Document Detail

Acquired hemophilia a: diagnosis, aetiology, clinical spectrum and treatment options.
MedLine Citation:
PMID:  21115138     Owner:  NLM     Status:  MEDLINE    
Acquired hemophilia A (AHA) is a rare disorder with an incidence of approximately 1 per million/year with a high mortality rate of more than 20%. The disease occurs due to autoantibodies against coagulation factor VIII (FVIII) which neutralize its procoagulant function and result in severe, often life-threatening bleeding. The antibodies arise in individuals with no prior history of hemophilia A. AHA may be associated with pregnancy, autoimmune diseases, malignancy, infections or medication and occurs most commonly in the elderly. Approximately 50% of the patients remain idiopathic with no known underlying pathological condition. Clinical manifestations include spontaneous hemorrhages into the skin, muscles or soft tissues or excessive bleeding during surgery. Hemarthrosis which is the hallmark of congenital severe hemophilia A seldom occurs in AHA. The diagnosis of AHA is based on the isolated prolongation of activated partial thromboplastin time (APTT) which does not normalize after the addition of normal plasma along with reduced FVIII levels. The treatment involves two aspects-eradication of antibodies and maintaining effective hemostasis during a bleeding episode. The protocols for eradication of antibodies include immunoadsorption, immunosuppression or immune tolerance induction (ITI). The treatment of acute bleeding episodes involves use of different bypassing agents like recombinant activated factor VIIa (rFVIIa, NovoSeven®) and activated prothrombin complex concentrate (aPCC, (FEIBA®) in case of patients with high titer inhibitors or with antifibrinolytics,1-deamino-8-D-arginine vasopressin (DDAVP) or FVIII concentrates in low titer inhibitor patients. The anti CD20 monoclonal antibody, rituximab, has shown very good results either singly or in combination with immunosuppressive regimens in patients who do not respond to standard immunosuppressors. The present review summarizes the diagnostic, aetiological, clinical and treatment aspects of AHA focusing on the recent advances in this area.
Shrimati Shetty; Manali Bhave; Kanjaksha Ghosh
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2010-11-27
Journal Detail:
Title:  Autoimmunity reviews     Volume:  10     ISSN:  1873-0183     ISO Abbreviation:  Autoimmun Rev     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-04-04     Completed Date:  2011-07-18     Revised Date:  2011-12-06    
Medline Journal Info:
Nlm Unique ID:  101128967     Medline TA:  Autoimmun Rev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  311-6     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier B.V. All rights reserved.
National Institute of Immunohematology ( ICMR), 13th Floor, KEM Hospital, Parel, Mumbai 400 012, India.
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MeSH Terms
Autoimmune Diseases* / diagnosis,  etiology,  physiopathology,  therapy
Hemophilia A* / diagnosis,  etiology,  physiopathology,  therapy
Comment In:
Autoimmun Rev. 2011 Nov;11(1):74; author reply 75   [PMID:  21803174 ]

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