Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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

A 20 year-old black male presented with a generalized lymphadenopathy, skin papules with maximum involvement of the head and trunk region, and elevated temperature. Ultrasound, CT and bone scans revealed infiltration of the kidneys and bones. Surgical specimens of the cervical lymph nodes showed massive infiltration of sinuses by histiocytes with engulfed lymphocytes and granulocytes in their cytoplasm. A similar finding was found in the excised dermis and nasopharyngeal mucosa. Immunohistochemical investigations showed a strong positive reaction of sinusoidal macrophages with anti S100 protein, anti alpha-1 antitrypsin and alpha-1 antichymotrypsin antibodies, negative CD1a. The engulfed granulocytes were positive with lysozyme and MAC387 antibodies; lymphocytes reacted with antilymphocytic antibodies L26, HLADR, UCHL-1 and OPD4 only rarely, possibly due to alterations of their cell membranes in the cytoplasmic environment of histiocytes. Occasional plasma cells were also entrapped in the macrophages. The best results were obtained with CD3 antibody which showed focal predominance of phagocytosed T cells. These findings confirm a nonselective nature of the emperipolesis. In situ hybridization with probes EBER and BHLF against Epstein-Barr virus sequences was negative. The pacient was treated by multidrug chemotherapy with a moderate regression of the infiltrates. The symptoms of the disease persisted for 10 months from the treatment start but further course of the disease is uncertain. The patients is lost to follow up.
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PMID:[Sinus histiocytosis with massive lymphadenopathy--a disseminated form of the Rosai-Dorfman syndrome]. 956 Sep 1