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  • Book
    Franco Rongioletti, Irina Margaritescu, Bruce R. Smoller, editors ; with the contributors of Werner Kempf.
    Summary: This book provides practitioners with a single volume that reviews the clinical and pathologic features of rarely encountered cutaneous neoplasms. The text includes an in-depth discussion of the clinical findings, as well as the histologic and immunologic features of these diseases that are rarely encountered, reported, or recognized. Additional laboratory data used to make and support the diagnoses with some therapeutic and prognostic hints are discussed for each entity. The volume is organized into general categories correlating with the cell(s) of origin. An atlas of clinical manifestations is presented for each entity that will serve as a bedside clinical reference, followed by a series of photomicrographs depicting the histologic changes. In all cases, recent updates on molecular tools helpful in attaining the diagnosis are added to the sections on histology and immunohistochemistry. Rare Malignant Skin Tumors is a valuable bedside reference for the practicing dermatopathologist, general pathologist reviewing skin biopsies, general dermatologists, cutaneous oncologists and Mohs? surgeons.
    Digital Access Springer 2015
  • Article
    Gelfand EW, Lee JJ, Dosch HM.
    Proc Natl Acad Sci U S A. 1979 Apr;76(4):1998-2002.
    A role for the enzymes adenosine deaminase (adenosine aminohydrolase, EC 3.5.4.4) and purine-nucleoside phosphorylase (purine-nucleoside:orthophosphate ribosyl-transferase, EC 2.4.2.1) in the functional maturation of lymphoid cells has been revealed by the association of inherited deficiencies of these enzymes and profound immune deficiency. Previous studies have suggested that the selective toxicity for lymphocytes may be mediated by the accumulation of toxic deoxynucleoside metabolites, likely through the action of specific kinases enriched in lymphoid cells. In order to study possible mechanisms whereby lymphocyte function may be impaired in these disorders, we have studied the effect of nucleosides and their deoxy analogues on both T and B lymphocyte growth and function. In the presence of deoxyguanosine, there was marked inhibition of T lymphoblast growth, phytohem-agglutinin-induced cell proliferation, and T suppressor cell activity. T helper cell activity and the differentiation of B cells to an antibody-secreting stage were unaffected. Deoxyadenosine was much less inhibitory, but in the presence of an inhibitor of adenosine deaminase, its effects on lymphocyte growth and function were markedly potentiated. The addition of deoxycytidine prevented deoxyadenosine toxicity in all assays, whereas it only interfered with deoxyguanosine effects on T lymphoblast growth. These studies provide some initial understanding for the selective loss of specific lymphocyte functions in individuals with inborn errors of purine metabolism.
    Digital Access Access Options