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

Nine children with histiocytosis X were observed. The course of the disease largely depended upon the degree of dissemination at the time of diagnosis. Bone and skin involvement healed completely with a relatively simple therapy using prednisone in combination either with vincristine or vinblastine. Complete or long lasting partial remissions could be achieved up to involvement of 4 organs. Three patients demonstrating histiocyte infiltrations in 5 organs died. They all were less than 2 years of age. Hyperproliferation of monocytopoiesis was present in all patients manifesting itself by a rise in the fraction of mononuclear phagocytes in bone marrow. This variation was paralleled by monocytosis in the peripheral blood in 5 of 7 cases. Serum muramidase levels were increased in 5 of 6 patients. However, there was no clearcut correlation to the degree of disease dissemination.
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PMID:Histiocytosis X in 9 children: clinical aspects and laboratory evaluations including an analysis of monocytopoiesis. 31 90

Immunohistochemical study on 26 cases of Langerhans cell histiocytosis (LCH) using several leukocyte antibodies in addition to traditionally used markers (S-100 protein and peanut agglutinin) revealed that the proliferating cells of LCH expressed UCHL1, MT1 as well as classically known positivity for S-100 protein, HLA-DR and peanut agglutinin but were negative for OPD4. In comparison to S-100 protein peanut agglutinin (PNA) using a two stage method produced weaker staining and positively stained cells were sparse. Also in this study, a small proportion of proliferating cells in LCH was observed to be reactive for both myeloid/macrophage antigens (KPI, MAC 387 and lysozyme) and Langerhans cell marker (S-100 protein), verifying the existence of a hybrid form of histiocytes.
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PMID:Immunohistochemical study on antigenic phenotype of Langerhans cell histiocytosis. 128 18

Histiocytosis X (HX) is characterized morphologically by a proliferation of Langerhans' cells (LC), and most often has an indolent, chronic course. To determine whether a distinct clinicopathologic entity of malignant histiocytosis X exists, the authors examined tissues from 31 patients with HX and divided them into four categories. Group A (19 patients) was characterized morphologically by benign-appearing LC and had an indolent course. The male:female (M:F) ratio was 10:9, and the mean age was 21 years (range, 2 months to 60 years). The immunophenotype of this group was S-100+, vimentin+, LN-2+, LN-3+, lysozyme-, LCA-, Leu-M1-. Group B (three patients) had benign-appearing LC, yet had an aggressive clinical course. All patients were male, with a mean age of 47 years (range, 3 years to 72 years). Organs involved included the liver, spleen, heart, thymus, lung, kidney, and pancreas. The immunophenotype was the same as for Group A. Group C (two patients) had atypical and malignant appearing LC, yet a relatively benign clinical course. The ages were four and 65 years, with one female and one male patient. In both patients, the cells were S-100+, vimentin+, LN-2+, LN-3+, and LCA-. Group D (seven patients) was characterized by atypical and malignant-appearing LC and an aggressive clinical course. The mean age was 25 years (range, congenital to 54 years) with one female and six male patients. Organs involved were the thymus, lungs, spleen, liver, kidney, brain, heart, pancreas, stomach, and muscle. Birbeck granules were found in two patients, and the one patient on which fresh tissue was available was CD1+. The typical immunophenotype was S-100+, vimentin+, LN-2+, LN-3+, Leu-M1-, lysozyme-. The results of our study indicate that (1) a distinct clinical entity of malignant HX, characterized morphologically by malignant-appearing LC and clinically by male predominance, atypical organ involvement, and an aggressive clinical course, does exist; and (2) the morphologic appearance of the LC is an imperfect predictor of the clinical severity of HX.
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PMID:Malignant histiocytosis X. A distinct clinicopathologic entity. 191 75

A 49-year-old Japanese woman was found to have innumerable, small sessile elevations throughout the stomach as revealed by radiographic and endoscopic examination. Endoscopic biopsy showed that the elevations were formed by a diffuse proliferation of histiocytes in the lamina propria mucosae. The histiocytes were characterized by abundant interdigitating cytoplasmic projections, Birbeck granules, an oval or indented nucleus, and an absence of phagocytosis. They stained for S-100 protein but not for lysozyme, alpha-1-antitrypsin, or nonspecific antigen cross-reacting with carcinoembryonic antigen. Five and a half years after the first diagnosis, most of the elevated lesions disappeared, but a few histiocytoid cells were noted microscopically. Systemic examination showed no abnormalities outside the stomach. The patient has continued to be well. This case was diagnosed as primary benign histiocytosis X of the stomach. Four similar cases have been reported. This is the first case that was diagnosed using biopsy techniques and in which the natural history--i.e., from multiple polypoid extension throughout the stomach to spontaneous remission--was observed.
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PMID:Primary benign histiocytosis X of the stomach. A report of a case showing spontaneous remission after 5 1/2 years. 198 67

A case of localized histiocytosis X of the penis was reported. The patient was a 9-year-old boy who had several tumor lesions on the glans penis and inner layer of prepuce. Biopsy specimens were studied by H-E staining and immunohistochemical staining for S-100 protein (S-100), lysozyme (Lys), leucocyte common antigen (LCA), and Leu-M1. They revealed diffuse infiltration of many atypical histiocytes, which were shown to be S-100+, Lys+, LCA- and Leu-M1-. This indicates that these cells were derived from T-zone histiocyte system. After complete remission of these tumors, the other one arose from anal mucosa. In the literature we could find only one case of a primary penile lesion reported by Myers and others.
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PMID:[A case of histiocytosis X of the penis]. 229 25

