 2, and Cytomegalovirus (CMV) in the pathogenesis of LCH, it remains, however, debated. Objectives: The EBV infection is reported to be associated with LCH. Nevertheless, no report could be found about involved Iranian children in English medical literature. In this study, we investigated the presence of EBV in Iranian children with LCH. Patients and Methods: In this retrospective study, in which we investigated the prevalence of presence of EBV DNA in LCH, using paraffin-embedded tissue samples of 30 patients with LCH and 30 age and tissue-matched controls, who were operated for reasons other than infectious diseases (between the years 2002 and 2012), by real-time polymerase chain reaction (RT-PCR) method, in the department of pediatric pathology. No ethical issues arose in the study, because only the pathology reports were reviewed, retrospectively, and the patients were anonymous. Results: There was a significant difference in prevalence of EBV presence between patients and controls. The EBV was found by RT-PCR in 19 (63.33%) out of 30 patients and only in eight (26.7%) of 30 control samples. The P = 0.004, was calculated using chi-square test (OR: 4.75; 95% CI: 1.58 ‒ 14.25). Conclusions: Our study is the first investigation performed on patients with LCH and its possible association with EBV in Iran. Considering the P = 0.004, which is statistically significant, the findings do support the hypothesis of a possible role for EBV in the pathogenesis of LCH. These results are in accordance with several previous investigations, with positive findings. PMID:26870310

  18. Activity and toxicity of 2-CDA in Langerhans cell histiocytosis: A single institutional experience

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    Biswas G

    2007-01-01

    Full Text Available Background : Langerhans cell histiocytosis (LCH is a rare disorder characterized by clonal proliferation of immature and abnormal bone marrow derived langerhans cells. Treatment is usually multimodal. Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and its proven efficacy in many lymphoproliferative disorders has made 2-CDA a rational choice in treatment of LCH. Aim : To evaluate the efficacy and toxicity profile of 2-CDA in children with relapsed or refractory LCH. Setting and Design : This is a pilot study and we present the initial data of the first seven patients treated at our institution. Materials and Methods : Seven patients of relapsed and refractory LCH were enrolled from July 2000 to June 2004. The cohort of seven patients included six males and one female with a median age at initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years. Three patients had received one prior chemotherapy regimen while the rest were heavily pretreated. Cladribine was administered over two hours IV daily for five days and repeated every four weeks. Results : After a median of six courses of cladribine (range, 2 to 9, two (33% patients achieved PR and two (33% patients have SD on imaging but are clinically better. None experienced grade 3 or 4 hematologic toxicity. At a median follow-up of 19 months (range, 8 to 52 months, five patients remain alive and one patient has died. Conclusion : Our study shows that single agent 2-CDA is active and well-tolerated in children with relapsed or refractory LCH.

  19. Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

    Science.gov (United States)

    Li, Cheng-Wei; Li, Man-Hui; Li, Jiang-Xiong; Tao, Ru-Jia; Xu, Jin-Fu

    2016-01-01

    Background Pulmonary Langerhans cell histiocytosis (PLCH) is an orphan disease in respiratory medicine, which most affects adult smokers. The purpose of this article was to discuss the clinical features, especially the radiologic features of PLCH patients during their hospitalization through a retrospective analysis on clinical data. Furthermore, the current literature was also reviewed. Methods Between December 2008 and June 2012, 14 patients with PLCH were assessed at Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. Among these patients, seven patients were diagnosed through tissue biopsy from the lung and one patient from enlarged cervical lymph nodes; the rest of six patients were diagnosed based on the clinical-radiological data. The data consisting of demographics, clinical presentation, smoking habits, pulmonary function tests (PFTs) and radiographic image from the medical records was analyzed retrospectively. Results The average age of patients (11 males and 3 females) was 42.79 (±13.71) years old. All male patients and one female patient had a long smoking history. The common manifestations were cough and exertional dyspnea. Spontaneous pneumothorax was found in three patients. Varieties of pulmonary shadows such as nodular, cystic, patch-like and cord-like were revealed by chest computed tomography (CT) examination. Large Langerhans cells (LCs) were discovered in biopsy tissue by immunohistochemical stains. Conclusions PLCH is still an orphan disease and maybe related to smoking. Clinical symptoms such as cough and exertional dyspnea are non-specific. We shall pay attention to recurrent pneumothorax as clinically it is associated with PLCH. The characteristic radiological manifestation is cystic or nodular shadow in the lungs, which plays crucial roles in diagnosing PLCH. PMID:27293848

  20. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

    Energy Technology Data Exchange (ETDEWEB)

    Makras, P. [Athens General Hospital, Department of Endocrinology and Diabetes, Athens (Greece); Athens General Hospital, Department of Radiology, Athens (Greece); Samara, C.; Antoniou, M.; Nikolakopoulou, Z. [Athens Hospital, 9. Pulmonary Department, Athens (Greece); Zetos, A. [General Hospital, Department of Pathology, Athens (Greece); Papadogias, D.; Piaditis, G.; Kaltsas, G.A. [Athens General Hospital, Department of Endocrinology and Diabetes, Athens (Greece); Toloumis, G. [Athens General Hospital, Department of Radiology, Athens (Greece); Andreakos, E.; Kontogeorgos, G.

    2006-01-01

    Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. (orig.)

  1. Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study

    Directory of Open Access Journals (Sweden)

    Narula G

    2007-01-01

    Full Text Available Context: Langerhans cell histiocytosis (LCH is a rare atypical cellular disorder characterized by clonal proliferation of Langerhans cells leading to myriad clinical presentations and highly variable outcomes. There is a paucity of Indian studies on this subject. Aim: To present the experience of management of LCH at a single institution. Settings and Design: This is a retrospective observational study of patients with LCH who presented at the Tata Memorial Hospital between January 1987 and December 2002. Materials and Methods: Fifty-two patients with LCH were treated in the study period. Due to the long observation period and variability in diagnostic and therapeutic protocols, the patients were risk-stratified based on present criteria. The disease pattern, management approaches and treatment outcomes of patients were recorded. Statistical Analysis Used: Statistical analyses were done using Student′s ′t′ test, test for proportion and survival estimates based on the Kaplan-Meier method. Results: The median age at presentation was 3 years and more than 48% of the patients had Group I disease. Skeleton, skin and lymphoreticular system were the commonly involved organs. Majority (80% required some form of therapy. The projected overall survival is 63% at 10 years and mean survival is 118 months. Seventeen percent of surviving patients developed long-term sequelae. Conclusions: The clinico-biologic profile of LCH patients in India is largely similar to international patterns except a higher incidence of lymphoreticular involvement. Majority of the patients respond favorably to therapy and have a good outcome, except a subset of Group I patients who warrant enrolment in clinical trials with innovative therapeutic strategies to improve outcome.

  2. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

    International Nuclear Information System (INIS)

    Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. (orig.)

  3. Tertiary lymphoid structures are confined to patients presenting with unifocal Langerhans Cell Histiocytosis.

    Science.gov (United States)

    Quispel, Willemijn T; Steenwijk, Eline C; van Unen, Vincent; Santos, Susy J; Koens, Lianne; Mebius, Reina; Egeler, R Maarten; van Halteren, Astrid G S

    2016-08-01

    Langerhans cell histiocytosis (LCH) is a neoplastic myeloid disorder with a thus far poorly understood immune component. Tertiary lymphoid structures (TLS) are lymph node-like entities which create an immune-promoting microenvironment at tumor sites. We analyzed the presence and clinical relevance of TLS in n = 104 H&E-stained, therapy-naive LCH lesions of non-lymphoid origin and applied immunohistochemistry to a smaller series. Lymphoid-follicular aggregates were detected in 34/104 (33%) lesions. In line with the lymphocyte recruitment capacity of MECA-79(+) high endothelial venules (HEVs), MECA-79(+)-expressing-LCH lesions (37/77, 48%) contained the most CD3(+) T-lymphocytes (p = 0.003). TLS were identified in 8/15 lesions and contained T-and B-lymphocytes, Follicular Dendritic Cells (FDC), HEVs and the chemokines CXCL13 and CCL21 representing key cellular components and TLS-inducing factors in conventional lymph nodes (LN). Lymphoid-follicular aggregates were most frequently detected in patients presenting with unifocal LCH (24/70, 34%) as compared to patients with poly-ostotic or multi-system LCH (7/30, 23%, p = 0.03). In addition, patients with lymphoid-follicular aggregates-containing lesions had the lowest risk to develop new LCH lesions (p = 0.04). The identification of various stages of TLS formation within LCH lesions may indicate a key role for the immune system in controlling aberrant histiocytes which arise in peripheral tissues.

