Aggressive combination therapy in the successful management of life-threatening intracranial hemorrhage in a patient with idiopathic thrombocytopenic purpura.

We describe acute therapy for a 13-year-old female from Panama with chronic idiopathic thrombocytopenia purpura (ITP) refractory to steroids, splenectomy, vinca alkaloids, and azathioprine. She presented with neurologic deterioration from a posterior fossa intracranial hemorrhage (ICH). This followed a 3-month history of severe dysfunctional uterine bleeding, progressive from menarche, which had required multiple red cell transfusions. Steroid and vinblastine therapy and transfusion of 40 U of platelets failed to increase the platelet count above 10,000/microliter. Development of a second larger ICH (frontal) produced morbid increase in intracranial pressure that necessitated neurosurgical decompression. Plasma exchange and colloid repletion with intravenous gamma globulin (1 g/kg) and an infusion of 20 U of platelets resulted in a transient rise in platelet count to 160,000/microliter, permitting surgery without bleeding. Danazol (800 mg/day) and conjugated estrogen (Premarin 25 mg/day) were begun to control the uterine bleeding. Intensive plasma exchange and i.v. IgG infusions were continued daily for 24 days, then twice weekly for several weeks. Platelet-bound IgG decreased almost 500% over the first 10 days of therapy, and platelets increased dramatically to 600,000/microliter after 3 weeks of therapy. The patient has remained amenorrheic with a normal platelet count for more than 24 months on daily danazol therapy and monthly infusions of i.v. IgG (0.4 g/kg/dose).