Pure red cell aplasia in autoimmune polyglandular syndrome with T lymphocytosis

Haematologica. 1998 Nov;83(11):1043-5.

Abstract

We report the onset of pure red cell aplasia (PRCA) in a patient with a history of polyglandular syndrome including Addison's disease, malabsorption syndrome, diabetes type I and gastric hyperplastic polyposis. An autoimmune origin for this complex disorder was not supported by the presence of organ specific antibodies, but T cells were found to be of polyclonal origin, as demonstrated by molecular analysis of T cell receptor (TCR) gene rearrangement. The pathophysiology of this case, based on laboratory findings and response to therapy, is discussed.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Adult
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / drug therapy
  • Bone Marrow / pathology
  • Gene Rearrangement, beta-Chain T-Cell Antigen Receptor
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Lymphocytosis / complications*
  • Lymphocytosis / drug therapy
  • Malabsorption Syndromes / complications
  • Malabsorption Syndromes / drug therapy
  • Male
  • Polyendocrinopathies, Autoimmune / complications*
  • Polyendocrinopathies, Autoimmune / drug therapy
  • Red-Cell Aplasia, Pure / complications*
  • Red-Cell Aplasia, Pure / drug therapy
  • Red-Cell Aplasia, Pure / physiopathology
  • T-Lymphocytes*

Substances

  • Immunosuppressive Agents