  4. Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis

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    Gelfand, Michael J.; Sharp, Susan E. [Cincinnati Children' s Hospital Medical Center, Section of Nuclear Medicine, Department of Radiology, Cincinnati, OH (United States); Palumbo, Joseph S. [Cincinnati Children' s Hospital Medical Center, Cancer and Blood Diseases Institute, Department of Pediatrics, Cincinnati, OH (United States)

    2014-08-23

    In Langerhans cell histiocytosis (LCH), FDG PET demonstrates active disease in bone. Other imaging modalities show the effects of bone destruction by LCH. To evaluate a selective CT method for reducing effective dose from FDG PET/CT in LCH, using whole-body modified attenuation correction CT at extremely low exposure settings, with repeat selective limited-volume CT at typical localization settings. Fifty-one PET/CT scans were performed in 23 LCH patients, median patient age 8.5 years (range: 1-25 years). Thirty-four were performed with modified attenuation correction CT settings, with bed positions (excluding head and neck) repeated at localization CT settings in regions with abnormal or difficult to interpret PET findings. Of 34 modified attenuation correction PET/CT scans, 10 required repeat localization CT of 1 to 3 bed positions (total: 17 bed positions). Lytic bone lesions were easily recognized at modified attenuation correction settings. Calculated average effective dose for the 34 whole-body CT scans at modified attenuation correction settings was 1.65 mSv. Average effective dose per patient for repeat imaging of 17 bed positions at localization settings was 1.19 mSv. Average total effective dose from CT for all 34 scans performed at the modified attenuation correction CT settings, including the 10 repeat localization CT scans, was 2.0 mSv. High-quality PET scans were consistently obtained with reduced FDG-administered activities of 3.7 MBq/kg (0.10 mCi/kg). In active LCH, abnormal FDG uptake was seen in all lytic bone lesions ≥9 mm, including cranial vault lesions. Substantial reduction in effective dose is possible using selective CT techniques for FDG PET/CT. (orig.)

  5. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    Science.gov (United States)

    Porto, Luciana; Schöning, Stefan; Hattingen, Elke; Sörensen, Jan; Jurcoane, Alina; Lehrnbecher, Thomas

    2015-01-01

    Background The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. Methods We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Results Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. Conclusions The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials. PMID:26401129

  6. A NEW LOOK AT LANGERHANS CELL HISTIOCYTOSIS: REVIEW OF A SERIES OF 55 CASES

    Institute of Scientific and Technical Information of China (English)

    LIU Yue-ping; ZHU Yun-ping; QU Yuan; GAO Li; LIU Xin-fan; XU Guo-zhen; LI Ye-xiong

    2005-01-01

    Objective: Langerhans cell histiocytosis (LCH) has been well described only in children. We analyzed the characteristics, reactivation, and outcome of LCH in a cohort of 55 patients across all ages. Methods: We reviewed the records of all patients with LCH treated at a single institute between Jan. 1974 and May 1998. Results: The 55 patients were 2 to 67 years of age (median, 31 years) at the time of diagnosis, and 85.5% were male. Forty patients (72.7%) had single-system LCH; Fifteen (27.3%) had multisystem disease. The head and neck was the most frequent tumor site (63.6%). LCH was not found in organs at risk of involvement (liver, spleen, bone marrow, and lungs). The frequency of bony invasion (23.6% overall) differed significantly according to age (15 years (66.7%) vs. Age >15 years (11.6%) (P=0.0005). At a median follow-up of 12 years, no patient died of LCH. The 5, 10-year survival estimates were 100%. The 5, 10-year disease-free survival estimates were 70.9% and 58.4%. The 5-year disease-free survival estimate was 58.3% for age ( 15 years vs. 74.4% for age >15 years (P=0.83) and 75% for single-system disease vs. 60% for multisystem disease (P=0.13). LCH was reactivated in 43.6% of patients, with a median of 14 months (range, 2-180 months). Three patients with recurrent disease experienced spontaneous remission. At the time of the most recent follow-up, 23.6% of survivors had active disease. Conclusion: LCH is not found exclusively in children and adolescents. The frequency of bone invasion is inversely related to age. Reactivation is very common regardless of the type of treatment, but the prognosis is generally good.

  7. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    International Nuclear Information System (INIS)

    The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials

  8. Selective CT for PET/CT: dose reduction in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    In Langerhans cell histiocytosis (LCH), FDG PET demonstrates active disease in bone. Other imaging modalities show the effects of bone destruction by LCH. To evaluate a selective CT method for reducing effective dose from FDG PET/CT in LCH, using whole-body modified attenuation correction CT at extremely low exposure settings, with repeat selective limited-volume CT at typical localization settings. Fifty-one PET/CT scans were performed in 23 LCH patients, median patient age 8.5 years (range: 1-25 years). Thirty-four were performed with modified attenuation correction CT settings, with bed positions (excluding head and neck) repeated at localization CT settings in regions with abnormal or difficult to interpret PET findings. Of 34 modified attenuation correction PET/CT scans, 10 required repeat localization CT of 1 to 3 bed positions (total: 17 bed positions). Lytic bone lesions were easily recognized at modified attenuation correction settings. Calculated average effective dose for the 34 whole-body CT scans at modified attenuation correction settings was 1.65 mSv. Average effective dose per patient for repeat imaging of 17 bed positions at localization settings was 1.19 mSv. Average total effective dose from CT for all 34 scans performed at the modified attenuation correction CT settings, including the 10 repeat localization CT scans, was 2.0 mSv. High-quality PET scans were consistently obtained with reduced FDG-administered activities of 3.7 MBq/kg (0.10 mCi/kg). In active LCH, abnormal FDG uptake was seen in all lytic bone lesions ≥9 mm, including cranial vault lesions. Substantial reduction in effective dose is possible using selective CT techniques for FDG PET/CT. (orig.)

  9. The value of bone scintigraphy in diagnosing of langerhans cells histiocytosis

    International Nuclear Information System (INIS)

    Purpose: To investigate the clinical value of bone scintigraphy in the diagnosis of Langerhans cells histiocytosis (LCH). Methods: Whole-body bone scintigraphy was performed in 23 patients, 16 males and 7 females, average age was 4.5+1.4, with histologic diagnosis of LCH. The clinical presentation varied from local mass (10 cases), tenderness (12 cases), othemorrhea (1 case), limb fatigue (3 cases), restriction of spinal activity (2 cases) to irregular fever, cough and rash (4 cases). 99Tcm-MDP was injected intravenously , the dose modified as age, according to the formula of [ adult dosage x [(age+l)/(age+7)] ] , and sedation was given to the young children who could not cooperate well. After 3 hours of administration whole-body bone scan was performed. Images were acquired using GE Millennium SPR, with a high-resolution low-energy collimator, 8 min/m sweep speed, matrix 1024 x 256, zoom 1. Results: 19 of 23 patients (82.6%) showed positive in their bone scan. 8 cases with cranial abnormal uptake, some of them exhibited peripheral high and central low uptake; clavicle, rib and pelvis involvements were 3 respectively; 2 cases in upper limb, 4 in lower limb, most lesions of limb were located in the diaphysis; spinal abnormal uptakes were seen in 5 patients, and 1 child had photon deficient area in the lower of sternium. Among 19 positive scintigram, single bone lesion was shown in 9 patients (47.3%), the most common lesion located in cranium (5 cases). Conclusion: Some characters of bone scintigraphy in LCH are useful in the diagnosis of this disease, and accompanied with comprehensive sensation of clinical manifestation of LCH, the diagnostic accuracy will be promoted. (authors)

  10. Langerhans cell histiocytosis in children: from the bench to bedside for an updated therapy.

    Science.gov (United States)

    Aricò, Maurizio

    2016-06-01

    Langerhans cell histiocytosis (LCH) is a rare disease, affecting subjects of any age, with extremely variable clinical manifestations. Although most patients with LCH have localized disease, requiring local or even no therapy, those patients with disseminated, 'multi-system' disease require specific therapy because they may be at risk for morbidity or even mortality. The current standard of care has developed empirically, based mainly on the experience of treating children with leukaemia and other haemo-proliferative disorders. At the time of writing, the combined use of vinblastine and prednisone remains the standard of care for children with multi-system LCH. The combination of cytarabine and cladribine is the current standard for second-line therapy of refractory cases with vital organ dysfunction. Recent advances in the knowledge of the pathogenesis of LCH may support a change in treatment strategy. Evidence of mutations that aberrantly activate RAF/MEK/ERK signalling in over two thirds of patients with LCH may direct a target therapy strategy. Vemurafenib, a small molecule widely used in the treatment of melanoma, is the main candidate for testing in prospective trials for patients with evidence of BRAF(V) (600E) mutation on lesional tissue. Additional molecules, including the recently approved trametinib, could follow. Identification of mutations in other genes in the remaining multisystem LCH cases could contribute to define a scenario in which target therapy becomes the main therapeutic choice in this intriguing disorder. However, because the long-term risks and benefits of these agents in children are unknown, and other effective treatments exist for many LCH patients, the optimal indications for administering a tyrosine kinase inhibitor to children is an open question. PMID:26913480

  11. Langerhans cell histiocytosis with multifocal sites. A case report; Histiocitose de celulas de Langerhans com acometimento multifocal. Relato de um caso

    Energy Technology Data Exchange (ETDEWEB)

    Albuquerque, Silvio Cavalcanti; Silveira, Andre; Ribeiro Neto, Joao Vicente [Instituto Materno Infantil de Pernambuco (IMIP), Recife (Brazil)

    1998-05-01

    The authors report a case of Langerhans cell histiocytosis in which the patient, at the time of the diagnosis, had lesions at the central nervous system, mediastinum and long bones, all detectable in simple roentgenogram. The recent reclassification of this disease is discussed together with it`s radiological findings. (author) 4 refs., 6 figs.

