Does Sjögren's syndrome predispose surgical patients to acquired hemophilia?
The authors report the first American case of a factor VIII inhibitor found in a patient with Sjögren'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 Sjögren's syndrome had been previously diagnosed via lower lip biopsy. After fasciotomy, signs and symptoms of intra-abdominal hemorrhage developed, and the patient was taken to laparotomy, where no hemorrhage was found. A coagulopathy developed, and the patient's 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 Sjögren's syndrome.
Dachman
A F
AF
Botsford General Hospital, Farmington Hills, Mich., USA.
Margolis
H
H
Aboulafia
E
E
D000368:Aged; D000614:Aminocaproates / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005169:Factor VIII / Q000008:administration & dosage; D015951:Factor Xa / Q000008:administration & dosage; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology*; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D011183:Postoperative Complications / Q000209:etiology*; D012859:Sjogren's Syndrome / Q000150:complications*; D013256:Steroids / Q000008:administration & dosage; D005719:gamma-Globulins / Q000008:administration & dosage
D000614:Aminocaproates; D006490:Hemostatics; D013256:Steroids; D005719:gamma-Globulins; D005169:Factor VIII; D015951:Factor Xa