Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seventeen cases of histiocytosis X presenting as lymphadenopathy, in which the initial diagnosis was based on lymph node biopsy, are reviewed, the characteristic histopathologic findings described, and the differential diagnosis discussed. Clinical evaluation reveals a broad spectrum of associated manifestations varying from solitary eosinophilic granuloma of lymph node to a disseminated Letterer-Siwe-like syndrome. Follow-up confirms the essentially benign nature of this disorder when defined by strict histologic criteria. The implications of these findings on the continuing controversy surrounding histiocytosis X are discussed.
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PMID:Lymphadenopathy as the initial manifestation of histiocytosis X. 39 7

Of 113 cases of sinus histiocytosis with massive lymphadenopathy, 13 patients had ophthalmic infiltrates. Eleven of the 13 had infiltrates in the orbital soft tissues, and five of these patients also had eyelid disease. One patient had infiltrates only within the eyelid, and one without disease in the orbit or eyelid had extensive infiltrates in the uveal tract. The microscopic differential diagnosis included a variety of lymphoreticular malignancies, storage diseases, histiocytosis X, rhinoscleroma, tuberculosis, and inflammatory pseudotumor of the orbit. These 13 patients with ophthalmic disease were similar clinically and pathologically to patients with sinus histiocytosis with massive lymphadenopathy who did not have ophthalmic disease.
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PMID:The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. 43 97

Ten patients with sinus histiocytosis with massive lymphadenopathy (SHML) also had cutaneous involvement. Seven of the ten were children. The skin lesions were solitary in three patients and multiple in seven. They were papular or nodular, up to 4 cm in diameter, and often had a xanthomatous appearance. Microscopically, they were constituted by a dermal infiltrate made up predominantly of histiocytes, plasma cells, and lymphocytes. Some of the histiocytes contained phagocytosed lymphocytes in their cytoplasm. The microscopic differential diagnosis includes dermatofibroma, xanthoma, Tangier disease, histiocytosis X, reticulohistiocytoma, juvenile xanthogranuloma, and leprosy.
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PMID:The cutaneous manifestations of sinus histiocytosis with massive lymphadenopathy. 62 44

The case of a two-year-old girl with generalized histiocytosis, probably induced by phenobarbital, is reported. Symptoms, including intermittent fever, systemic lymphadenopathy, maculopapular skin eruption and hepatosplenomegaly, suggested Langerhans cell histiocytosis. Laboratory examinations revealed leukocytosis with lymphocytosis and eosinophilia and a high LDH serum level, while GOT and GPT were within normal ranges. Cytological studies of lymph node and pleural effusion specimens revealed proliferation and infiltration of Langerhans cell histiocytes with eosinophilia. No histiocyte proliferation was observed in the bone marrow or skin. The clinical manifestations shown by the patient were, however, transient, and improved spontaneously after the discontinuation of phenobarbital. The case was considered to be one of phenobarbital hypersensitivity syndrome based on clinical course and laboratory findings. The mechanism and differential diagnosis of the syndrome are discussed.
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PMID:A case of hypersensitivity syndrome resembling Langerhans cell histiocytosis during phenobarbital prophylaxis for convulsion. 129 59

Using a monoclonal antibody specific to the Lewis X antigen (anti-Lex), the authors studied 103 cases of Hodgkin's disease (HD) in comparison with 57 cases of non-Hodgkin's lymphoma (NHL); three cases of granulocytic sarcoma (GS); two cases of malignant histiocytosis (MH); one case of monoblastic leukemia (ML); one case of interdigitating reticulum cell sarcoma (IRCS); six cases of histiocytosis X (HX); one case of reticulohistiocytoma (RH); 44 various reactive conditions of the lymph node (LN). Reed-Sternberg and related (R-S) cells stained selectively in 80 of 92 cases of HD (87.0%), excluding 11 cases of lymphocyte predominance type. The stain was better in B-5-fixed specimens than in formalin-fixed specimens, showing a dense deposit of reaction products at a paranuclear site and on the cell surface. The staining results were compared with those of Leu-M1 and found to be superior both qualitatively and quantitatively (detection rate of R-S cells: 87.0% versus 68.5% of Leu-M1). Granulocytes, rare epithelioid histiocytes, and some endothelial and/or erythrocytes also stained with anti-Lex. The stain had positive results in three cases of GS showing a diffuse cytoplasmic staining pattern. Of NHL, two of 29 peripheral T-cell lymphomas stained to show rare paranuclear deposits without cell surface staining. The stain had negative results in MH, ML, IRCS, HX, and RH. Of 45 reactive LN, minute subcapsular collections of Lewis X+, altered-appearing Langerhans'-like cells, were observed in all ten LN from human immunodeficiency virus (HIV)-associated persistent generalized lymphadenopathy (PGL). The stain had negative results in all other various reactive conditions of LN. In conclusion, Lewis X staining is useful as a marker for R-S cells in paraffin sections with staining results superior to those of Leu-M1. Lewis X staining also detects subcapsular clustering of altered-appearing Langerhans'-like cells in PGL, which has not been described previously and warrants additional study.
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PMID:The Lewis X antigen. A new paraffin section marker for Reed-Sternberg cells. 170 18