  12. Multifocal Langerhans cell histiocytosis in an adult with a pathological fracture of the mandible and spontaneous malunion: A case report

    Science.gov (United States)

    SHI, SAILANG; LIU, YANMING; FU, TAO; LI, XIUZHEN; ZHAO, SHIFANG

    2014-01-01

    Langerhans cell histiocytosis (LCH) is rare in the adult population and even rarer with jaw involvement. The current study presents the case of a 39-year-old male who complained of recurrent pain, swelling of the gingiva and an occasional pus-like discharge in the right mandible for one year. The patient was previously prescribed antibiotics, but this did not resolve the problem. An initial panoramic radiograph showed an osteolytic lesion and bone fracture in the right mandible. Eight months later, a new radiograph showed the spontaneous malunion of the fractured mandible. The patient was eventually diagnosed with Langerhans cell histiocytosis by histopathology and immunohistochemistry. Further lesions were found in the ribs and ilium by nuclear bone scanning. The patient was subsequently treated with systemic chemotherapy, and the lesions are currently effectively being controlled. This study is the first to show that spontaneous intralesional bone regeneration may lead to reunification of the mandible fracture caused by LCH in an adult. PMID:25120660

  13. Multifocal Langerhans cell histiocytosis of bone: Indications for radiotherapy; Histiocytose langerhansienne osseuse multifocale: place de la radiotherapie

    Energy Technology Data Exchange (ETDEWEB)

    Gaundong Mbethe, G.L.; Dejean, C.; Henriques de Figueiredo, B.; Sargos, P.; Italiano, A.; Kantor, G. [Service de radiotherapie, centre regional de lutte contre le cancer, institut Bergonie, 33 - Bordeaux (France)

    2010-12-15

    Langerhans cell histiocytosis is a non-malignant proliferative disease of unknown etiology that can affect one or more organs. This is a rare disease, 1 to 2/100, 000, affecting mainly children with a male predominance. The osseous lesions are the most frequent (60 to 90%). There is however no consensus treatment for the management of these sites. We report the cases of two patients successfully treated with radiotherapy after primary chemotherapy, at doses of 15 Gy in ten sessions of 1.5 Gy for one patient and 18 Gy in ten fractions of 1.8 Gy for the other. Single or multifocal bone Langerhans cell histiocytosis without visceral involvement is a benign, self-limiting affection in most cases. Some bone lesions could be treated by radiotherapy alone. But the high variability of doses currently given in the literature does not allow determining the lowest effective dose limiting the risk of secondary neoplasia or impaired growth in children, in whom lower doses of 6 to 8 Gy are recommended. The decision of radiotherapy must be weighed against the risk of the disease. Caution should be the rule in this non-malignant tumour pathology. (authors)

  14. Isolated Langerhans Histiocytosis in Thyroid: Thyroidectomy or Chemotherapy?

    OpenAIRE

    Attakkil, Anoop; Thorawade, Vandana; Jagade, Mohan; Kar, Rajesh; Parelkar, Kartik

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disorder of mononuclear phagocytic system whose clinical presentation varies from the localised involvement of a single bone to a widely disseminated disease. Langerhans cell histiocytosis rarely involves the thyroid gland and isolated involvement of thyroid is even rarer. We report a case of an eight-year-old male child diagnosed with Langerhans cell histiocytosis limited to thyroid gland with review of literature. Should thyroidectomy be done or...

  15. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female

    Science.gov (United States)

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  16. A case of invasive Langerhans cell histiocytosis localizing only in the lung and diagnosed as pneumothorax in an adolescent female.

    Science.gov (United States)

    Dejima, Hitoshi; Morita, Shigeki; Takahashi, Yusuke; Matsutani, Noriyuki; Iinuma, Hisae; Kondo, Fukuo; Kawamura, Masafumi

    2015-01-01

    In infants, Langerhans cell histiocytosis (LCH) is associated with poor clinical outcomes as Langerhans cells invade and damage multiple organs, a presentation that is different from that in adults. Here, we present a case of a 15-year-old female who visited ourclinic complaining of right chest pain and dyspnea. She was diagnosed with right pneumothorax by chest X-ray. Chest computed tomography showed multiple cystic changes in the bilateral lung. Additionally, bullous lesions occupying the upper lobe and multiple white tiny nodules on the surface of the lung were observed by thoracoscopy. These nodules comprised proliferating atypical CD1a/S-100-positive cells invading the pulmonary parenchyma, leading to the diagnosis of LCH. Because of the extensive invasion into the pulmonary parenchyma, chemotherapy was administered. This case of LCH was unique in that the age of onset was atypical and the tumor cells occupied a single organ, despite their malignant behavior. PMID:26045867

  17. Coincident expression of the chemokine receptors CCR6 and CCR7 by pathologic Langerhans cells in Langerhans cell histiocytosis.

    Science.gov (United States)

    Fleming, Mark D; Pinkus, Jack L; Fournier, Marcia V; Alexander, Sarah W; Tam, Carmen; Loda, Massimo; Sallan, Stephen E; Nichols, Kim E; Carpentieri, David F; Pinkus, Geraldine S; Rollins, Barrett J

    2003-04-01

    It has been suggested that a switch in chemokine receptor expression underlies Langerhans cell migration from skin to lymphoid tissue. Activated cells are thought to down-regulate CCR6, whose ligand macrophage inflammatory protein-3 alpha (MIP-3 alpha)/CCL20 is expressed in skin, and up-regulate CCR7, whose ligands are in lymphoid tissues. In Langerhans cell histiocytosis (LCH), pathologic Langerhans cells (LCs) accumulate in several tissues, including skin, bone, and lymphoid organs. We have examined 24 LCH cases and find that pathologic LCs expressed CCR6 and CCR7 coincidentally in all cases. Furthermore, MIP-3 alpha/CCL20 is expressed by keratinocytes in involved skin and by macrophages and osteoblasts in involved bone. Expression of CCR6 by pathologic LCs may contribute to their accumulation in nonlymphoid organs such as skin and bone, whereas CCR7 expression may direct them to lymphoid tissue. Histiocytes in Rosai-Dorfman disease and hemophagocytic syndrome also coexpressed CCR6 and CCR7, suggesting that this may be a general attribute of abnormal histiocytes.

  18. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report

    Science.gov (United States)

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign. PMID:27065773

  19. Localized Langerhans cell histiocytosis masquerading as Brodie's abscess in a 2-year-old child: a case report.

    Science.gov (United States)

    Chang, Wei-Fang; Hsu, Yi-Chih; Wu, Yi-Der; Kuo, Chun-Lang; Huang, Guo-Shu

    2016-01-01

    Langerhans cell histiocytosis (LCH), formerly known as histiocytosis X, refers to a spectrum of diseases characterized by idiopathic proliferation of histiocytes that produce either focal (localized LCH) or systemic manifestations (Hand-Schüller-Christian disease and Letterer-Siwe disease). Localized LCH accounts for approximately 60-70 % of all LCH cases. Osseous involvement is the most common manifestation and typically involves the flat bones, along with lesions of the skull, pelvis, and ribs. Localized LCH in bone shows a wide spectrum of clinical manifestations and radiologic features that may mimic those of infections as well as benign and malignant tumors. The diagnostic imaging findings of localized LCH are also diverse and challenging. The penumbra sign is a common and characteristic magnetic resonance imaging (MRI) feature of Brodie's abscess, but is rarely seen in localized LCH. In this report, we describe a case of localized LCH misdiagnosed as Brodie's abscess in a 2-year-old child based on clinical symptoms, laboratory findings, and pre-diagnostic MRI findings (penumbra sign). Therefore, the penumbra sign is not sufficient to clearly establish the diagnosis of Brodie's abscess, and the differential diagnosis of localized LCH should be considered when a child with an osteolytic lesion presents with a penumbra sign.

  20. Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society.

