Multiple autoimmune manifestations in a splenectomized subject with HLA-B8

Idiopathic thrombocytopenic purpura developed in a 31-year-old man, and he underwent a splenectomy because of his failure to respond to steroid therapy. Subsequently, during a six-year follow-up, chronic active hepatitis, Coombs' positive hemolytic anemia, and pulmonary interstitial fibrosis developed. Since such a clustering of autoimmune manifestations in a single subject is unusual, possible contributing factors were sought. We suggest that both the splenectomy and the histocompatibility antigen HLA-B8, which the patient was found to carry, may have contributed to this rare clinical syndrome.