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Pure red cell aplasia associated with type I autoimmune polyglandular syndrome: successfull responce to therapy with mycophenolate mofetil: case report and review of literature
  1. Milena Bakrac (vdvd{at}mailcity.com)
  1. Institute of Hematology, Serbia and Montenegro
    1. Vladimir Jurisic (vdvd{at}mailcity.com)
    1. University of Kragujevac, Serbia and Montenegro
      1. Tanja Kostic
      1. Institute for Molecular Genetics and Genetic Engineering, Belgrade, Serbia, Serbia and Montenegro
        1. Vera Popovic
        1. Institute of Endocrinology, Serbia and Montenegro
          1. Sandra Pekic
          1. Institute of Endocrinology,, Serbia and Montenegro
            1. Nada Kraguljac
            1. Institute of Hematology, Serbia and Montenegro
              1. Milica Colovic
              1. Institute of Hematology, Serbia and Montenegro

                Abstract

                We report the simultaneous appearance of pure red cell aplasia syndrome (PRCA) and autoimmune polyglandular syndrome type I (APS I) associated with high percentage of ãä TCR+ lymphocytes in peripheral blood. PCR analyses confirmed lymphocytes’ monoclonality for TCR ã/ä rearrangement. Treatment with mycophenolate mofetil led to decrease of the total lymphocyte count, significant decrease of ã/ä T cell count, restoration of CD4/CD8 subset ratio, and consequently increase of hemoglobin level four months after therapy. The lymphocytes obtained after therapy showed high expression of Fas (CD95) molecule and high apoptotic rate compared to healthy donor lymphocytes.

                • Pure red cell aplasia
                • T cell subsets
                • cell culture
                • mycophenolate mofetil
                • poliglandular sundrome

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