Rituximab in the Treatment of Acquired Hemophilia

Department of Pharmacy Practice, College of Pharmacy, University of Illinois Medical Center at Chicago, 60612-7229, USA.
Annals of Pharmacotherapy (Impact Factor: 2.06). 07/2006; 40(6):1151-7. DOI: 10.1345/aph.1G658
Source: PubMed


To review published literature evaluating the use of rituximab for treatment of acquired hemophilia.
An English-language literature search was conducted using MEDLINE (1966-January 2006). References of identified articles were subsequently reviewed for additional data. Search terms included rituximab, acquired hemophilia, and inhibitors.
Available data suggest that B-cell depletion by rituximab in patients with acquired hemophilia may contribute to the eradication of inhibitors to coagulation factors. B-cells have been shown to be essential in the development of autoimmunity or an acquired immune response. Beneficial effects of rituximab, after failure of established therapies, have been reported in case reports and one small, open-label trial.
Although data are limited, administration of rituximab appears to be an effective option for treatment of patients with acquired hemophilia after established therapies have failed. Patients given rituximab experienced cessation of recurrent bleeding, normalization of factor VIII activity, and eradication of inhibitors. A complete response to rituximab (undetectable inhibitors, normalization of factor levels) generally occurred several weeks to months after initiation of therapy.

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    • "The exact mechanism of action of intravenous immunoglobulins (IVIG) is not fully understood, however possible mechanisms proposed include neutralization caused by the presence of anti-idiotype antibodies, suppression of inhibitor production and temporary displacement of inhibitors. A total dose of 2 g/kg over 2 or 5 days induced complete or partial remission in 30% of patients particularly those with low titers.62 Multiple courses are needed to obtain a sustained response. "
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    ABSTRACT: Acquired haemophilia A is a serious and potentially fatal bleeding disorder. Diagnosis is difficult and maybe delayed due to its rarity. The high mortality rate and the complex nature of treatment necessitate patient management at a haemophilia centre, where the required expertise and resources are available. Prompt diagnosis is crucial and early initiation of therapy could be life saving. Management includes initial control of bleeding followed by an approach to eradicate the coagulation factor inhibitor. In this paper we describe our local experience with acquired haemophilia A, which resulted in the successful control of major bleeding at presentation and eradication of inhibitors.
    Full-text · Article · Jan 2012 · Mediterranean Journal of Hematology and Infectious Diseases
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    • "As compared with acquired haemophilia, which is always due to auto-antibodies against FVIII, the pathogenic mechanisms underlying AVWS are more heterogeneous. Previous reports have shown that rituximab is an option for the correction of acquired haemophilia (Stachnik, 2006). For our two patients with AVWS, rituximab was initiated 1 month after the last administration of IVIg, as an alternative treatment, expecting a correction of their disease and the FVIII/VWF complex. "

    Preview · Article · Mar 2009 · British Journal of Haematology
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    ABSTRACT: Hemophilia A is classically caused by a congenital deficiency of factor VIII, but an acquired form due to inhibitors to factor VIII (FVIII) typically presents later in life. Patients who develop such acquired factor VIII inhibitors may present with catastrophic bleeding episodes, despite having no prior history of a bleeding disorder. Though the disorder is rare, it is known to cause significant morbidity and mortality. This review will focus on what is currently known about acquired hemophilia A, its pathogenesis, its associated etiologies, and its treatment.
    Full-text · Article · Feb 2006 · Hematology
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