Central nervous system bleeding in hemophiliacs

Abstract

From an estimated population of 2500 hemophiliacs seen between 1965 and 1976, 71 with documented central nervous system (CNS) bleeding were studied retrospectively: 56 had factor VIII deficiency and 15 had factor IX deficiency. More than two-thirds were less than 18 yr old, and one-third were age 3 yr or less when CNS bleeding occurred. Thirty-eight (54%) had a history of recent trauma; one-half of these had a long symptom-free interval of 4 ± 2.2 days (1 SD). Four had hypertension and three had underlying congenital anomalies. No etiology was apparent in 38%. Sixty-five patients had intracranial bleeding. Those with intracerebral bleeding had a poorer prognosis than did those with subarachnoid or subdural bleeding. Intraspinal bleeding occurred in six patients. The combined mortality rate was 34%. Of 47 survivors, 12 (26%) had recurrent bleeding in the absence of known trauma. Recurrent bleeding 1 yr or more after the initial episodes seemed to be more common in factor IX-deficient than in factor VIII-deficient patients. Clotting factor concentrates to maintain minimum blood levels at 30%-50% of normal were usually given for at least 10-14 days in those who survived. Forty-seven percent (22 of 47 survivors) had neurologic sequelae, such as mental retardation, seizure disorders, or motor impairment. From these observations we conclude that the evaluation and treatment of intracranial bleeding in hemophiliacs should include (1) prompt replacement therapy with factor VIII or IX for either cranial-spinal axis trauma or CNS signs and symptoms in the absence of a history of trauma, (2) documentation of bleeding by computerized tomography scanning or other diagnostic techniques, and (3) prolonged replacement therapy in patients with documented CNS bleeding.