The histiocytoses of childhood include Langerhans' cell histiocytosis, haemophagocytic syndrome (familial and reactive), sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease), juvenile xanthogranuloma and malignant histiocytosis. These disorders show wide variation in their clinical presentation, prognosis and genetic implications. All are characterized by localized or generalized proliferation of histiocytes, but they differ in their morphology, histochemical and immunochemical staining patterns and electronmicroscopical features. On the basis of a comprehensive clinical history and critical interpretation of morphology and immunocytochemistry using an appropriately selected panel of antibodies, a diagnosis can be reached in the majority of cases.
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PMID:The histiocytoses of childhood. 139 47

The histiocytoses, whether reactive or neoplastic, can be related histologically and immunophenotypically to their normal counterparts within the histiocytic system. This system has two subsets: The dendritic (antigen-presenting) cells and the phagocytic histiocytic (antigen-processing) cells. Dermatopathic lymphadenitis and Langerhans cell histiocytosis (histiocytosis X) are reactive proliferative disorders of dendritic cells. Malignancies of dendritic cells exist, but they are very rare. Benign proliferations of phagocytic histiocytes include the hemophagocytic syndromes, both familial and reactive, as well as sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and histiocytic necrotizing lymphadenitis (Kikuchi's disease). Neoplasms of phagocytic histiocytes include acute monocytic leukemia and the very rare entities, malignant histiocytosis and true histiocytic lymphoma. The latter must be distinguished from sinusoidal, large cell anaplastic lymphomas.
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PMID:The histiocytoses: clinical presentation and differential diagnosis. 215 Mar 25

Twenty-two patients with Langerhans cell histiocytosis (LCH) were managed in the UCLA Department of Radiation Oncology from 1974 to 1987. Their median age was 17 years (range 1-42 years) and median followup 4.5 years (range 1-13 years). Fourteen patients had disease localized to a bone (13) or a soft tissue (one). Eight patients had LCH involving multiple bones (3) or soft tissues plus bones (5). Nine of 10 patients less than 19 years old had disease confined to bone(s) compared to 7 of 12 older patients. Fifty-six sites of LCH (40 bone, 16 soft tissue) were irradiated. Pediatric patients received therapy to 15 sites (14 bone, 1 soft tissue) and adults 41 sites (26 bone, 15 soft tissue). Median dose for bone lesions was 900 cGy (range 600-1,500) and for soft tissue 1,500 cGy (range 600-2,600). Local control was achieved in 46 of 56 sites (82%). Control rates for bone and soft tissue lesions were 35 of 40 (88%) and 11 of 16 (69%), respectively. Fifteen of 15 sites in pediatric patients were controlled compared to 29 of 41 (72%) in adults. Ten sites (18%) recurred 10 months to 4 years after irradiation. Recurrences were in-field and noted only in adults with involvement of multiple soft tissues plus bones. All recurrent soft tissue lesions had been treated with orthovoltage. Five patients developed new foci of LCH subsequent to irradiation. Four of these patients had disease involving soft tissues and bones. One patient with progressive LCH initially presented with a single bone lesion. The acute and chronic effects of radiation therapy were minimal. One patient suffered transient enlargement of adenopathy. An elevated serum TSH level was detected 9 years after irradiation of a cervical vertebra in another patient.
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PMID:Radiation therapy in the management of Langerhans cell histiocytosis. 230 26

We describe an infant who presented at birth with numerous haemorrhagic and encrusted skin lesions, hepatomegaly, lymphadenopathy, raised hepatic transaminases, leucopenia and thrombocytopenia. The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemistry, which demonstrated the presence of CD1, S-100 and DR positive cells in the skin infiltrate. The skin lesions resolved spontaneously after 6 weeks but recurred at 3 months and again were self involuting with resolution by 9 months. Persistent circulating T-cell abnormalities, including T-cell lymphopenia and the presence and persistence of peripheral blood CD1 + cells were noted throughout the first year of life.
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PMID:Congenital self-healing Langerhans cell histiocytosis with persistent cellular immunological abnormalities. 233 23

The physiology of the histiocyte (macrophage) in health and disease is reviewed briefly. An overview of the so-called primary malignant, pseudomalignant, and benign histiocytic disorders, excluding histiocytosis X, is presented. The malignant histiocytosis with erythrophagocytosis, the pseudomalignant histiocytic diseases (such as sinus histiocytosis with massive lymphadenopathy and regressing atypical histiocytosis), and the solitary lesions with histologic malignant and atypical storiform histiocytosis are described. Two groups of adult histiocytic diseases are reviewed; one is characterized by nonfamilial and familial histiocytic dermatoarthritis and the other by multiple widespread benign lesions, such as xanthoma disseminatum, generalized eruptive histiocytoma, nodular non-X histiocytosis, and various xanthomatous eruptions associated with paraproteinemia. Finally, multiple benign cutaneous histiocytic lesions of childhood, such as juvenile xanthogranuloma and congenital self-healing histiocytosis, are included.
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PMID:Primary histiocytic dermatoses. 299 86


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