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)

Two major subsets of histiocytic cells are recognized within the lymphoreticular system: the dendritic cells or antigen-presenting cells and the phagocytic histiocytes or antigen-processing cells. Reactive and proliferative lesions of histiocytes can be related to these functional subsets. Reactive proliferations with a major dendritic cell component include dermatopathic lymphadenitis and Langerhans cell histiocytosis. Rare neoplasms derived from dendritic cells have been described. Benign proliferative lesions of phagocytic histiocytes include sinus histiocytosis with massive lymphadenopathy, the hemophagocytic syndromes including familial erythrophagocytic lymphohistiocytosis, Kikuchi's disease, and the various granulomatous lesions of lymph nodes. Although the hemophagocytic syndromes were often interpreted in the past as a variant of malignant histiocytosis (histiocytic medullary reticulosis), the reactive nature of this process is now accepted. The inciting event is usually an infection, and it is hypothesized that the histiocytic proliferation may result from exaggerated lymphokine production in an immunocompromised host. Reactive lesions of histiocytes are much more common than their rare neoplastic counterparts. True histiocytic lymphomas must be distinguished from the more common sinusoidal large cell immunoblastic lymphomas which usually express the CD30 antigen.
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PMID:Histiocytoses of lymph nodes: biology and differential diagnosis. 306 18

Two young patients presented with generalised lymphadenopathy, otorrhoea, otitis, and rash. Over the next few years chronically discharging sinuses began to form over enlarged nodes and histological appearances were typical of histiocytosis X. In neither case were micro-organisms isolated from the lesions, and in both patients healing occurred with immunosuppressive agents. Chronic dermal sinus formation secondary to lymph node disease has never before been recorded as a manifestation of histiocytosis X. Histiocytosis X should therefore be considered in the differential diagnosis of "suppurative" lymphadenopathy so that appropriate treatment may be given without delay.
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PMID:Chronic dermal sinuses as a manifestation of histiocytosis X. 308 14

The authors report on 21 cases of "primary" xanthoma of bone. Twenty of the patients were older than 20 years old. The male-female ratio was 2:1. The presenting symptom was pain in 13 patients and neurologic symptoms in 2; in 6 patients, the lesion was an incidental finding. All but one of the lesions in this series were solitary, and the flat bones (pelvis, rib, skull) were the most frequently involved sites. Radiographically, a well-defined, sometimes expansile lytic lesion, with either a small area of surrounding reactive bone or a distinct sclerotic margin, was seen. Microscopically, foam cells, giant cells, cholesterol clefts, and fibrosis were present in varying degrees. In none of these cases was there an identifiable underlying lesion. The differential diagnosis includes Erdheim-Chester disease (a multisystemic granulomatosis) and bone involvement in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). More important is the differential diagnosis with metastatic clear cell carcinoma. Xanthoma of bone is a benign lesion, and complete or even partial removal is effective. Xanthomas may represent a "burnt-out" benign condition such as fibrous dysplasia or histiocytosis X.
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PMID:Xanthoma of bone. 314 Jun 52

A mouse monoclonal antibody (MAC 387) with specificity for monocytes and tissue histiocytes was produced by immunization of a BALB/c mouse with peripheral blood monocyte components derived by affinity chromatography of detergent-solubilized monocyte material on Sepharose 4B coupled to rabbit anti-monocyte antibodies. MAC 387 strongly stained the cytoplasm of cells of the monocyte/macrophage series on paraffin sections after controlled trypsinization of sections. The antibody showed broad reactivity for a variety of tissue histiocytes, including infiltrating and reactive histiocytes, alveolar macrophages, Kupffer cells, follicle-center macrophages, splenic red pulp macrophages, tumor-infiltrating macrophages, sinus histiocytes, epithelioid giant cells (variably), and cases of histiocytosis X and dermatopathic lymphadenopathy. Molecular weight data obtained by Western blotting, immunoprecipitation, and immunoaffinity-purification revealed that the antigen was present in different forms in the monocyte and granulocyte. In the granulocyte, free alpha (Mr 12 KD) and beta (Mr 14 KD) chains expressing the MAC 387 epitope were found together with associations of one alpha and one beta chain linked by disulfide bonds to yield a heterodimer of Mr 26 KD. In the monocyte, free alpha and beta chains are not found, but instead the heterodimer and associations of two (Mr 56 KD) and four (Mr 112 KD) heterodimers are disulfide-linked together. This new monoclonal reagent should have particular value for identification of tissue histiocytes in routine paraffin sections and particularly for demonstration of histiocytes in malignant lymphomas.
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PMID:Identification of tissue histiocytes on paraffin sections by a new monoclonal antibody. 330 45