    Science.gov (United States)

    Aricò, M; Girschikofsky, M; Généreau, T; Klersy, C; McClain, K; Grois, N; Emile, J-F; Lukina, E; De Juli, E; Danesino, C

    2003-11-01

    Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year

  1. A rare case of solitary brain Langerhans cell histiocytosis with intratumoral hemorrhage in a patient affected by Turner syndrome

    Science.gov (United States)

    Granata, Francesca; Morabito, Rosa; Grasso, Giovanni; Alafaci, Elisabetta; Salpietro, Francesco M.; Alafaci, Concetta

    2016-01-01

    Background: Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of cells with characteristics similar to bone marrow-derived Langerhans cells. The case of a young woman, affected by Turner syndrome and a solitary intraparenchymal LCH associated with an osteolytic lesion of the overlying skull, is presented. Case Description: The patient, with an insidious history of headache and a growing soft mass in the left frontal region, presented with a sudden generalized tonic-clonic epileptic seizure. Neuroradiological investigations showed an osteolytic lesion of the left frontal bone and an underlying brain lesion associated with recent signs of bleeding. The patient was operated on with a complete removal of the lesion. The postoperative course was uneventful. Conclusions: The clinical, neuroradiological, and intraoperative findings are presented, along with a review of the literature. Although rare, LCH should be considered in the differential diagnosis when a scalp lesion occurs with a progressive growing. PMID:27127696

  2. Eosinophilic granuloma of bone and biochemical demonstration of 49-kDa CD1a molecule expression by Langerhans-cell histiocytosis.

    Science.gov (United States)

    Cambazard, F; Dezutter-Dambuyant, C; Staquet, M J; Schmitt, D; Thivolet, J

    1991-09-01

    Histiocytic cells infiltrating the lesions in eosinophilic granuloma of bone as well as in cutaneous histiocytosis X were studied using a murine monoclonal antibody (MA) produced with proliferating cells from an eosinophilic granuloma of bone. This MA reacts with Langerhans cells (LC) of normal human skin or mucous membranes and with proliferating cells of eosinophilic granuloma of bone and skin lesions of Letter-Siwe disease, as shown by immunohistochemistry and immunogold labelling. As other murine MA's obtained after immunization with human cortical thymocytes, this MA immunoprecipitates the 49-kDa CD1a antigen found on human LC and thymic-cell surfaces but not its breakdown product after treatment with trypsin, as demonstrated by analysis of immunoelectron labelling, cytofluorometry and gel electrophoresis. This first production of a CD1a MA from an eosinophilic granuloma supports the concept of Langerhans-cell histiocytosis.

  3. Langerhans cell histiocytosis: retrospective evaluation of 123 patients at a single institution.

    Science.gov (United States)

    Braier, J; Chantada, G; Rosso, D; Bernaldez, P; Amaral, D; Latella, A; Balancini, B; Masautis, A; Goldberg, J

    1999-01-01

    The aim of this study was to retrospectively evaluate clinical characteristics at diagnosis and outcome of patients with Langerhans cell histiocytosis (LCH). From October 1987 to March 1996, 133 patients with confirmed LCH were admitted to Hospital JP Garrahan in Buenos Aires (123 evaluable). Median age was 5 years (range 15 days to 18 years). Initial organ involvement included bone 114 patients, ear 34, skin 30, liver 18, lung 14, lymph nodes 14, spleen 12, diabetes insipidus 9, and bone marrow 2. Nineteen patients had organ dysfunction, pulmonary 14, hematological 14, and hepatic 12. Two groups were defined: Group A included patients with single system disease (uni- or multifocal) and group B multisystem (with or without organ dysfunction). In group A (n = 82), 24 patients were treated with chemotherapy (prednisone and vinblastine), 21 with surgery, 15 received radiotherapy, and 22 were only observed. Patients of group B (n = 41) were treated with chemotherapy consisting of prednisone and vinblastine, DALHX 83, or LCH1-based chemotherapy. At a median follow-up of 3 years (range 1 month-8 5/12 years) 93% of patients of group A and 39% of group B survive free of reactivation. In group B, 22% had a reactivation and 39% died of progressive disease. Sequelae were detected in 35 patients (28%), which included diabetes insipidus in 17, hearing loss in 13, bony sequelae in 11, sclerosing cholangitis in 6, and lung fibrosis with bullae in 6. Two patients had a subsequent malignant disease. A total of 17 (14%) patients died and 16 of them belonged to the group B: 13 died of progressive disease, 2 due to sclerosing cholangitis (with sepsis in one case and encephalitis in the other one), 1 with progressive disease and associated myelofibrosis, and 1 patient of group A with active disease and brain stem tumor. Patients who had organ dysfunction had a reactivation free survival of 32%. All these patients survived with sequelae. Logistic regression analysis showed that organ

  4. Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

    Science.gov (United States)

    Tono, Hisayuki; Fujimura, Taku; Kakizaki, Aya; Furudate, Sadanori; Ishibashi, Masaya; Aiba, Setsuya

    2015-01-01

    Langerhans cell histiocytosis (LCH) is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH. PMID:26500535

  5. Adult Onset of BRAFV600E-Mutated Langerhans Cell Histiocytosis with Cutaneous Involvement Successfully Diagnosed by Immunohistochemical Staining

    Directory of Open Access Journals (Sweden)

    Hisayuki Tono

    2015-09-01

    Full Text Available Langerhans cell histiocytosis (LCH is characterized by the clonal proliferation of Langerhans cells; it is categorized as a single-system disease with single or multifocal lesions, and as a multi-system disease with or without the risk of organ involvement. Although the skin is not categorized as a risk organ, the precise diagnosis of skin lesions is necessary to determine the protocol for the treatment of LCH. In this report, we describe a 28-year-old Japanese man with adult onset of BRAFV600E-mutated LCH with cutaneous involvement successfully diagnosed by immunohistochemical staining. Our report suggests that immunohistochemical staining for the BRAFV600E gene could be a diagnostic tool to determine the clinical type of LCH.

  6. Langerhans' cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult.

    Science.gov (United States)

    Tyagi, Devendra K; Balasubramaniam, Srikant; Savant, Hemant V

    2011-07-01

    Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  7. Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

    Directory of Open Access Journals (Sweden)

    Devendra K Tyagi

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  8. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease and diffuse large B-cell lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Moore James C

    2008-03-01

    Full Text Available Abstract Introduction Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease, is a rare and benign source of lymphadenopathy first described in 1969, which mimics neoplastic processes. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy or extranodal lesions consisting of tissue infiltrates composed of a polyclonal population of histiocytes. Since its description greater than 400 cases have been described, sometimes in patients with a variety of treated and untreated neoplastic diseases. However, the literature contains reports of only 19 cases of Rosai-Dorfman Disease in association with lymphomas, Hodgkin's or non-Hodgkin's. The majority of these cases have the two diagnoses, malignant lymphoma and Rosai-Dorfman Disease, separated in time. Interestingly, infradiaphragmatic lymphadenopathy was a feature in the majority of previously reported cases of Rosai-Dorfman Disease and non-Hodgkin's lymphoma. Case presentation This report provides details of a case with co-existing sinus histiocytosis with massive lymphadenopathy and diffuse large B cell non-Hodgkin's lymphoma. This case is the fifth described case of simultaneous Rosai-Dorfman Disease and concurrent non-Hodgkin's lymphoma. Unfortunately, the diagnosis of a clinically aggressive diffuse large B cell lymphoma was made at autopsy. The aggressive biological behavior of the diffuse large B cell lymphoma in this patient may have been related to the underlying immune dysregulation believed to be part of the pathophysiology of Rosai-Dorfman Disease. Conclusion Taken together this report and the preceding reports of Rosai-Dorfman Disease and non-Hodgkin's lymphoma suggests that in cases with a diagnosis of Rosai-Dorfman Disease in the setting of prominent infradiaphragmatic lymphadenopathy, clinicians should maintain a high index of suspicion for the presence of occult non-Hodgkin's lymphoma especially if the

  9. Spontaneous extradural hemorrhage due to Langerhans cell histiocytosis of the skull in a child: A rare presentation

    Science.gov (United States)

    Bakhaidar, Mohamad G.; Alghamdi, Fahad A.; Baeesa, Saleh S.

