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)

The antigenic properties of normal epidermal Langerhans cells and their neoplastic counterparts (histiocytosis X cells) were compared with those of other types of macrophages, by immunohistochemical staining of skin and lymph node biopsies with a panel of mono-clonal antibodies. The phenotype of Langerhans cells, histiocytosis X cells, dermal macrophages and interdigitating reticulum cells were similar (HLA-DR+, OKT 6+/-, Leu3+/-, C3-receptor-, Ig-complex-, R4/23-, MO2-), and differed markedly from those of follicular dendritic cells (HLA-DR-, OKT6-, Leu3-, C3 receptor+, Ig+, R4/23+, MO2-, and histiocytic reticulum cells (HLA-DR+/-, OKT6-, Leu3+/-, C3-receptor+, Ig-complex+, R4/23-, MO2+/-). This indicates that Langerhans cells are related to the interdigitating reticulum cells found in the T cell areas of lymphoid organs, and demonstrates that Langerhans cells do not express C3b, C3bi or C3d receptor molecules. Our data also emphasise that the Leu3 antigen is not restricted to cells of the T cell lineage.
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PMID:Normal and neoplastic Langerhans cells: phenotypic comparison with other types of macrophages. 387 94

Malignant lymphomas of the skin, excluding mycosis fungoides, are pathologically, immunologically, and clinically heterogeneous. Varying patterns and degrees of cutaneous infiltration are encountered in all histologic subtypes of non-Hodgkin's lymphomas. Immunologic studies have shown relatively equal numbers of cases with B and T cell phenotypes, but true histiocytic lymphomas of the skin also occur. Patients may be of any age, and they may have lymphoma in any clinical stage. A low clinical stage and a low-grade histologic subtype are significant factors for long survival. The differential diagnosis includes a variety of lymphoid infiltrates that are referred to as a group as cutaneous lymphoid hyperplasia; it also includes lymphomatoid papulosis as well as nonlymphoid cutaneous infiltrates such as myeloid leukemias, histiocytosis X, malignant histiocytosis, regressing atypical histiocytosis, and neuroendocrine (Merkel) cell carcinoma. Distinction of cutaneous lymphomas from these entities requires comprehension of multiple variables, including immunologic as well as morphologic and clinical factors.
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PMID:Malignant lymphomas of the skin: their differentiation from lymphoid and nonlymphoid cutaneous infiltrates that simulate lymphoma. 391 53

The immunological phenotype of the cells involved in skin and lymph node lesions from two cases of histiocytosis X (H-X) were analysed by immunofluorescence techniques using combinations of heterologous and monoclonal antisera to Ia-like antigen and human cortical thymocyte (HTA-1) determinant. These cells were also characterised by a new technique using simultaneous immunofluorescence and enzyme histochemistry for acid phosphatase (ACPase). The major cell type in the lesions was found to express the same Ia+, HTA-1+ phenotype as normal epidermal Langerhans' cells (LC) and was unreactive for ACPase. Additional cell types included Ia-, HTA-1- multinucleate giant cells and residual lymphoid populations. These findings endorse previous concepts that H-X is a proliferation of abnormal LC and emphasise the heterogeneous nature of the cells involved in the disease.
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PMID:Combined immunological and histochemical analysis of skin and lymph node lesions in histiocytosis X. 617 64

Levels of total lipids, cholesterol and triglycerides were followed in 30 children with malignant hematological diseases, or solid tumors; in addition, chylomicrons, VLDL, LDL and HDL were separated by electrophoresis on polyacrylamide gel. Nine of the 13 children with acute lymphoblastic leukaemia presented an increase of triglycerides and VLDL at time of diagnosis; all other parameters (cholesterol, HDL, LDL, chylomicrons) were normal. The lipid profile went back quickly to normal when hematological remission was obtained and remained stable throughout the course of the disease whatever the evolution might be. Five more patients in relapse were studied and did not present abnormalities. Similar results were obtained in 1/2 patients with acute myeloblastic leukaemia and in 3/5 patients with lymphoma. No lipid profile abnormality was observed in four patients with solid tumor and in one patient with histiocytosis X. The values of triglycerides and VLDL in pathological cases will be given. The endogenous nature of this disturbance is discussed as well as its relation with the rate of malignant lymphoid proliferation.
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PMID:Study of blood lipids in 30 children with a malignant hematological disease or carcinoma. 633 57

