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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Skin biopsies and a lymphnode of three children with infantile Histiocytosis-X (Letter-Siwe Disease) were studied with enzymehistochemical and sheep-erythrocyte rosetting techniques. The majority of cells making up the infiltrates of skin and lymphnode showed rather weak acid phosphatase and nonspecific esterase activity but considerable leucyl-beta-naphtylamidase activity. Sheep-erythrocyte rosetting techniques performed on frozen sections indicated the presence of receptors for the Fc fragment of IgG, but no receptors for C3 could be demonstrated. Cells with the same enzymehistochemical characteristics could be found in thymus-dependent areas of normal spleen, of normal and reactive lymphnodes and in thymic medulla but not in B-cell areas or thymic cortex. It is suggested that Histiocytosis-X cells belong to the Mononuclear Phagocyte System and that they are related to or identical with cells normally present in the thymus dependent areas of the lymphoid tissue involved with the functioning of cell-mediated immunity.
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PMID:Infantile histiocytosis X. 15 Mar 6

The authors report on a case of disseminated Langerhans' cell histiocytosis with a clinical presentation and a bone marrow simulating, at onset, an acute leukemia non lymphoid. A hepatic needle biopsy performed for the progressive enlargement of the liver oriented the diagnosis towards a Langerhans' cell histiocytosis. The morphological, immunohistochemical and ultrastructural study of these cells showed them to be undetermined, i.e. Langerhans' cell precursors.
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PMID:Disseminated histiocytosis with undetermined Langerhans' cells simulating an acute non lymphoid leukemia. 174 96

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.
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PMID:Urticating histiocytosis: a mast cell-rich variant of histiocytosis X. 242 66

A 48-year-old female patient was admitted because of increasing in size xanthomas of the eyelids lasting since 9 years (despite surgical treatment) and narrowing considerably the palpebral fissures. Five years earlier the diagnosis of type II b hyperlipoproteinaemia was made. Presently, besides palpebral lesions, a painless plum-sized tumour was found in the right frontoparietal area and two hard tumours of similar size were situated bilaterally in the submandibular area. In the biopsy specimens of all these tumours histological examination demonstrated lymphoid cells with numerous germinative centres. The diagnosis of histiocytosis X was followed by treatment with prednisone which resulted in a decrease in the size of palpebral xanthomas and improvement of the general condition of the patient.
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PMID:[Atypical course of histiocytosis X]. 262 63

A new B-lymphoma cell line (DEAU-cell line) was established from a diffuse large-cell lymphoma (centroblastic type) and was successfully grafted in athymic nude mice. Monoclonal antibodies (MoAbs) were generated using splenocytes of DEAU-tumor bearing mice. Before the fusion experiments, cellular immunity of the mice bearing growing DEAU tumors was restored by injection of spleen cells from conventional Balb/C mice. Spleen cells from conventional Balb/C mice immunized with DEAU-cell line were also used for the generation of MoAbs. Four MoAbs (DBB.42 and DBA.44 from normal Balb/C mice, and DNA.7 and DND.53 from athymic nude mice) were investigated because they identified B-cell-associated antigens not destroyed by fixatives. DBB.42 recognized a pan-B cell-associated antigen (molecular weight (mol wt) = 45 Kd). DBA.44 detected a B-cell antigen (mol wt not determined) expressed on a subpopulation of B lymphocytes in the mantle zone of lymphoid follicles. DNA.7 also defined a B-cell antigen (43 Kd) mainly expressed on germinal center cells. Similarly, DND.53 recognized a B-cell antigen (two bands of mol wt 20 Kd and 35 Kd, respectively) mainly expressed on germinal center cells and mantle zone lymphocytes and interdigitating reticulum cells in the paracortical area. Major differences were found in the reactivities of these MoAbs on malignant lymphomas. DBB.42 was positive with almost all B-cell lymphomas and some T-cell lymphomas. Within the group of low-grade B-cell lymphomas, DBA.44 reacted principally with hairy-cell leukemia. DNA.7 reacted mainly with high-grade B-cell lymphomas with a weak positivity in low-grade B-cell lymphomas. DND.53 reacted with all but one B-cell lymphoma, cells of histiocytosis X, and Reed-Sternberg cells. These findings indicate that new MoAbs can be generated by using spleen cells from athymic mice bearing human tumors as well as by new lymphoid cell lines. The MoAbs so generated, as in the present study, are deemed potentially useful for the recognition of B-cell lymphomas in routine diagnostic histopathology. In addition, DND.53 could be of value for the diagnosis of histiocytosis X and the detection of Reed-Sternberg cells in Hodgkin's disease.
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PMID:Production of anti-B monoclonal antibodies (DBB.42, DBA.44, DNA.7, and DND.53) reactive on paraffin-embedded tissues with a new B-lymphoma cell line grafted into athymic nude mice. 267 17