    2016-01-01

    Eosinophilic granuloma (EG) represents a local form of Langerhans cell histiocytosis that occurs mostly in children. It usually presents with a gradually enlarging painless skull mass, and rarely presents a rapid clinical deterioration. This 7-year-old boy who was diagnosed with EG, based on a magnetic resonance imaging scan, after presenting with a painless right parietal swelling of 7-week duration. Three weeks prior his scheduled surgery, he presented to the emergency department with a 2-day history of sudden increased of the subcutaneous swelling associated with a headache, vomiting, and decreased the level of consciousness; there was no history of trauma. Brain computed tomography revealed a right parietal bone defect with large subgaleal and extradural hematoma. He underwent emergent surgical excision of the skull lesion and evacuation of the hematoma. Histopathological examination confirmed the diagnosis of EG. We aim to raise the awareness of physicians of this rare spontaneous hemorrhagic complication of EG and review the literature. PMID:27195034

  10. A Case Report: The Diagnosis and Therapeutic Evaluation for a Rare Disease of Langerhans Cell Histiocytosis Involving Thyroid

    Science.gov (United States)

    Cai, Ye-Feng; Wang, Qing-Xuan; Ni, Chun-Jue; Dong, Si-Yang; Lv, Lin; Li, Quan; Chen, En-Dong; Zhang, Xiao-Hua

    2015-01-01

    Abstract Langerhans cell histiocytosis (LCH) involving the thyroid gland is extremely rare. Currently, the diagnosis and therapeutic evaluation for LCH involving thyroid is a challenge. We reported a rare case of LCH involving thyroid, presenting as painless thyroid goiters, and successfully performed positron emission tomography/computed tomography (PET/CT) to make an accurate diagnosis and therapeutic evaluation for LCH. Although the histology or cytology is the golden standard for the diagnosis of LCH involving thyroid, the PET/CT should be keep in mind when LCH involving thyroid with inconclusive cytologic results. During the treatment of LCH, PET/CT can be performed to assess the therapeutic effect and select the most effective and reliable treatment for LCH. PMID:26554785

  11. Central diabetes insipidus as a very late relapse limited to the pituitary stalk in Langerhans cell histiocytosis.

    Science.gov (United States)

    Nakagawa, Shunsuke; Shinkoda, Yuichi; Hazeki, Daisuke; Imamura, Mari; Okamoto, Yasuhiro; Kawakami, Kiyoshi; Kawano, Yoshifumi

    2016-07-01

    Central diabetes insipidus (CDI) and relapse are frequently seen in multifocal Langerhans cell histiocytosis (LCH). We present two females with multifocal LCH who developed CDI 9 and 5 years after the initial diagnosis, respectively, as a relapse limited to the pituitary stalk. Combination chemotherapy with cytarabine reduced the mass in the pituitary stalk. Although CDI did not improve, there has been no anterior pituitary hormone deficiency (APHD), neurodegenerative disease in the central nervous system (ND-CNS) or additional relapse for 2 years after therapy. It was difficult to predict the development of CDI in these cases. CDI might develop very late in patients with multifocal LCH, and therefore strict follow-up is necessary, especially with regard to symptoms of CDI such as polydipsia and polyuria. For new-onset CDI with LCH, chemotherapy with cytarabine might be useful for preventing APHD and ND-CNS. PMID:27089406

  12. Fibrosing mediastinitis with superior vena cava obstruction as the initial presentation of Langerhans' cell histiocytosis in a young child

    International Nuclear Information System (INIS)

    We present a 2-year-old girl with an unusual presentation of Langerhans' cell histiocytosis (LCH). Five months prior to admission to our hospital, she received IV steroids for bronchial obstruction. On admission, clinical signs of SVC obstruction were evident and a mediastinal mass was evident on the chest radiograph and MRI. Biopsy revealed fibrosing mediastinitis. Five months later, osteolysis was present on a skull radiograph. Surgical biopsy of the skull lesion revealed LCH. This case is unique because it demonstrates a rare initial manifestation of LCH that has not been previously reported. Furthermore, the primary, solitary mediastinal manifestation without calcifications was histologically interpreted as fibrosing mediastinitis, and the final diagnosis of LCH was only made after identifying the skull lesion. (orig.)

  13. Whole-body MRI in children with Langerhans cell histiocytosis for the evaluation of the skeletal system

    International Nuclear Information System (INIS)

    Purpose: the usefulness of whole-body MRI (WB-MRI) for the detection of skeletal lesions in patients with Langerhans cell histiocytosis should be documented on the basis of case presentations. Materials and methods: in six patients with histologically proven Langerhans cell histiocytosis, 14 WB-MRI examinations were performed to evaluate the skeletal system within disease staging (6 primary, 8 follow-up examinations). The examinations were performed on a 1.5 Tesla, 32-channel whole-body scanner. The examination protocol consisted of T1-weighted and STIR sequences in coronal and sagittal orientation. For comparison, radiographs of the initial skeletal lesions and those that were additionally detected on WB-MRI were available. Results: in 4 patients no additional skeletal lesions were found on WB-MRI besides the initial lesion leading to the diagnosis of unifocal single system disease. In 2 patients WB-MRI was able to identify additional skeletal lesions. In a 5 1/2 year-old boy with the primary lesion located in the cervical spine, a second lesion was detected in the lumbar spine on the initial scan and in the skull and proximal femur during follow-up examination. In a 12 year-old girl with a primary lesion of the thoracic spine, WB-MRI diagnosed additional lesions in the pelvic bone and the tibia. In both patients the diagnosis of multifocal skeletal involvement led to chemotherapy. During follow-up examination, the healing response under therapy could be demonstrated. Comparison with conventional imaging showed that especially lesions located in the spine or the pelvis were not detectable on radiographs even when knowing the MR results. (orig.)

  14. 朗格汉斯细胞组织细胞增多症研究进展%RESEARCH PROGRESS OF LANGERHANS CELL HISTIOCYTOSIS

    Institute of Scientific and Technical Information of China (English)

    王海燕; 侯梅

    2011-01-01

    Langerhans cell histiocytosis is a rare histiocytic disorder, the pathogenesis of which is not clear. It has various biological behaviors and the clinical course is complex, as well as non-specific, so the diagnosis is often delayed or mistakened. We present a review of Langerhans cell histiocytosis , highlighting new insights into pathogenesis, diagnosis and management of the disease. '%朗格汉斯细胞组织细胞增多症是一种少见的组织细胞异常性疾病,其发病机制不清,生物学行为多样,临床表现相对复杂,并且缺乏特异性,极易延迟诊断及误诊.本文就朗格汉斯细胞组织细胞增多症的发病机制、诊断与治疗作一综述.

  15. Insufficiency of Bone Scintigraphy in Vertebral Lesions of Langerhans Cell Histiocytosis Compared to F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Diagnostic Computed Tomography

    Science.gov (United States)

    Koç, Zehra Pınar; Şimşek, Selçuk; Akarsu, Saadet; Balcı, Tansel Ansal; Onur, Mehmet Ruhi; Kepenek, Ferat

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy. PMID:25800594

  16. Insufficiency of Bone Scintigraphy in Vertebral Lesions of Langerhans Cell Histiocytosis Compared to F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography and Diagnostic Computed Tomography

    Directory of Open Access Journals (Sweden)

    Zehra Pınar Koç

    2015-02-01

    Full Text Available Langerhans cell histiocytosis (LCH is a benign disorder related to the histiocytes which can infiltrate bone tissue. The most effective method for demonstrating severity of this disease is PET/CT and bone scintigraphy might show bone lesions. We present a seventeen year old male patient with disseminated LCH presented with exophtalmos and having multiple vertebral lesions which were identified by F-18 FDG PET/CT scan and diagnostic CT but not in the bone scintigraphy

  17. Langerhans cell histiocytosis of the mandible in bone scintigraphy and CT

    International Nuclear Information System (INIS)

    The authors present a case of a 4-year old boy with a quickly growing tumor of the jaw. The CT examination revealed a destructive tumor in the body of the mandible involving soft tissues. A diagnosis of eosinophilic granuloma of the mandible was confirmed by a biopsy of the tumor. Skeletal scintigraphy showed areas of increased and decreased radiotracer uptake. The fusion of CT and scintigraphy images showed that the cold focus corresponds with the osteolytic area and the hot focuses are larger than the areas of osseous reconstruction shown in CT. Conclusion: In cases of histiocytosis skeletal scintigraphy and CT are complementary methods that enable one to make an assessment of the extent of the disease. (author)

  18. A Case Report of Syringocystadenoma Papilliferum Mimicking Basal Cell Carcinoma on the Face

    Directory of Open Access Journals (Sweden)

    Engin Sezer

    2012-03-01

    Full Text Available Syringocystadenoma papilliferum is clinically characterized by verrucous nodules or plaques, mainly observed on the scalp during childhood. Histopathologically, epidermal invagination showing luminal epithelial cells with decapitation secretion is diagnostic. Herein, we describe an unusual late-onset case of syringocystadenoma papilliferum mimicking basal cell carcinoma on the face.

  19. Macrophages in Langerhans cell histiocytosis are differentiated toward M2 phenotype: their possible involvement in pathological processes.

