Abstract
The treatment of patients with acquired hemophilia is challenging due to life-threatening hemorrhages, delayed response, and adverse effects to immunosuppressive agents. Even though immunoadsorption (IA) rapidly removes autoantibodies against factor VIII, this intervention's effectiveness is still a matter of debate. We aimed to study important outcomes of IA as adjunctive treatment in patients with acquired hemophilia. We performed comprehensive literature searches in MEDLINE and EMBASE databases. Clinical and laboratory data of all patients treated in our institution were additionally included. Literature searching yielded 498 records, of which 10 studies describing 106 patients were finally included. The number of patients varied from 1 to 65, and patients' ages ranged between 14 and 89. Treatment criteria in most patients were (1) failed response to immunosuppressive treatment alone, and/or (2) uncontrollable bleeding episodes, and/or (3) high inhibitor titer. Methodological quality was moderate. The number of IA sessions varied from 1 to 24. Within our institution, 12 patients have been treated since 2002; median age was 76 years (range 34-86); median titer of factor VIII inhibitor was 20 Bethesda units (range 3-214). Pooled estimates, modeling a random-effect binominal distribution incorporating the Freeman-Tukey double arcsine transformation, were 86% in case of factor VIII recovery (95% confidence interval 76%-94%), 95% for reduction of factor VIII inhibitor (83%, 100%), and 7% in case of death (0%, 18%). Our data suggest that IA might be a beneficial adjunctive treatment in patients with high-risk acquired hemophilia, but future studies shall confirm this observation.
Highlights
Treatment of patients with acquired hemophilia is challenging
Cyclophosphamide was used in addition to steroids as immunosuppressive treatment in all patients
Other factor concentrates (recombinant factor VIIa, activated prothrombin complex concentrate, FVIII concentrate), and intravenous immunoglobulin G were used in some patients
Summary
Treatment of patients with acquired hemophilia is challenging. Patients present with large ecchymosis, extensive muscle hemorrhages, gastrointestinal bleeding, or even intracerebral hemorrhage [1,2,3]. The mortality is high because of bleeding complications and treatment complications such as infection [4,5]. The pathogenesis of acquired hemophilia is heterogeneous. The most common associated conditions are malignancy [6,7], autoimmune disorder [8,9,10,11], drug-induced [12,13], or postpartum status [14,15]. Factors that predict improved overall survival are the achievement of complete remission, age below
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