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

A 60-year-old man with a history of diabetes insipidus presented with a left groin mass, leg edema, and retroperitoneal adenopathy. Biopsy results of the involved lymph nodes were typical of eosinophilic granuloma. The patient was treated with etoposide and prednisone and had a complete regression of his lymphadenopathy and edema. This response suggests that etoposide may be a useful agent in the management of histiocytosis X.
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PMID:Multifocal eosinophilic granuloma. Response of a patient to etoposide. 371 52

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

The classification of histiocytic proliferations in the skin is confusing, largely due to faulty use of terms such as reticulum-cell sarcoma and reticulosis. Immunohistochemical staining procedures and ultrastructural studies have made it possible to differentiate the different cells of the mononuclear phagocyte system. The proliferations discussed here were those of the mononuclear phagocyte system that are associated with characteristic skin manifestations. They include proliferations of monocytes (acute monocytic leukemia), proliferations of tissue macrophages or histiocytes (histiocytic sarcoma and its disseminated form, malignant histiocytosis; familial histiocytoses; and multicentric reticulohistiocytosis), proliferations of Langerhans' cells (histiocytosis X), and proliferations of as yet undefined cells of the mononuclear phagocyte system (sinus histiocytosis with massive lymphadenopathy). Clinical features were presented, and special emphasis was placed on incidence, form, localization, histopathology, and marker profile of the proliferating cells in the skin lesions. The close relationship of the different entities was discussed, as was the importance of immune dysfunction.
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PMID:Cutaneous histiocytic proliferations. 391 76

A case of sinus histiocytosis with massive lymphadenopathy in a boy of 13 with multiple subcutaneous tumor-like formations 0.5 to 2.5 cm in diameter is described. The general condition of the patient was not changed despite the 7-month course of the disease. Mild anemia and increased ESR were observed. Microscopically the formations presented massive "dense" infiltrates of lymphoid, plasma cells, and histiocytes-macrophages with foci of fibrosis and xanthomatosis. Since the morphological picture of sinus histiocytosis with massive lymphadenopathy may to some extent imitate the substrate of some tumor diseases of the hemopoietic and lymphoid tissues (malignant histiocytosis, histiocytosis X, lymphogranulomatosis, lymphosarcoma), their differential diagnosis is presented.
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PMID:[Sinus histiocytosis with massive lymphadenopathy]. 660 46

The present paper deals with immunohistochemical and ultrastructural study of the lymph nodes of sinus histiocytosis with massive lymphadenopathy (Rosai and Dorfman, SHML) of a 12-year-old Japanese boy. This is the fourth case in Japan. Osseous manifestation was also found in the bilateral ulnae. With hallmarks of S-100 protein and interdigitating cytoplasmic extensions, the phagocytizing histiocytes proliferating in the sinuses were considered to be derived mostly from interdigitating cells in the paracortex or T cell dependent area, which have heretofore been regarded as nonphagocytizing. Furthermore, it is most interesting that lymphoid cells bearing thymic cortical cell-antigen (OKT 6) were increasingly recognized in the patient's peripheral blood. These results suggested that SHML is a specialized reactive histiocytosis analogous to histiocytosis X and histiocytic medullary reticulosis.
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PMID:Sinus histiocytosis with massive lymphadenopathy. A histogenic analysis of histiocytes found in the fourth Japanese case. 674 50


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