    Science.gov (United States)

    Ohnishi, Koji; Komohara, Yoshihiro; Sakashita, Naomi; Iyama, Ken-Ichi; Murayama, Toshihiko; Takeya, Motohiro

    2010-01-01

    Although numerous macrophages are found in the lesions of Langerhans cell histiocytosis (LCH), their activation phenotypes and their roles in the disease process have not been clarified. Paraffin-embedded LCH samples were examined on immunohistochemistry and it was found that CD163 can be used to distinguish infiltrated macrophages from neoplastic Langerhans cells (LC). The number of CD163-positve macrophages was positively correlated with the number of multinucleated giant cells (MGC), indicating that most MGC are derived from infiltrated macrophages. A significant number of CD163-positive macrophages were positive for interleukin (IL)-10 and phospho-signal transducer and activator of transcription-3 (pSTAT3), an IL-10-induced signal transduction molecule. This indicates that these macrophages are polarized to anti-inflammatory macrophages of M2 phenotype. Tumor-derived macrophage-colony-stimulating factor (M-CSF) was considered to responsible for inducing M2 differentiation of infiltrated macrophages. The number of CD163-positive macrophages in different cases of LCH varied, and interestingly the density of CD163-positive macrophages was inversely correlated with the Ki-67-positivity of LC. Although the underlying mechanism is not fully elucidated, macrophage-derived IL-10 was considered to be involved in the suppression of tumor cell proliferation via activation of STAT3. PMID:20055949

  20. Pulmonary langerhans cell histiocytosis in adults: high-resolution CT - pathology comparisons and evolutional changes at CT

    International Nuclear Information System (INIS)

    To compare high-resolution (HR) CT and histopathological findings and to evaluate serial CT findings in pulmonary Langerhans cell histiocytosis (PLCH). We reviewed CT of lung lesions in 27 adults (M:F = 20:7, mean age, 41 ± 12.3 years) with PLCH. After evaluating lung abnormalities including nodules, micronodules, thick-walled, thin-walled, and bizarre-shaped cysts and reticulation, observers compared CT findings obtained at lung biopsy sites with histopathological findings. The final CT was compared with the initial CT to determine disease extent changes. The most frequently observed patterns of lung abnormalities were micronodules (n = 24, 89%), thick-walled (n = 22, 82%), and thin-walled (n = 22, 82%) cysts. Even thin-walled and bizarre cysts harboured active inflammatory Langerhans cell sheets and eosinophils in their walls. In thin-walled cysts, we noted pericystic inflammatory cell infiltrations along the alveolar walls, as well as pericystic emphysema. Thin-walled or bizarre cysts demonstrated a tendency to coalesce with surrounding cysts via their cystic wall destruction. Fourteen (52%) patients showed improvement and nine (33%) showed progressing disease. More than half of patients with pulmonary PLCH show improvement at follow-up CT. Even thin-walled cysts harbour active inflammatory cells on histopathology and exhibit improvement at follow-up CT. (orig.)

  1. A case of Langerhans' cell histiocytosis associated with Hodgkin's lymphoma: Fine-needle aspiration cytologic and histopathological features.

    Science.gov (United States)

    Das, Dilip K; Sheikh, Zafar A; Alansary, Taiba A; Amir, Thasneem; Al-Rabiy, Fatma N; Junaid, Thamradeen A

    2016-02-01

    Langerhans cell histiocytosis (LCH) can be associated with a variety of malignant neoplasms, the most common being malignant lymphoma, especially Hodgkin's lymphoma (HL). In this report, we describe the fine needle aspiration (FNA) cytologic features of a case with concurrent LCH and HL in a lymph node. A 20-year-old man presented with an enlarged left upper cervical lymph node. FNA smears from the swelling revealed numerous CD1a+ and S-100+ Langerhans-type cells (LCs) along with many eosinophils, neutrophils, and lymphocytes; there were also large atypical cells with enlarged nuclei having prominent nucleoli. The cytodiagnosis was LCH and the possibility of association with or trans-differentiation into a lymphoma was suggested. The histopathological diagnosis of the excised left cervical lymph node was classical HL-nodular sclerosis type (CHL-NS) with LCH. The lacunar type Reed-Sternberg (RS) cells were positive for CD30 and CD15, and the LCs were positive for CD1a and S-100 protein. PET/CT imaging demonstrated hypermetabolic lymph nodes in neck, abdomen, thorax and pelvis as well as pulmonary nodules and a splenic mass. The patient received 13 courses of chemotherapy and two years later, the enhanced CT revealed regressive course of the disease. PMID:26608102

  2. Pulmonary langerhans cell histiocytosis in adults: high-resolution CT - pathology comparisons and evolutional changes at CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyo Jin; Lee, Ho Yun; Kim, Tae Sung [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Lee, Kyung Soo [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Samsung Medical Center, Department of Radiology, Seoul (Korea, Republic of); Johkoh, Takeshi [Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Department of Radiology, Hyoko (Japan); Tomiyama, Noriyuki [Osaka University Graduate School of Medicine, Department of Radiology, Osaka (Japan); Han, Joungho [Sungkyunkwan University School of Medicine, Department of Pathology, Samsung Medical Center, Seoul (Korea, Republic of)

    2011-07-15

    To compare high-resolution (HR) CT and histopathological findings and to evaluate serial CT findings in pulmonary Langerhans cell histiocytosis (PLCH). We reviewed CT of lung lesions in 27 adults (M:F = 20:7, mean age, 41 {+-} 12.3 years) with PLCH. After evaluating lung abnormalities including nodules, micronodules, thick-walled, thin-walled, and bizarre-shaped cysts and reticulation, observers compared CT findings obtained at lung biopsy sites with histopathological findings. The final CT was compared with the initial CT to determine disease extent changes. The most frequently observed patterns of lung abnormalities were micronodules (n = 24, 89%), thick-walled (n = 22, 82%), and thin-walled (n = 22, 82%) cysts. Even thin-walled and bizarre cysts harboured active inflammatory Langerhans cell sheets and eosinophils in their walls. In thin-walled cysts, we noted pericystic inflammatory cell infiltrations along the alveolar walls, as well as pericystic emphysema. Thin-walled or bizarre cysts demonstrated a tendency to coalesce with surrounding cysts via their cystic wall destruction. Fourteen (52%) patients showed improvement and nine (33%) showed progressing disease. More than half of patients with pulmonary PLCH show improvement at follow-up CT. Even thin-walled cysts harbour active inflammatory cells on histopathology and exhibit improvement at follow-up CT. (orig.)

  3. [Central diabetes insipidus in adult patients--the first sign of Langerhans cell histiocytosis and Erdheim-Chester disease. Three case studies and literature review].

    Science.gov (United States)

    Adam, Z; Balsíková, K; Krejcí, M; Pour, L; Stĕpánková, S; Svacina, P; Hermanová, M; Vanícek, J; Krupa, P; Stanícek, J; Koukalová, R; Neubauer, J; Krivanová, A; Mayer, J; Hájek, R

    2010-02-01

    Central diabetes insipidus with an onset in adulthood is very rare. Unlike in children, central diabetes insipidus in adults is more frequently caused by inflammatory processes and neoplastic infiltrations that do not originate from the neuronal tissue than primary neuronal tissue tumours. Rare histiocytic neoplasias (Langerhans cell histiocytosis, xanthogranulomatosis and Erdheim-Chester disease) have a specific affinity to hypothalamus and the pituitary stalk not only in paediatric patients but also when occurring in adults. We describe 3 cases of central diabetes insipidus with an onset in adulthood. Diabetes insipidus was the first sign of Langerhans cell histiocytosis in 2 patients, and it was the first sign of Erdheim-Chester disease in one patient. MR imaging showed pathological infiltration and dilated pituitary stalks in all 3 patients. PET-CT proved useful in differential diagnosis, showing further extracranial pathological changes either on the basis of significant glucose accumulation or on the basis of CT imaging. The Langerhans cell histiocytosis in the first patient has also manifested itself as an infiltration of the perianal area with intensive accumulation of fluorodeoxyglucose (FDG) - SUV 8.6 and gingival inflammation indistinguishable from parodontosis. Histology of the perianal infiltrate confirmed Langerhans cell histiocytosis. Infiltration of the pituitary stalk disappeared from the MR image after 4 cycles of 2-chlordeoxyadenosin (5 mg/m2 5 consecutive days). The PET-CT of the 2nd patient showed only borderline accumulation of FDG in the ENT area, while simultaneously performed CT imaging showed cystic restructuring of the pulmonary parenchyma and nodulations consistent with pulmonary Langerhans cell histiocytosis. Bronchoalveolar lavage identified higher number of CD1 and S100 positive elements, consistent, once again, with pulmonary LCH also affecting pituitary stalk and ear canal. The PET-CT of the third patient showed increased activity

  4. Intensified and prolonged therapy comprising cytarabine, vincristine and prednisolone improves outcome in patients with multisystem Langerhans cell histiocytosis: results of the Japan Langerhans Cell Histiocytosis Study Group-02 Protocol Study.

