Immunoregulatory abnormalities in Evans syndrome.
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.
D000293:Adolescent; D000328:Adult; D000743:Anemia, Hemolytic / Q000150:complications / Q000276:immunology*; D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D003298:Coombs Test; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000096:biosynthesis; D007958:Leukocyte Count; D008297:Male; D013577:Syndrome; D013601:T-Lymphocytes / Q000276:immunology*; D013921:Thrombocytopenia / Q000150:complications / Q000276:immunology*
D001323:Autoantibodies; D007136:Immunoglobulins