Acquired hemophilia A: Clinical features, surgery and treatment of 34 cases, and experience of using recombinant factor VIIa

Lak, M. and Sharifian, R. A. and Karimi, K. and Mansouritorghabeh, H. (2010) Acquired hemophilia A: Clinical features, surgery and treatment of 34 cases, and experience of using recombinant factor VIIa. Clinical and Applied Thrombosis/Hemostasis, 16 (3). pp. 294-300.

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Abstract

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. © 2010 The Author(s).

Item Type: Article
Additional Information: Cited By :25 Export Date: 16 February 2020 CODEN: CATHF Correspondence Address: Mansouritorghabeh, H.; Immunology Research Center, Ghaem Hospital, Mashhad University of Medical Sciences, Mashhad 91766-99199, Iran; email: Mansouritorghabe@mums.ac.ir
Uncontrolled Keywords: Acquired hemophilia A Factor VIII inhibitor Hemorrhage Inhibitor elimination Surgery management activated prothrombin complex autoantibody blood clotting factor 8 cyclophosphamide immunoglobulin prednisolone recombinant blood clotting factor 7a steroid tranexamic acid absence of side effects adult aged antibody titer article bleeding clinical article clinical feature compartment syndrome ecchymosis female hemarthrosis hematoma hematuria hemophilia A human male menorrhagia priority journal rare disease remission single drug dose symptom treatment indication treatment outcome Adolescent Aged, 80 and over Autoimmune Diseases Blood Loss, Surgical Compartment Syndromes Drug Therapy, Combination Factor VIIa Factor VIII Humans Immunoglobulins, Intravenous Immunosuppressive Agents Iran Middle Aged Neoplasms Postpartum Hemorrhage Recombinant Proteins Retrospective Studies Young Adult
Subjects: WO Surgery
Divisions: Mashhad University of Medical Sciences
Depositing User: mr lib4 lib4
Date Deposited: 02 Mar 2020 04:43
Last Modified: 02 Mar 2020 04:43
URI: http://eprints.mums.ac.ir/id/eprint/13069

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