    Science.gov (United States)

    Morimoto, Akira; Shioda, Yoko; Imamura, Toshihiko; Kudo, Kazuko; Kawaguchi, Hiroshi; Sakashita, Kazuo; Yasui, Masahiro; Koga, Yuhki; Kobayashi, Ryoji; Ishii, Eiichi; Fujimoto, Junichiro; Horibe, Keizo; Bessho, Fumio; Tsunematsu, Yukiko; Imashuku, Shinsaku

    2016-07-01

    The JLSG-96 study reported very low mortality rates for children newly diagnosed with multifocal Langerhans cell histiocytosis (LCH). The JLSG-02 study was performed to further improve the prognosis from 2002 to 2009. The present study compared the therapeutic results of these two studies in terms of multisystem disease. All patients were treated with 6 weeks of the Induction A regimen, comprising cytarabine, vincristine and prednisolone, followed by maintenance therapy. Poor responders to Induction A were switched to Induction B. JLSG-02 has been revised from JLSG-96 in the following respects: prednisolone dosage during Induction A increased; duration of maintenance therapy extended from 24 to 48 weeks; cyclosporine introduced to Induction B for progressive disease. One hundred forty-seven children with multisystem LCH were evaluated. Of these, 84 were positive for risk of organ involvement (RO) and 63 were RO-negative. At the 6-week point, 76.2 % of RO+ and 93.7 % of RO- patients responded to Induction A. Five-year event-free survival (EFS) was 46.2 % [95 % confidence (CI), 35.5-56.9] for RO+ and 69.7 % (58.4-81.1) for RO-, which was significantly superior to that in JLSG-96 [26.8 % (13.3-40.4) and 38.9 % (16.4-61.4), respectively]. The intensified induction and prolonged maintenance regimens in JLSG-02 improved EFS in patients with multisystem LCH. PMID:27040279

  5. Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Nemoto, Kenji; Oh-Ishi, Shuji; Inui, Toshihide; Nakazawa, Mariko; Hyodo, Kentaro; Nakajima, Masayuki; Kanazawa, Jun; Miura, Yukiko; Takaku, Takio; Minami, Yuko; Hayashihara, Kenji; Saito, Takefumi; Kawabata, Yoshinori

    2016-01-01

    Pulmonary arterial hypertension (PAH) secondary to pulmonary Langerhans cell histiocytosis (PLCH) is known to be a relatively common complication and is associated with a poor prognosis. However, the optimal therapeutic approach for these cases remains to be established. A 57-year-old man visited our hospital because of a progressive dry cough. A thoracic computed tomography examination showed a combination of diffuse thick-walled cysts and reticulonodular shadows that were predominant in bilateral upper lobes of the lungs. He was diagnosed as having PLCH based on the results of video-assisted thoracoscopic lung biopsies. During a 3-year clinical course, his condition deteriorated despite smoking cessation. A systemic evaluation demonstrated precapillary PAH caused by PLCH (PAH-PLCH), and treatment with tadalafil, a phosphodiesterase-5 inhibitor, was started. During a 50-month period of treatment with tadalafil, improvements in his dyspnea, 6-min walking distance, and hemodynamics were maintained without either overt hypoxemia or pulmonary edema. We considered that tadalafil therapy may be a useful option in the treatment of patients with PAH-PLCH. PMID:27330952

  6. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis.

    Science.gov (United States)

    Abbritti, M; Mazzei, M A; Bargagli, E; Refini, R M; Penza, F; Perari, M G; Volterrani, L; Rottoli, P

    2012-08-01

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical-radiological and immunological data from 12 Caucasian patients (6M, 7 smokers and 5 ex-smokers, mean age 36±8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p<0.05) and at the end of follow-up (time 1, p<0.05) and with FEV1 values at time 0 (p<0.05) and time 1 (p<0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value. PMID:21549535

  7. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical–radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical–radiological and immunological data from 12 Caucasian patients (6 M, 7 smokers and 5 ex-smokers, mean age 36 ± 8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p < 0.05) and at the end of follow-up (time 1, p < 0.05) and with FEV1 values at time 0 (p < 0.05) and time 1 (p < 0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value.

  8. Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Abbritti, M. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Mazzei, M.A. [Dept. Human Pathology and Oncology, Section of Radiological Sciences, Siena University, Siena (Italy); Bargagli, E., E-mail: bargagli2@gmail.com [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Refini, R.M.; Penza, F.; Perari, M.G. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy); Volterrani, L. [Dept. Human Pathology and Oncology, Section of Radiological Sciences, Siena University, Siena (Italy); Rottoli, P. [Respiratory Diseases Section, Dept. Clinical Medicine and Immunological Sciences, Siena University (Italy)

    2012-08-15

    Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease. Clinical-radiological and immunological data from 12 Caucasian patients (6 M, 7 smokers and 5 ex-smokers, mean age 36 {+-} 8 years) were recorded at onset and after a follow-up period of 4 years. Application of the semi-quantitative CT score revealed a prevalently cystic pattern at onset and follow-up in the majority of the patients. Patients with a prevalently nodular pattern developed cystic lesions during follow-up. Interestingly, significant correlations were found between the extent of cystic lesions and DLCO values at onset (time 0: p < 0.05) and at the end of follow-up (time 1, p < 0.05) and with FEV1 values at time 0 (p < 0.05) and time 1 (p < 0.05). Patients with progressive functional decline were those with CT evidence of severe cystic alterations. The results suggest that high resolution CT scan of the chest is mandatory for characterizing PLCH patients at diagnosis and during follow-up. The proposed CT score of the chest showed potential prognostic value.

  9. Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature

    OpenAIRE

    Gao, Limin; Li, Huifang; LI, GANDI; Liu, Weiping; Li, Jinnan; Zhang, Wenyan

    2015-01-01

    We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck...

  10. Fluidic and air-stable supported lipid bilayer and cell-mimicking microarrays.

    Science.gov (United States)

    Deng, Yang; Wang, Yini; Holtz, Bryan; Li, Jingyi; Traaseth, Nathan; Veglia, Gianluigi; Stottrup, Benjamin J; Elde, Robert; Pei, Duanqing; Guo, Athena; Zhu, X-Y

    2008-05-14

    As drug delivery, therapy, and medical imaging are becoming increasingly cell-specific, there is a critical need for high fidelity and high-throughput screening methods for cell surface interactions. Cell membrane-mimicking surfaces, i.e., supported lipid bilayers (SLBs), are currently not sufficiently robust to meet this need. Here we describe a method of forming fluidic and air-stable SLBs through tethered and dispersed cholesterol groups incorporated into the bottom leaflet. Achieving air stability allows us to easily fabricate SLB microarrays from direct robotic spotting of vesicle solutions. We demonstrate their application as cell membrane-mimicking microarrays by reconstituting peripheral as well as integral membrane components that can be recognized by their respective targets. These demonstrations establish the viability of the fluidic and air-stable SLB platform for generating content microarrays in high throughput studies, e.g., the screening of drugs and nanomedicine targeting cell surface receptors.

  11. [Pneumothorax secondary to pulmonary histiocytosis X].

    Science.gov (United States)

    Bianchi, M; Cataldi, M

    1999-01-01

    The authors report a case of pneumothorax in a young man suffering from pulmonary histiocytosis. They take this observation as a starting point for discussing about the so-called "histiocytosis X". The term "histiocytosis" is a term for a variety of proliferative disorders of histiocytes or macrophages. There is a small cluster of conditions characterized by proliferation of a special type of histiocyte called the "Langerhans' cell". The proliferation of Langerhans' cells results from disturbances in immunoregulation. Particularly, there are three clinicopathologic entities: *Letterer-Siwe disease (acute disseminated Langerhans' cells histiocytosis), *multifocal eosinophilic granuloma or Hand-Schüller-Christian disease (multifocal Langerhans' cells histiocytosis), *unifocal eosinophilic granuloma (unifocal Langerhans' cells histiocytosis). In the past, these were collected in the not specific term of histiocytosis X. Most patients with Langerhans' cell granulomatosis have diseases limited to the lung, only a small proportion have extrapulmonary involvement or a systemic disorder. Typically, there are expanding, erosive accumulations of Langerhans' cells within the medullary cavities of bones. In this reported case, a patient, two years before, was affected by osteolytic bone lesion in the left femur. Pulmonary histiocytosis is often seen in smokers in the third and fourth decade of life. The 20% of patients are asymptomatic and found to have an abnormal chest radiograph; a lesser proportion presents with pneumothorax. Some patients develop progressive fibrotic lung disease and respiratory failure. Chest radiographs may show bilateral reticular or reticulonodular infiltrates, sometimes with cystic changes, often with sparing of the costophrenic angles, but high resolution computerized tomography scans may be virtually diagnostic based on the predilection of the cysts and nodules for the upper lung zones. Sometimes the typical Langerhans' cells may be recovered in

  12. Histiocytosis: a review focusing on neuroimaging findings

    Directory of Open Access Journals (Sweden)

    Larissa Barcessat Gabbay

    2014-07-01

    Full Text Available Objective: Histiocytosis is a systemic disease that usually affects the central nervous system. The aim of this study is to discuss the neuroimaging characteristics of Langerhans cell histiocytosis (LCH, the most common of these diseases; and the non-Langerhans cells histiocytosis (NLCH, which includes entities such as hemophagocytic syndrome, Erdheim-Chester and Rosai-Dorfman diseases. Method: Literature review and illustrative cases with pathologic confirmation. Results: In LCH, the most common findings are 1 osseous lesions in the craniofacial bones and/or skull base; 2 intracranial, extra-axial changes; 3 intra-axial parenchymal changes (white and gray matter; 4 atrophy. Among the NLCH, diagnosis usually requires correlation with clinical and laboratory criteria. The spectrum of presentation includes intraparenchymal involvement, meningeal lesions, orbits and paranasal sinus involvement. Conclusion: It is important the recognition of the most common imaging patterns, in order to include LCH and NLCH in the differential diagnosis, whenever pertinent.

