Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P20020 (adenosine triphosphatase)
3,299 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A combined histologic, immunohistologic, enzyme histochemical, and immunologic study has been carried out in a 7-year-old girl with recurring extramediastinal monocentric giant lymph node hyperplasia of hyaline-vascular type. A large panel of monoclonal and polyclonal antibodies to lymphoid and nonlymphoid cell markers were tested on frozen and paraffin-embedded lymph node tissue as well as on cell suspension and peripheral blood. Tissue enzyme histochemical study, including a conventional hematologic panel, was performed on frozen and plastic-embedded sections. The pattern was dominated by nodular aggregates of round BA-1+ Leu-14+ HLA-DR+ ATPase+ lymphocytes with polyclonal sIgD and sIgM positivity and lacking cIg and BA-2 staining. Leu-1+/Leu-4+, OKT6+, OKT10+, Leu-7+, and CALLA+ cells were few or absent in the nodules, whereas DRC-1+ BA-2+ HLA-DR+ 5'-Nuc+ cells formed a dendritic network in the outer portion of the nodules. No immunoreactivity for lymphoid and nonlymphoid cell markers, including cytokeratin and keratin, was detected in centrinodular histiocytic-like cells. Particularly, the Hassall's-like structures contained a target-like positivity for laminin, and consisted of flattened acid phosphatase (AP), alpha-naphthyl acetate esterase (ANAE), 5'-nucleotidase (5'-Nuc), and adenosine triphosphatase (ATPase) positive cells, whose enzyme profile overlapped with that of the histiocytic-like cells. The extranodular areas were mainly composed of Leu-1+/Leu-4+ lymphocytes with Leu-3a+/OKT4+ phenotype and, to a lesser extent, of OKT6+ OKT10+ lymphoid cells and scattered cells with markers of histiocytic lineage. The abundant vascular component was generally identified by laminin positivity and, in smaller proportion, it was positive for Factor VIII-related antigen. Most of the medium-sized vessels with high endothelium had marked AP, ANAE, and ATPase activities. The process observed resulted from vascularized nodular aggregates of nontransformed B-cells with the phenotype of primary follicle lymphocytes, associated to centrinodular histiocytic-like cells with a distinct enzyme profile.
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PMID:Immunohistochemical, enzyme histochemical, and immunologic features of giant lymph node hyperplasia of the hyaline-vascular type. 242 88

This study reviews data on the histogenesis of Kaposi's sarcoma and angiosarcoma derived from clinical features, histology, electron microscopy, enzyme histochemistry, and immunochemistry of both diseases. Their hemorrhagic clinical appearance contrasts the predominantly lymphatic histologic features of vessels in early lesions. Investigations performed to resolve the debate whether these tumors arise from blood vessel or lymphatic endothelium show remarkably similar results for both conditions. Electron microscopy reveals Weibel - Palade bodies in a minority of cases, but features consistent with less well-differentiated blood vessel endothelium may be seen in a greater proportion of tumors. Enzyme histochemistry generally shows absence of adenosine triphosphatase and alkaline phosphatase in tumor cells; a pattern of enzymes similar to that found in normal lymphatic endothelium. Conflicting data arises from the large number of immunohistochemical studies performed on both conditions. Factor VIII-related antigen and Ulex europaeus agglutinin-I have been most frequently employed, but the specificity of these agents for blood vessel endothelium is debatable. Panendothelial markers show consistent labeling of both tumors, but marker studies employing a wide range of monoclonal antibodies specific for blood vessel endothelium have shown occasional positive labeling of tumor cells. A number of studies have claimed absence of labeling with specific blood vessel monoclonal antibodies, but at present no study employing a specific marker for lymphatic endothelium has been reported. Although the demonstration of specific markers for blood vessel endothelium in these tumors has been variable, the data would be compatible with lesions arising from undifferentiated stem cells that proliferate with varying degrees of differentiation toward blood vessel endothelium. An alternative hypothesis for the histogenesis of Kaposi's sarcoma would be one of multicentric hyperplasia containing lymphatic venular anastamoses with elements of both lymphatic and blood vessel endothelium.
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PMID:Histogenesis of Kaposi's sarcoma and angiosarcoma of the face and the scalp. 266 17