Immunoregulatory abnormalities in Evans syndrome

Am J Hematol. 1983 Dec;15(4):381-90. doi: 10.1002/ajh.2830150409.


Immune function in six patients with Evans syndrome (Coombs-positive hemolytic anemia and immune thrombocytopenia) was compared to that in seven with chronic ITP. The two groups differed in measurements of T-cell subsets and immunoglobulin production. Evans syndrome patients had decreased T4 (T-helper) (P = 0.025), increased T8 (T-suppressor) (P = 0.008), and a decreased ratio of T4:T8 cells (P = 0.0009) when compared to controls. Results in chronic ITP patients were similar to those in controls. Serum IgG, IgM, and IgA levels and in vitro synthesis of IgG and/or IgM were decreased in most Evans syndrome patients. Diminished in vivo and in vitro immunoglobulin synthesis in Evans syndrome is consistent with the decreased T4:T8 ratio in these patients. The altered T4:T8 ratio may represent an unsuccessful response to an autoimmune process in which the trigger is unknown.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Hemolytic / complications
  • Anemia, Hemolytic / immunology*
  • Anemia, Hemolytic, Autoimmune / immunology
  • Autoantibodies / immunology*
  • B-Lymphocytes / immunology*
  • Child
  • Child, Preschool
  • Coombs Test
  • Female
  • Humans
  • Immunoglobulins / biosynthesis
  • Leukocyte Count
  • Male
  • Syndrome
  • T-Lymphocytes / immunology*
  • Thrombocytopenia / complications
  • Thrombocytopenia / immunology*


  • Autoantibodies
  • Immunoglobulins