  13. Breast cancer cell behaviors on staged tumorigenesis-mimicking matrices derived from tumor cells at various malignant stages

    Energy Technology Data Exchange (ETDEWEB)

    Hoshiba, Takashi [Graduate School of Science and Engineering, Yamagata University, 4-3-16 Jonan, Yonezawa, Yamagata 992-8510 (Japan); International Center for Materials Nanoarchitectonics (MANA), National Institute for Materials Science, 1-1 Namiki, Tsukuba, Ibaraki 305-0044 (Japan); Tanaka, Masaru, E-mail: tanaka@yz.yamagata-u.ac.jp [Graduate School of Science and Engineering, Yamagata University, 4-3-16 Jonan, Yonezawa, Yamagata 992-8510 (Japan)

    2013-09-20

    Highlights: •Models mimicking ECM in tumor with different malignancy were prepared. •Cancer cell proliferation was suppressed on benign tumor ECM. •Benign tumor cell proliferation was suppressed on cancerous ECM. •Chemoresistance of cancer cell was enhanced on cancerous ECM. -- Abstract: Extracellular matrix (ECM) has been focused to understand tumor progression in addition to the genetic mutation of cancer cells. Here, we prepared “staged tumorigenesis-mimicking matrices” which mimic in vivo ECM in tumor tissue at each malignant stage to understand the roles of ECM in tumor progression. Breast tumor cells, MDA-MB-231 (invasive), MCF-7 (non-invasive), and MCF-10A (benign) cells, were cultured to form their own ECM beneath the cells and formed ECM was prepared as staged tumorigenesis-mimicking matrices by decellularization treatment. Cells showed weak attachment on the matrices derived from MDA-MB-231 cancer cells. The proliferations of MDA-MB-231 and MCF-7 was promoted on the matrices derived from MDA-MB-231 cancer cells whereas MCF-10A cell proliferation was not promoted. MCF-10A cell proliferation was promoted on the matrices derived from MCF-10A cells. Chemoresistance of MDA-MB-231 cells against 5-fluorouracil increased on only matrices derived from MDA-MB-231 cells. Our results showed that the cells showed different behaviors on staged tumorigenesis-mimicking matrices according to the malignancy of cell sources for ECM preparation. Therefore, staged tumorigenesis-mimicking matrices might be a useful in vitro ECM models to investigate the roles of ECM in tumor progression.

  14. Common Acute Lymphoblastic Leukemia Ph+ Following Langerhans Cell Histiocytosis in a Multi-Malformed Child with INV (9 (p12;q13 (mat:Case Report

    Directory of Open Access Journals (Sweden)

    Pavelić,Jasminka

    2010-08-01

    Full Text Available The occurrence of Langerhans cell histiocytosis (LCH and another malignancy in the same patient is infrequent but has been recognized. The genetic changes that could be responsible for LCH and/or concomitant leukemia development are obscure. To the best of our knowledge, this is the first description of constitutional maternally derived inv (9 (p12;q13 in an LCH patient, and also of the development of common ALL Ph after LCH diagnosis and therapy. The potential significance of these findings [inv (9LCHALL Ph] and their mutual relationship are unknown. Therefore, cooperative studies of large numbers of patients are needed to identify the common risk factors, if any.

  15. Langerhans Cell Histiocytosis in an Adult with Involvement of the Calvarium, Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the Use of Intravenous Immune Globulin

    Science.gov (United States)

    Dardis, Christopher; Aung, Thandar; Shapiro, William; Fortune, John; Coons, Stephen

    2015-01-01

    We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin. PMID:25873887

  16. Langerhans Cell Histiocytosis in an Adult with Involvement of the Calvarium, Cerebral Cortex and Brainstem: Discussion of Pathophysiology and Rationale for the Use of Intravenous Immune Globulin

    Directory of Open Access Journals (Sweden)

    Christopher Dardis

    2015-02-01

    Full Text Available We report a case of Langerhans cell histiocytosis in a 64-year-old male who presented with symptoms and signs of brain involvement, including seizures and hypopituitarism. The diagnosis was confirmed with a biopsy of a lytic skull lesion. The disease affecting the bone showed no sign of progression following a short course of cladribine. Signs of temporal lobe involvement led to an additional biopsy, which showed signs of nonspecific neurodegeneration and which triggered status epilepticus. Lesions noted in the brainstem were typical for the paraneoplastic inflammation reported in this condition. These lesions improved after treatment with cladribine. They remained stable while on treatment with intravenous immune globulin.

  17. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    International Nuclear Information System (INIS)

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  18. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    Energy Technology Data Exchange (ETDEWEB)

    Samet, Jonathan [Northwestern University Feinberg School of Medicine, Department of Medical Imaging, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Weinstein, Joanna [Northwestern University Feinberg School of Medicine, Departments of Pediatrics, Division of Hematology/Oncology/Stem Cell Transplant, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Fayad, Laura M. [The Johns Hopkins University School of Medicine, The Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States)

    2016-05-15

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  19. Treatment of Children with Multiple System Langerhans Cell Histiocytosis by Japan Langerhans Cell Histiocytosis Study Group Protocol%JLSG方案治疗儿童多系统朗格汉斯细胞组织细胞增生症

    Institute of Scientific and Technical Information of China (English)

    黄俊彬; 薛红漫; 陈岩岩; 黄科

    2013-01-01

    The purpose of this study was to evaluate the efficiency of Japan Langerhans Cell Histiocytosis Study Group (JLSG) Protocol in treatment of children suffering from multiple system langerhans cell histiocytosis (MS-LCH). The clinical features, therapeutic response and prognosis of 11 children who were diagnosed and treated by JLSG in our department during October 2004 through October 2011 were analyzed. Among all 11 cases, 8 males and 3 females, the age at diagnosis was from 3 month to 6.5 years old with a median age of 3 years old. There were 10 cases of LCH with multi-system involvement (MS-LCH) and 1 case of single-system involvement (SS-LCH). Among those MS-LCH patients, 5 patients had risk organ involvement, and the other 5 patients did not develop risk organ involvement. All patients had been treated with JLSG protocol. The results showed that 4 cases achieved good response after 6-week induction treatment and the time of drug discontinuation were 5-20 months without relapse; 3 cases achieved partial response after 6-week induction treatment, among them 1 case did not relapse after discontinuation of drugs for 19 months, 1 case was still receiving maintenance treatment, 1 case abandoned induction treatment; 4 patients got no response(NR) or progressive disease after 6-week of induction treatment and were switched to salvage therapy, among them, 2 patients had stopped treatment for 2-20 months without relapse, 1 patient was still receiving maintenance treatment, one had changed to another therapy. It is concluded that the most of childhood LCH can be effectively controlled by immunochemical therapy based on the JLSG protocol. For children with LCH who has a poor response after 6-week induction treatment, LCH can still be well controlled if switched to salvage treatment.%本研究观察以JLSG(Japan Langerhans Cell Histiocytosis Study Group,JLSG)方案为基础治疗儿童多系统朗格汉斯细胞组织细胞增生症(MS-LCH)的疗效.

  20. Mimicking Retinal Development and Disease With Human Pluripotent Stem Cells.

    Science.gov (United States)

    Sinha, Divya; Phillips, Jenny; Joseph Phillips, M; Gamm, David M

    2016-04-01

    As applications of human pluripotent stem cells (hPSCs) continue to be refined and pursued, it is important to keep in mind that the strengths and weaknesses of this technology lie with its developmental origins. The remarkable capacity of differentiating hPSCs to recapitulate cell and tissue genesis has provided a model system to study stages of human development that were not previously amenable to investigation and experimentation. Furthermore, demonstration of developmentally appropriate, stepwise differentiation of hPSCs to specific cell types offers support for their authenticity and their suitability for use in disease modeling and cell replacement therapies. However, limitations to farming cells and tissues in an artificial culture environment, as well as the length of time required for most cells to mature, are some of the many issues to consider before using hPSCs to study or treat a particular disease. Given the overarching need to understand and modulate the dynamics of lineage-specific differentiation in stem cell cultures, this review will first examine the capacity of hPSCs to serve as models of retinal development. Thereafter, we will discuss efforts to model retinal disorders with hPSCs and present challenges that face investigators who aspire to use such systems to study disease pathophysiology and/or screen for therapeutics. We also refer readers to recent publications that provide additional insight and details on these rapidly evolving topics. PMID:27116663