Acquired Factor VIII Deficiency and Polyradiculoneuropathy

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Abstract
A 77-year-old man was hospitalized with a two-month history of progressive weakness in all four extremities. Electromyography and studies of nerve-conduction velocity revealed acute denervation consistent with acute idiopathic polyradiculoneuropathy, a condition resembling the Guillain—Barré syndrome. During the hospitalization, a coagulopathy developed, manifested by purpuric lesions and abnormal prothrombin and partial-thromboplastin times. Laboratory analysis revealed a delayed inhibitor to factor VIII. Corticosteroid therapy was begun in an attempt to suppress production of the inhibitor. After 10 days of therapy, there was no change in the abnormal coagulation-test results, and a large retroperitoneal hematoma developed spontaneously. Therapy was begun with factor VIII concentrate followed by cyclophosphamide, vincristine, and prednisone, as described by Lian et al.1 Shortly after receiving the first course, the patient died of aspiration pneumonia.