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)

A detailed immunologic study of three cases of sinus histiocytosis with massive lymphadenopathy (SHML) was performed to better characterize this rare disorder. One patient had prominent cervical lymphadenopathy that regressed spontaneously, whereas the other two patients had persistent cervical lymphadenopathy and recurrent infections. The first patient was otherwise healthy and had normal immunologic studies. One of the latter patients had a relative increase in blood B cells, a decreased level of serum immunoglobulin A (IgA), decreased blood lymphocyte mitogenic responses to multiple mitogens (37-42% of controls), and cutaneous anergy. The other patient with persistent disease also had a relative increase in blood B cells, polyclonal hypergammaglobulinemia, and circulating immune complexes, as well as decreased blood T cells and markedly decreased blood lymphocyte responses to mitogens (12-37% of controls). Immunohistochemical stains of the lymph nodes of the three patients revealed a characteristic phenotype for the sinus histiocytes: S-100 protein, 3/3; CD14 (Leu M3) 3/3; CD11c (Leu M5), 1/1; CD71 (OKT9), 3/3; CD4 (Leu 3a), 2/3; CD1a (OKT6), 1/3; alpha-1-antitrypsin, 3/3; alpha-1-antichymotrypsin, 3/3; CD35 (C3b), 1/1; CD11b (Mo1), 0/3; CD15 (Leu M1), 0/3; HLA-DR, 0/3; and lysozyme, 0/3. This phenotype suggests that the cells of SHML have features of both the Langerhans/interdigitating cell and mononuclear phagocyte lineages. Emperipolesis by the histiocytes of B cells, T cells, and natural killer cells was demonstrated by a double-staining technique. Our findings indicate that patients with SHML may have a variably expressed immunodeficiency that predisposes them to recurrent infections.
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PMID:Sinus histiocytosis with massive lymphadenopathy: a spectrum of disease associated with immune dysfunction. 171 75

A 54-year-old man was admitted because of right supraclavicular lymphadenopathy of some weeks duration. Computed axial tomography revealed a large multinodular lesion in a supraclavicular lymph node. The patient then had a supraclavicular lymph node biopsy. Light microscopy showed a tumor whose structure was suggestive of an interdigitating cell sarcoma. Enzyme and immunohistochemical analysis showed that the tumor cells possessed membranous adenosine triphosphatase activity, intracytoplasmic S100 protein, surface CD1a and CD4 antigens, and HLA-DR antigen. Ultrastructural examination showed that the cells exhibited many interdigitating cytoplasmic extensions, but no Birbeck granules. DNA content analysis of the tumor cells proved that the cells were malignant. These data are consistent with derivation from a lymph node interdigitating cell.
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PMID:Lymph node interdigitating cell sarcoma. A case report. 172 55

We describe the clinical, ultrastructural, and immunophenotypical characteristics of four cases of an unusual type of T cell leukemia. Clinical features included high WBC, ranging from 26-148 x 10(9)/liter, bone marrow infiltration, splenomegaly, and lymphadenopathy. Skin involvement was not documented at presentation, but it was seen as a terminal event in one patient with a pattern of dermal lymphocytic infiltration different from that usually seen in Sezary syndrome. By ultrastructural analysis, the circulating lymphoid cells were indistinguishable from small Sezary cells in two cases, resembled large Sezary cells in one case, and consisted of a mixture of small Sezary cells and prolymphocytes in the remaining case. The cells from all cases had a mature T cell phenotype, TdT-, CD1a-, CD2+/-, CD3+, CD5+. In addition, the cells were either CD8+, CD4- or CD8+, CD4+ or CD4-, CD8-; and, in only one case, the findings were similar to those of Sezary syndrome cells: CD4+, CD8-, CD7-, BE-2+. In the latter case, serological and immunological assays were positive for HTLV-I while these were negative in two other patients investigated. The features of these patients suggest that Sezary cell leukemia is a distinct clinico-pathological entity although the alternative diagnosis of adult T cell leukemia/lymphoma could not be excluded in the HTLV-I+ case. Sezary cell leukemia appears to be resistant to current chemotherapy regimens and is associated with an aggressive clinical course and short survival.
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PMID:Sezary cell-like leukemia: a distinct type of mature T cell malignancy. 236 82

The morphological, enzymatical, immunocytochemical and functional properties of Langerhans' cells are briefly reviewed. Langerhans' cells are located mainly in the squamous stratified epithelia, but are also present in the thymus and in superficial lymphnodes. At the ultrastructural level, they are characterized by unique cytoplasmic organelles, the Birbeck granules, whose function is still unknown. Langerhans' cells possess strong ATPase activity and are weakly positive for alpha-naphtyl acetate esterase and for acid phosphatase; they are immunoreactive for CD1a (T6), class II MHC antigens and S-100 protein. In some pathological conditions, like dermatopathic lymphadenopathy and Langerhans' cell histocytosis, Langerhans' cells also are characterized by the expression of monocyte-macrophage antigens. Langerhans' cells act as antigen-presenting cells to T lymphocytes; their functional capacity is strictly dependent on the levels of expression of class II MHC antigens. Langerhans' cells are of bone marrow origin and are derived from a circulating precursor which is probably related to the monocyte.
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PMID:The Langerhans' cells. 268 41

