Does Sjogren's syndrome predispose surgical patients to acquired hemophilia?

Abstract

The authors report the first American case of a factor VIII inhibitor found in a patient with Sjogren's syndrome. The inhibitor was isolated from a patient with no known hematologic disorder who was seen with a compartment syndrome of the left thigh after sustaining a fall from bed. The Sjogren's syndrome had been previously diagnosed via lower lip biopsy. After fasciotomy, signs and symptoms of intraabdominal hemorrhage developed, and the patient was taken to laparotomy, where no hemorrhage was found. A coagulopathy developed, and the patients hemoglobin continued to fall. A complete factor analysis revealed a factor VIII inhibitor that was quantified at 40 Bethesda units. After vigorous therapy-which included factor concentrates, immunoglobulins, steroids, antifibrinolytic agents, and blood replacement-the patient's bleeding stopped and she continued to convalesce under hospital care until her ultimate death from respiratory problems. Acquired hemophilia with a factor VIII inhibitor may be associated with Sjogren's syndrome.