Anti-hi Antibody-induced Cold Agglutinin Disease Associated with Idiopathic Thrombocytopenic Purpura: Evans Syndrome.

A 70-year-old female was admitted with anemia and thrombocytopenia. Her blood type was B Rho (+). Her RBC was 250×104/μl, Hb7.8g/dl, Ht 23.2%, Retic. 55‰, WBC 2900/μl, T. bil 0.3mg/dl, LDH 917U/L, and Haptoglobin <10mg/dl. Cold agglutinin titer was×1024, direct Coombs test (-), indirect Coombs test (4+). Antibody screening test in saline revealed serum agglutinated adult O cells (4+) (×512), adult i cells (+) (×64), cord cells (-) and B cells (-), leading to an antibody diagnosis of anti-HI antibody. She subsequently developed thrombocytopenia (16×103/μl) and PAIgG was 54.8ng/107 platelets, giving a diagnosis of idiopathic thrombocytopenic purpura together with cold agglutin disease. The presence of cold agglutinin disease due to anti-HI antibody and development of ITP indicated this to be a case of Evans syndrome. Hemolytic anemia due to anti HI antibody is very rare, as is the association of ITP with cold agglutination disease.