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

The present study shows that Langerhans cells can be differentiated from interdigitating cells at the light microscopic level. Superficial lymph nodes and skin taken from necropsies and the lymph nodes of dermatopathic lymphadenopathy (DPL) were used for this experiment. Sections of lymph node and skin were embedded using the acetone, methyl benzoate and xylene (AMeX) method and dendritic cells were immunostained with anti S-100 protein antibody (S-100, and OKT-6 (CD1a) using the restaining method. Langerhans cells in the skin were positive for both CD1a and S-100. Dendritic cells positive for both CD1a and S-100, and dendritic cells positive for S-100, but not for CD1a were observed in superficial lymph nodes. In normal superficial lymph nodes, there were more interdigitating cells than Langerhans cells. The majority of the dendritic cells in the DPL were Langerhans cells. We conclude that the S-100 and CD1a positive cells are Langerhans cells, and the S-100 positive-CD1a negative cells are interdigitating cells.
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PMID:Differentiation of Langerhans cells from interdigitating cells using CD1a and S-100 protein antibodies. 754 32

We describe the case of a patient with peripheral gamma/delta T-cell lymphoma (T-ML) with hepatosplenomegaly, generalized lymphadenopathy, and bone marrow involvement. A 44-year-old man had lymphoma, which became clinically apparent 2 months after the onset of myositis and insulin-dependent diabetes mellitus. A cervical lymph node biopsy specimen showed diffuse infiltration by large neoplastic cells with vascular proliferation. The neoplastic cells expressed the T-cell receptor (TCR)delta chain detected by TCR delta 1 and delta-TCS1, CD3, CD30, CD45RO, and epithelial membrane antigen, but not the TCR beta chain detected by beta F1, CD1a, CD2, CD4, CD5, CD7, CD8, CD25, HLA-DR, and terminal deoxynucleotidyl transferase. The cells had a clonal rearrangement of TCR gamma chain gene and a germ-line configuration of immunoglobulin heavy chain gene and TCR beta chain gene. Despite chemotherapy, the patient died of refractory lymphoma 4 months after diagnosis. Examination at autopsy revealed that the main hepatic and splenic neoplastic infiltration sites were the portal area and white pulp, respectively. Our patient differed from those with gamma/delta T-ML with hepatosplenic involvement reported previously with respect to the hepatic and splenic neoplastic infiltration patterns and the presence of lymphadenopathy.
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PMID:Gamma/delta T-cell lymphoma with hepatosplenomegaly: report of a case. 836 90

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

Skin involvement is common in sinus histiocytosis with massive lymphadenopathy (SH, Rosai-Dorfman disease), but pure cutaneous cases are rare. A 70-year-old woman presented with a 10-year history of large red-orange nodules and plaques on her upper arms, face, and buttocks, without evidence of lymphadenopathy or internal involvement. Distinctive histopathologic differences were observed according to the duration of the lesions. In recent lesions, the dermal infiltrate was mostly composed of sheets of characteristic SH cells; on the other hand, in long-lasting lesions, the presence of xanthomatous changes and prominent fibrosis, in keeping with the self-limited nature of this disease, raises problems of differential diagnosis with other xanthohistiocytic disorders. Immunophenotypic studies showed that the SH cells are S-100+ CD1a negative-activated macrophages, capable of lysosomal activity. The adhesion molecule pattern of SH cells (CD11b+, CD11c+, CD18+, CD62L+, and CD103+) was similar to that of circulating monocytes, suggesting their recent migration from the bloodstream.
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PMID:Immunohistologic findings and adhesion molecule pattern in primary pure cutaneous Rosai-Dorfman disease with xanthomatous features. 970 Mar 80

We describe the morphologic, immunohistologic, and genotypic characteristics of 13 cases of true histiocytic lymphomas. Six cases presented with primary gastrointestinal involvement, five with lymphadenopathy, the other sites involved being the bone marrow and the skin. The neoplastic cells displayed large abundant eosinophilic cytoplasm, occasionally vacuolated with folded or bizarre-shaped nuclei with prominent nucleoli. Mitotic figures were numerous. Multinucleated cells were common. The pattern of growth was usually diffuse and noncohesive. Spindle cell sarcoma-like areas were evident in five cases, with a prominent foam cell component in four cases. All cases expressed histiocyte-associated markers (CD68, lysozyme, alpha-1-antitrypsin), CD45 or CD45RO, and were negative for CD1a, epithelial, and B- and T-cell lineage-specific markers. Reactivity for S-100 was observed in a variable proportion of cells in 11 cases. The proliferation fraction varied from 3 to 88%. Genotypic analysis for T-cell receptor or immunoglobulin gene rearrangement demonstrated a germline configuration in all cases. We demonstrate that true histiocytic lymphoma is a rare distinctive pathologic entity that may be defined by immunohistochemical criteria and that recognition among histiocytic disorders is important for clinical and prognosis reasons.
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PMID:True histiocytic lymphoma: a morphologic, immunohistochemical, and molecular genetic study of 13 cases. 980 31


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