Acquired hemophilia A: clinical features, surgery and treatment of 34 cases, and experience of using recombinant factor VIIa.
Acquired hemophilia A is rare, but life-threatening disorder caused by autoantibody against factor VIII. As it is useful to gather more data on epidemiology, clinical pictures and therapy of it, we evaluated relevant medical findings in 34 acquired hemophiliacs from Dec 1999 to Dec 2007. Eight patients (23.5%) had low titers (<10 Bethesda Unit BU) and 26 patients (76.5%) had high titers of inhibitors (>10 BU). The mean of inhibitors was 548.38 +/- 359.27 SD BU. The most common hemorrhagic symptoms were hematoma 21 (33.33%), ecchymosis 16 (25.39%), hemarthrosis 8 (12.69%), hematuria 6 (9.52%), menorrhagia 4 (6.34%), compartment syndrome 3 episodes (4.76%). The eliminator therapies were recruited according to titers of inhibitor and types of bleeding and it's results were 27 efficient treatments (79.4%), 5 partial efficient treatment (14.7%) and two treatments inefficient (5.9%). Elimination therapy using steroid alone or with combination can terminate complete remission in most cases.
Lak
Manijeh
M
Hematology Department, Hemophilia center, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Sharifian
Ramezan Ali
RA
Karimi
Katayon
K
Mansouritorghabeh
Hassan
H
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000453:epidemiology*; D016063:Blood Loss, Surgical / Q000517:prevention & control; D003161:Compartment Syndromes / Q000209:etiology / Q000517:prevention & control / Q000601:surgery; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005169:Factor VIII / Q000276:immunology; D015942:Factor VIIa / Q000627:therapeutic use*; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology; D006470:Hemorrhage / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007492:Iran / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000097:blood / Q000150:complications; D006473:Postpartum Hemorrhage / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology; D011239:Prednisolone / Q000627:therapeutic use; D011994:Recombinant Proteins / Q000627:therapeutic use; D012189:Retrospective Studies; D055815:Young Adult
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D003520:Cyclophosphamide; D005169:Factor VIII; D011239:Prednisolone; C103587:recombinant FVIIa; D015942:Factor VIIa