Immunohistochemically, the presence of lysozyme (LZ) has been detected by the antibody against human LZ in cytoplasm of cells from granulomatous and histiocyte-proliferative skin diseases. To detect LZ in these cells morphologically, I have done electron microscopic observations of the following skin diseases; sarcoidosis, lupus vulgaris, lupus miliaris disseminatus faciei (LMDF), tattoo granuloma, lichen nitidus, foreign body granuloma, granuloma annulare, xanthelasma, xanthoma tuberosum, xanthoma planum, juvenile xanthogranuloma, giant cell tumor of tendon sheath, dermatofibroma, malignant fibrous histiocytoma, dermatofibrosarcoma protuberans, granulation tissue of burn, hypertrophic scar, and histiocytosis X. From both the immunohistochemical and the electron microscopic features it was concluded that a) immunohistochemically LZ-positive cells from lesions of sarcoidosis, lupus vulgaris, LMDF and tattoo granuloma had a number of electron-lucent bodies (ELB) or microvesicles in their cytoplasm, b) lichen nitidus and xanthoma tuberosum had few LZ-positive cells and the ELB were not observed, and c) the other diseases were LZ-negative, and the ELB were also absent. It is suggested that LZ is present in the ELB which are observed electron microscopically.
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PMID:[Lysozyme-positive cells and ultrastructural findings in granulomatous and histiocyte-proliferative skin diseases]. 254 57

We report a new case of histiocytic proliferation, which histologically resembles histiocytosis X, in a lymph node affected by non-Hodgkin's malignant lymphoma. This brings the total number of such reported cases to 12. Histiocytosis X cells, with folded nuclei, expressed S100 protein and an antigen recognized by anti-CD1 monoclonal antibodies. Ultrastructural study did not show any Birbeck granules and demonstrated a morphology similar to that of interdigitating cells. In the absence of Birbeck granules, the term 'Langerhans' cell granulomatosis' is not correct and should be replaced by either 'interdigitating cell granuloma' according to immunohistochemistry and ultrastructure or 'histiocytosis X-like granuloma' according to optical morphology. The fact that some cells with folded nuclei were positive for lysozyme argues in favor of the existence of transitional cells between histiocytes hnd interdigitating cells. The 11 other reported cases were reviewed. In 6 cases, this type of granuloma was associated with B cell lymphoma. In 3 cases the lymphoma was also probably of B cell type. In 2 cases, no information could be found. We could speculate that these histiocytosis X-like lesions are reactive, resulting from immune disturbances due to the lymphoma and/or the treatment.
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PMID:Lymph node interdigitating cell granuloma associated with non-Hodgkin's malignant lymphoma. A case report and review of the literature. 270 Dec 67

A case of localized histiocytosis X of the eyelid was reported. The patient was a 33 year-old man who had a tumorous lesion of the right lower eyelid that had not responded to antibiotic treatment. The lesion, accompanied with induration, slightly protruded on the conjunctival side, and the surface of the lesion was smooth and not ulcerative. No abnormal findings were noted by systemic and laboratory examination results. The patient underwent excision of the lesion and its surrounding tissue in the right lid. The specimen was fixed in formalin and embedded in paraffin. Histopathologic examination revealed diffuse infiltrates of many atypical histiocytes, which were immunohistochemically stained with antibodies to S-100 protein, lysozyme and Leu-M1. The diagnosis of localized histiocytosis X of the lid was made. It is possible that these atypical histiocytes may be classified as a category of T-zone histiocyte immunohistochemically. The postoperative course was uneventful and without recurrence.
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PMID:[A case of localized histiocytosis X of the eyelid]. 278 88

The expression of macrophage antigens KP1, Mac, lysozyme, and alpha-1-antichymotrypsin was investigated on routine paraffin sections from 17 cases of Langerhans' cell histiocytosis (LCH). All the major clinical forms were represented, including single lesions and monosystemic and multisystemic disease. In all the cases, a variable fraction (3-35%) of LCH cells was immunoreactive with KP1 and anti-Mac; the staining pattern was quite typical because the immunoreaction product was often confined to the perinuclear space and the Golgi area. LCH cells containing lysozyme and AACT were detected less frequently; however, in positive cases the percentage of LCH cells immunoreactive for lysozyme and AACT was in the same range as that of KP1-positive cells. On immunostained cytosmears (one case), about 10% of the CD1a-positive cell population was reactive for the macrophage antigens CD14 and PAM-1. No association was noted between the number of KP1-positive cells and the clinical form and/or anatomic site of the lesion. Phagocytic macrophages were significantly and diffusely immunoreactive with KP1 and anti-Mac and for AACT and lysozyme. Multinucleated giant cells with irregular nuclei were frequently observed; these cells were rarely S-100 positive, were consistently stained by KP1 and AACT, and were occasionally anti-Mac positive. The authors' findings suggest that antimacrophage monoclonals, in conjunction with S-100 protein, may represent a useful tool to establish the diagnosis of LCH in paraffin-embedded material.
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PMID:Expression of macrophage-associated antigens in tissues involved by Langerhans' cell histiocytosis (histiocytosis X). 278 88

Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. Our results suggested that xanthomatous lesions of the skin to be composed of the histiocytic proliferation of two different cell lineages, i.e. S100+lyso-NCA- T-zone histiocytes and S100-lyso+NCA+ tissue macrophages. Only lesions of histiocytosis X were composed of the former cells. It is suggested that these markers will be useful in determining the delineation of the histiocytic system on the basis of functional heterogeneity.
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PMID:Immunohistochemical study on cutaneous histioproliferative lesions. 282 48


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