A survey of patients with acquired haemophilia in a haemophilia centre over a 28-year period.
Data of patients with acquired inhibitors to clotting factors seen in a haemophilia centre from 1970-98 was collected. Twenty-four patients with anti-factor VIII antibodies and four with acquired von Willebrand disease case records were evaluable. Two-thirds of the patients were females and their age ranged from 2 to 92 years (median 69). There was no correlation between the inhibitor titres and the severity or the pattern of bleeding. Porcine factor VIII and activated prothrombin complex were both effective for acute bleeds. Prednisolone and cyclophosphamide proved valuable for suppression of the antibodies without adverse effects even in the elderly group of patients. Three deaths were directly related to bleeding. This review has shown the life-threatening nature of this acquired bleeding disorder, its variable clinical presentation and the importance of early referral to a specialist centre for appropriate therapy.
Yee
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Katharine Dormandy Haemophilia Centre and Haemostasis Unit, Department of Haematology, Royal Free and University College Medical School, University College London, UK. rfhm0003@rfhsm.ac.uk
Taher
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A
Pasi
K J
KJ
Lee
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CA
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000818:Animals; D000906:Antibodies / Q000097:blood; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003625:Data Collection; D005169:Factor VIII / Q000276:immunology / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000191:economics / Q000628:therapy*; D006470:Hemorrhage; D006784:Hospitals, Teaching / Q000706:statistics & numerical data*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D012189:Retrospective Studies; D013552:Swine; D013997:Time Factors
D000906:Antibodies; D001779:Blood Coagulation Factors; D016756:Immunoglobulins, Intravenous; D011994:Recombinant Proteins; D005169:Factor VIII; C065655:anti-inhibitor coagulant complex; D015942:Factor VIIa