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

Four cases of benign Langerhans cell histiocytosis limited to the skin were studied. In all three self-healing cases (cases 2, 3, and 4) many dense bodies, myelin bodies, and worm bodies were found. In one chronic case (case 1) none of these was identified. In all four cases, in addition to CD1, HLA-DR, and S-100 stains, interferon-gamma and S-100 beta-subunit were positive in the dermal tumor cells. Both interferon-gamma and S-100 beta-subunits were negative in the normal epidermal Langerhans cells. A comprehensive literature review yielded 87 cases of skin-limited Langerhans cell histiocytosis. These cases could be subgrouped into three categories: (1) those that resolved spontaneously, (2) those that responded to therapy and had no recurrence, and (3) those with persistent or recurrent lesions, not responding to therapy but still limited to the skin.
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PMID:Immunohistochemistry and electron microscopy in Langerhans cell histiocytosis confined to the skin. 168 9

An unusual adult case of Langerhans cell histiocytosis is presented. The case initially mimicked granuloma annulare, both clinically and histologically. The patient subsequently had development of extensive cutaneous involvement that pathologically revealed a diffuse infiltrate of CD1-positive histiocytic cells containing Langerhans granules. Extensive investigations failed to detect systemic involvement. The patient's cutaneous eruption did not respond to various therapeutic interventions, including phototherapy with oral psoralen with long-wave UV radiation in the A range (PUVA) and systemic vinblastine sulfate. Marked but temporary clinical and histologic improvement was achieved with total body electron beam radiotherapy. The nosology of this case is discussed in the context of the various histiocytic proliferative disorders.
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PMID:Primary cutaneous Langerhans cell histiocytosis in an adult. 192 63

Langerhans cells and their pathologic counterparts can be identified in paraffin sections using immunohistochemical staining for S-100 protein. This procedure is useful in confirming a diagnosis of Langerhans cell histiocytosis (LCH). However, many other cell types are also positive for S-100 protein. Positive staining for CD1 (Leu 6) supports a diagnosis of LCH, but requires frozen tissue. A panel of antibodies would be desirable in confirming a diagnosis of LCH, particularly if these antibodies could be used on paraffin-embedded material. We studied the pattern of staining for commercially available monoclonal antibodies MT1, MT2, MB2, and LN1, which were originally marketed as lymphocyte markers, using paraffin-embedded tissue sections of cases of LCH. In all 20 cases pathologic Langerhans cells stained positively with MT1 only. Various other S-100 protein-positive lesions were also examined with MT1 and were consistently negative for MT1. Other cutaneous histiocytic and mast cell lesions were positive with MT1, but S-100 protein negative. Our results demonstrate that the monoclonal antibody MT1 serves as an additional marker for LCH and, together with S-100 protein, would make up a diagnostic panel of antibodies for LCH to be used on routine paraffin-embedded sections.
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PMID:Monoclonal antibody MT1: a marker for Langerhans cell histiocytosis. 219 Jan 98

In this report, we describe a patient with the classic histology of pulmonary histiocytosis X, who had bilateral reticulonodular densities and mediastinal lymph node involvement. The diagnosis was confirmed by the use of electron microscopy and immunohistochemical markers (PS100, HLA-DR, and CD1), which allowed us to recognize the lymph node infiltration of X histiocytes. An association of mediastinal lymph node enlargement with pulmonary histiocytosis X has been reported but it has never been histologically documented.
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PMID:Pulmonary histiocytosis X with mediastinal lymph node involvement. 224 Aug 46

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

Human epidermal Langerhans cells express two (CD1a and CD1c) of the three human thymic cell surface differentiation antigens (CD1a, CD1b, and CD1c). The first cluster of differentiation antigens (CD1) is defined by a group of monoclonal antibodies (MCA). All these MCA were obtained after immunization of mice or rats with human cortical thymocytes. OKT6 MCA (a CD1a MCA) was the first to be described as reactive with human epidermal Langerhans cells. We produced a murine MCA, called DMC1, after immunization with proliferating Langerhans cells of Eosinophilic Granuloma of the bone (Histiocytosis X). In tissues DMC1 MCA reacted with epidermal dendritic cells (Langerhans cells) in the skin and cortical thymocytes in the thymus as observed on indirect immunofluorescence. At the ultrastructural level, DMC1 MCA was specific for Birbeck granule-containing Langerhans cells and did not react with melanocyte and keratinocyte populations. The quantitative analysis of immunoelectron labeling and the cytofluorometric study showed that the intensity of labeling was inversely correlated with the concentration of trypsin used in the preparation of epidermal cell from skin samples. DMC1 MCA precipitated a protein with a relative mass of 49,000 (CD1a molecule) from lysates of iodinated epidermal Langerhans cells under reducing conditions. It recognized the original CD1a molecule (Mr 49,000) but not the membrane breakdown product of CD1a (Mr 27,000) brought about by trypsin.
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PMID:DMC1: a monoclonal antibody produced from histiocytosis X cells which reacts with the native CD1a molecule of human epidermal Langerhans cells. 246 37

