Massive hemoperitoneum with splenic infarction in Evans syndrome

Evans syndrome is a very rare hematologic autoimmune disease, characterized by a direct Coombs' positive hemolytic anemia and immune thrombocytopenic purpura without a known underlying etiology. The clinical course is generally chronic with frequent relapses and remissions. Evans syndrome usually is complicated by hemolytic or thrombocytopenic symptoms. This is seldom associated with thrombosis or infarction. Reported here is a case with massive hemoperitoneum because of splenic infarction with rupture, in an 18-month-old male patient with Evans syndrome, and the embolization of splenic artery. This article also carries clinical and imaging features and the review of medical literature.