A 51-year-old male with acute lymphoblastic leukemia whose course was complicated by primary fibrinolysis and spontaneous rupture of the spleen is described. The patient was treated with various drug combinations: vincristine and prednisone, later by cytosine arabinoside and finally by prednisone, methotrexate and 6-mercaptopurine. Four months after the diagnosis he developed epistaxis and petechiae. The coagulation tests were compatible with primary fibrinolysis and he responded to treatment with ε-aminocaproic acid (EACA). One month and a half later he developed again epistaxis, ecchymoses, splenomegaly and an acute abdomen. The coagulation tests revealed primary fibrinolysis. The operation revealed a ruptured spleen. After splenectomy the treatment with EACA was continued and, despite an improvement in the coagulation tests, the patient died.

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