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

In this study the antigenic profile of Hodgkin (H) and Sternberg-Reed (SR) cells from cases of Hodgkin's disease was analysed using a large panel of monoclonal and polyclonal antibodies reactive with cells of lymphoid and haemotopoietic origin. The aim of this investigation was, firstly, to throw light on the origin of H and SR cells and, secondly, to determine whether there is any evidence to support recent suggestions that H and SR cells differ antigenically between different histological categories of Hodgkin's disease. Frozen sections (from 24 cases) and paraffin sections (83 cases) were stained by immunoenzymatic methods and the results compared with those obtained from staining a wide variety of reactive and neoplastic tissue samples (including examples of tuberculosis, sarcoidosis, malignant histiocytosis, histiocytosis X, osteomyelosclerosis and non-Hodgkin's lymphoma). The results revealed that H and SR cells of all types of Hodgkin's disease consistently lack markers found on null cells, B cells, T cells, cells of monocyte/macrophage series, interdigitating reticulum cells, dendritic reticulum cells and erythropoietic and thrombopoietic cells. However, H and SR cells constantly expressed an antigen detectable with the recently produced monoclonal antibody Ki-I. The vast majority of typical and lacunar type H and SR cells contained the granulocyte-related antigens detected by monoclonal antibodies TU5, TU6, TU9 and 3C4, whereas other more or less specific granulopoietic cell markers (such as peroxidase, chloroacetate esterade, lysozyme, cationic leukocyte antigen and OKMI) were consistently absent. H and SR cells in cases of nodular paragranuloma (nodular type of Hodgkin's disease with lymphocyte predominance) were not monotypic in light chain type (as has been previously reported), but rather contained chi and lambda chains within the same cells, as do typical and lacunar type H and SR cells. Immunostaining of normal and hyperplastic lymphoid tissue with the Ki-I antibody led to the detection of a new, as yet unidentified, small-cell population of unknown origin and function, which is present between, around, and within cortical follicles. It is concluded from these findings that H and SR cells constitute a unique cell type that differs in many properties from all other known cell types. Furthermore, H and SR cells of the various histological types of Hodgkin's disease are more closely related than previously believed. It is suggested that the hitherto unknown cell population detected with the monoclonal antibody Ki-I in normal lymphoid tissue is the normal equivalent of H and SR cells.
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PMID:Identification of Hodgkin and Sternberg-reed cells as a unique cell type derived from a newly-detected small-cell population. 675 30

The immunoreactivity of a CD1a monoclonal antibody (MAb), denoted 010, was investigated by means of the streptavidin-biotin-peroxidase method in formalin-fixed and paraffin-embedded tissues from 47 cases. The samples comprised reactive lymphoid proliferations of skin, tonsil, and lymph node including dermatopathic lymphadenopathy and Langerhans' cell histiocytosis, Hodgkin's and non-Hodgkin's lymphomas, and thymomas. Interdigitating and dermal dendritic cells, veiled cells, Langerhans' cells, and also cortical thymocytes and their neoplastic counterparts displayed immunostaining with MAb 010 in paraffin sections. These results are identical to previous ones reported for other CD1a MAbs in fresh or frozen specimens. The findings suggest that the binding site of 010 is a fixation-resistant epitope of CD1a antigen which has not been previously identified.
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PMID:Immunohistochemical detection of CD1A antigen in formalin-fixed and paraffin-embedded tissue sections with monoclonal antibody 010. 750 72

Kimura's disease is a chronic inflammatory disorder of unknown etiology, presenting usually as painless subcutaneous swellings in the head and neck region or in the salivary glands. The cytologic features of fine-needle aspirates of eight cases of Kimura's disease were studied with reference to the histologic appearance of the subsequent surgical specimens. In the cytologic smears, the prominent feature was the presence of significant numbers of eosinophils in a background of lymphoid cells. Fragments of collagenous tissue and Warthin-Finkeldey polykaryocytes occasionally were seen. In the cell block, vascular proliferation and fibrosis were useful features, providing further support to the diagnosis. The constellation of these features is characteristic of Kimura's disease and should suggest this diagnosis in the appropriate clinical setting. For initial diagnosis, excisional biopsy is important for the exclusion of malignant lymphoma, histiocytosis X, angiolymphoid hyperplasia with eosinophilia and other reactive lymphadenopathies. Nonetheless, fine-needle aspiration cytology may be valuable in the diagnosis of recurrent lesions of Kimura's disease and may spare the patient from repeated biopsies.
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PMID:Cytologic features of Kimura's disease in fine-needle aspirates. A study of eight cases. 808 55

A rare case of concurrent plasma cell granulomas (PCG) of the lung and the central nervous system (CNS) is reported. A 30-year-old man was presented with recurrent left headaches lasting for two years. Computerized tomographic (CT) scan and magnetic resonance imaging (MRI) of the head disclosed a process extending from the lateral aspect of the left cavernous sinus to the tentorium cerebelli and the infratemporal fossa through the foramen ovale. At the same time, chest-X ray and CT scan showed three symptomless masses of the pulmonary right lower lobe. Histological examination of cerebral samples and of one of the pulmonary nodules revealed the presence of a fibrous tissue containing numerous lymphocytes and plasma cells as well as remnants of vascular and respiratory structures. Immunohistochemical study proved these cells to be polyclonal. Ultrastructural analysis confirmed the presence of lymphoid cells and failed to disclose any argument for meningioma or histiocytosis X. The differential diagnostic problems of PCG are discussed as well as considerations about clinicopathological features, histogenesis and pathogenesis of inflammatory pseudotumours (IPT).
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PMID:Coexistence of plasma cell granulomas of lung and central nervous system. 883 77


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