A 25-year single institutional experience withi autoimmunefactorvininhibitors (acquired hemophilia)

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Abstract

Background: Acquired hemophilia (aHA) is a rare, potentially fatal bleeding disorder.! Presentation is similar to that of patients (pts) with congenital hemophilia A (cHA), bull has its onset in older age or postpartum period and occurs in both genders. Aims: Ascertain! the natural history and response to therapy in pts with acquired hemophilia (aHA).| Methods: Review of medical records of all pts with aHA diagnosed between 1975 and 1999. Results: Over a 25yr period approximately 400 pts with a diagnosis (Dx)of hemophilia A were seen at Mayo Clinic, of which, 40 pts, (22 m and 18 f), mean age 60.91 yrs (24 to 86), had aHA. Mean duration of symptoms prior to Dx was 1.6 months (1 wk to 9 m). In one asymptomatic pt, Dx was made during work up of a prolonged aPTT. Bleeding manifestations included cutaneous 80% (n=32), soft tissue 50% (n=20), hematuriai 20% (n=8), postoperative 15% (n=6), gastrointestinal 10% (n=4), epistaxis] 10%(n=4),hemarthrosis 8%(n=3) and hemoptysis in one(2%). Most inhibitors werel idiopathic (58%; n=23), while an associated condition was found in 17 pt (42%)] postpartum 12% (n=5), autoimmune disorder 25% (n=10), malignancy 5% (n=2). Thel median human inhibitor titer (HIT) was 33 Bethesda Units (BU)(1 to 666); with cross reactivity to porcine FVIII demonstrable in 10 pt (out of 22 that were tested). Median porcine inhibitor titer (PIT) was 9 BU (1-63). Median PIT/HIT cross reactivity was 5.8 %. Six/8 pt treated with prothrombin complex concentrates for acute hemorrhage had a good clinical response, while 6/6 pt treated with porcine FVIII had a good clinical response Chronic immunosuppression in 34 pts included prednisone [P] (9), cyclophosphamide [C] (8) and both [P+C] (17). Of the évaluable pts, 7 pts treated with P responded with a decrease in bleeding and inhibitor titer, of which 3 had complete disappearance of the inhibitor. 3/3 évaluable pts treated with C had complete disappearance of inhibitors. All 10 pts treated with P+C, for whom data was available, had disappearance of the inhibitor] 23 pt were alive at last follow up: mean 28 months (0 to 273 months). Hemorrhage! resulted in death of 4/40 pts. Six died of non-hemorrhagic causes and the cause of death in| 4 pts was unknown. Conclusion: Prevalence of aHA is likely higher than commonl)j believed. Delayed Dx probably contributes to mortality and morbidity. Once recognized.1 treatment of acute hemorrhage with appropriate clotting factor concentrates is successful in most of the pts, while long term immuno-suppression with prednisone with or without] cytoxan appears to be effective.

Original languageEnglish (US)
Pages (from-to)268a
JournalBlood
Volume96
Issue number11 PART I
StatePublished - Dec 1 2000

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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