A man with sideroblastic anemia had a splenectomy because of a salmonella abscess of the spleen that had ruptured into the colon. Two months later he developed recurrent thrombophiebitis and fatal thromboembolism associated with thrombocytosis. A review of the literature showed multiple additional cases of sideroblastic anemia with thrombocytosis and thromboembolism after splenectomy. In many of these cases the patient died. Splenectomy for treatment of a sideroblastic anemia probable is contraindicated. If splenectomy is done, long-term therapy to avoid thromboembolic complications probably should be maintained for many months or even years.