A case of a myasthenic patient with the association of unifocal histiocytosis X (eosinophilic granuloma) and lymphoid follicular hyperplasia of the thymus is presented. The combined diagnosis was made on histological grounds and supported by immunohistochemistry. Two aspects of this case are of interest: (1) it is the first reported case of the association of histiocytosis X and lymphoid follicular hyperplasia of the thymus in a myasthenic patient; and (2) the pathogenesis of this peculiar association does not seem to be fortuitous but rather might be related to the general derangement of the immune system present in myasthenia gravis.
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PMID:Histiocytosis X and lymphoid follicular hyperplasia of the thymus in myasthenia gravis. 278 37

The morphological, ultrastructural and immunophenotypic properties of Histiocytosis-X (H-X) cells were investigated in a lymph node involved by Letterer-Siwe (L-S) disease. H-X cells were T6+ (CD1a), S-100+, T4+ (CD4) and HLA-DR+; in addition they were consistently T11+ (CD2) and were stained by antibodies directed against receptors for transferrin (T9), C3bi (OKM-1/CD11b), IgG-Fc (Leu-11/CD16) and Interleukin-2 (IL-2R/CD25). On immunostained cytosmears, T6+ cells were highly polymorphic and a prominent fraction (45%) showed immature morphology, characterized by lymphoid appearance. Cells expressing macrophage markers (ANAE, AACT, Leu-M3/CD14, PAM-1) were 10-fold fewer than T6+ cells and did not show a lymphoid morphology. At TEM level, H-X cells were characterized by poor content of LC granules and by the presence of myelin-like laminated bodies and of lysosome-like dense bodies. The immunophenotypic properties of H-X cells were compared to those of epidermal Langerhans cells (LCs) and of LCs present in lymph nodes of three cases of dermatophatic lymphadenitis. Epidermal LCs were T6+/HLA-DR+, and sometimes faintly T4+. Lymph node LCs were T6+, S-100+, T4+, HLA-DR+, and showed the same variety of surface receptors detected in H-X cells; furthermore, in a case with massive infiltration of the paracortex by T6+ cells, lymph node LCs were faintly T11+ and some of the T6+ cells had lymphoid aspect. Our findings suggest that the H-X cell population of L-S disease is not homogeneous, but is composed of discrete cell subsets with distinctive antigenic and morphological traits closely resembling those of cells of LC lineage at different maturational stages.
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PMID:Letterer-Siwe disease: immunohistochemical evidence for a proliferative disorder involving immature cells of Langerhans lineage. 313 61

The morphological features of Langerhans cell histiocytosis (histiocytosis X) are characteristic but the diagnosis can on occasion be difficult. A case is presented that illustrates the diagnostic value of immunohistochemistry in the differential diagnosis of this condition. The cells of Langerhans cell histiocytosis were found to express CD1, CD4, CD11b and CD11c. They also reacted with EBM11, UCHM1, KB61 and HLA-DR. Occasional cells showed nuclear staining with Ki67, but no other lymphoid antigens were detected. Immunoreactivity of the cells of Langerhans cell histiocytosis with antibodies that recognize antigens present on macrophages provides further evidence for immunological similarities between these cell types.
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PMID:Langerhans cell histiocytosis: an unusual case illustrating the value of immunohistochemistry in diagnosis. 331 71

A 50-year-old male with a history of tonsillar and right axillary lymph node enlargement due to atypical lymphoid hyperplasia presented two years later with marked bilateral axillary and inguinal lymphadenopathy. The lymph node biopsy showed a composite lymphoma (follicular, mixed, small and large cell plus B-immunoblastic sarcoma) with associated focal Langerhans' cell granulomatosis (LCG) (Histiocytosis X). The diagnosis of composite lymphoma was supported by the immunohistochemical demonstration of two different monoclonal patterns in the follicular and diffuse areas. The typical Birbeck's granules were demonstrated ultrastructurally in LCG areas, which also stained with S-100 protein. LCG may coexist with malignant lymphoma, however, it appears to be confined to the neoplastic nodes with no tendency to systemic spread. It is important to recognize this association so that the impact of this apparently benign lesion (LCG) not be overestimated and that the subsequent management of the patient be directed according to the type of the coexisting malignant lymphoma.
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PMID:Composite lymphoma with immunoblastic features and Langerhans' cell granulomatosis (histiocytosis X). 351 49

Granulomatous lesions of the cranio-facial area are frequent and various in their nature: lymphohistiocytic with or without eosinophils, tuberculoid-like with epithelioid and giant cells, or sometimes made essentially of giant cells. Their etiology can be known or easy to find: foreign body granuloma, sarcoidosis, leprosy, rhinoscleroma, fungal diseases especially zygomycosis and rhinosporidiosis, parasitic diseases. The lethal midline granuloma is a clinical entity characterized by its necrotic and relentlessly progressive destructive presentation. After elimination of a malignant process, especially lymphoid, and of a Wegener's granulomatosis the diagnosis will be "idiopathic midline non-healing granuloma". Some of them will stay located at the facial area; others will disseminate as a malignant disease. Central giant cell granuloma and histiocytosis X, especially eosinophilic granuloma, are two other varieties of granuloma, different of the former granulomatous infiltrates by their clinical presentation and their evolution.
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PMID:[Craniofacial granulomatous lesions]. 352 24


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