Familial evans syndrome: A report of an affected sibship

Purpose: This report describes the clinical course of three siblings, all o f whom had Evans syndrome in childhood. Patients: The coexistence of autoimmune hemolytic anemia and thrombocytopen ia, in the absence of a known underlying cause, led to the diagnosis of Eve ns syndrome in a 4-month-old girl and subsequently in her two brothers when they were 4 and 13 years old. Results: The 4-month-old girl had a life-threatening relapsing course unres ponsive to corticosteroids, intravenous gammaglobulin, thymectomy, and cycl ophosphamide. She eventually responded to splenectomy. Her two brothers had milder disease that responded to corticosteroids. Cytogenetic analyses rev ealed the presence of a familial Y;15 translocation in all three children a nd their father. Conclusion: There are few reported cases of familial Evens syndrome, and th ey are usually associated with an inherited congenital abnormality. We repo rt the unusual finding of three siblings with the disease and no known cong enital abnormality.