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

To evaluate the effect of cigarette smoking on the number, distribution, and differentiated state of dendritic cells (DC) and Langerhans cells (LC) in the human lung, we have quantitated the number of these cells present in the bronchioles and alveolar parenchyma of lung tissue from nonsmokers and cigarette smokers using anti-CD1 monoclonal antibodies which react preferentially with DC (M241) and LC (T6). M241+ DC were found in the bronchiolar submucosa and alveolar parenchyma of nonsmokers; T6+ LC were present within the bronchiolar epithelium. Cigarette smoking was associated with a twofold increase in the total number of cells of DC/LC lineage and a 30-fold increase in the number of T6+ cells, many of which contained Birbeck granules (LC), present in the alveolar parenchyma. Most LC found in the parenchyma of smokers were observed in close association with areas of alveolar type II pneumocyte hyperplasia. Cigarette smoking did not change the number of differentiated state of cells of DC/LC lineage within the bronchioles. Both DC and LC are present in the human lung. Cigarette smoking has an important effect on the number, distribution, and differentiated state of these cells, which may explain why most adult patients who develop Langerhans cell granulomatosis are smokers.
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PMID:Cigarette smoking-induced changes in the number and differentiated state of pulmonary dendritic cells/Langerhans cells. 271 39

Plasminogen activators (PA) play an important role in cell migration and tissue degradation. Considering the strong epidermotropism of atypical mononuclear cells in histiocytosis X (HX) skin infiltrates leading to intraepidermal abscess formation, it was the purpose of this study to look for tissue-type PA (t-PA) and/or urokinase-type PA (u-PA) on HX cells. Four monoclonal antibodies against PA were used, employing the alkaline phosphatase anti-alkaline phosphatase (APAAP) technique on cryostat sections from four patients with HX. Twenty percent to 40% of infiltrating cells in the epidermis expressed the t-PA antigen. t-PA+ cells were present in the follicular centers of human tonsil, absent in normal epidermis and scanty in cutaneous infiltrates from mycosis fungoides and lupus erythematosus. Double labeling with anti-PA and T6 (CD1) or S100 protein revealed some of the HX cells to express both antigens (t-PA+ CD1+ or t-PA+ S100+). We conclude that cutaneous infiltrates of HX contain PA+ dendritic cells which are different from normal Langerhans cells and which may be responsible for the strong epidermal alterations in HX.
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PMID:Cutaneous infiltrates of histiocytosis X contain plasminogen activator-bearing epidermotropic dendritic cells different from Langerhans cells. 311 52

Proliferating cells in histiocytosis X (histiocytosis X cells) share many structural and immunophenotypic features with Langerhans cells, leading to the assumption that histiocytosis X represents a proliferative disorder of Langerhans cells. Because, depending on their state of activation and/or differentiation, Langerhans cells exhibit a varying immunophenotype, we investigated whether histiocytosis X cells display a similar phenotypic heterogeneity and, if so, whether the heterogenous biological behavior of histiocytosis X is reflected by differences in the immunophenotype of the proliferating cells. In 21 patients suffering from different clinical manifestations of histiocytosis X, proliferating cells uniformly expressed class I and II alloantigens, T200, CD1, CD4, and S100 protein. In 12 of 21 cases, histiocytosis X cells additionally exhibited immunocytochemically detectable amounts of C3b and C3bi receptors and certain monocyte/macrophage antigens (CDw14, Ki-M1, Ki-M6). This immunophenotypic heterogeneity of histiocytosis X cells could not be correlated with clinical course, prognosis, and final outcome of the disease in a given patient. The capacity of histiocytosis X cells to immunophenotypically mimic various states of Langerhans cell activation and/or differentiation, however, underscores the concept of histiocytosis X as a proliferative disorder of Langerhans cell origin.
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PMID:The phenotypic spectrum of histiocytosis X cells. 328 Jun 96


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