Rituximab in der Behandlung der erworbenen Hämophilie A bei einem Patienten mit Polymyalgia rheumatica

Subscribe to RSS DOI: 10.1055/s-0037-1619069 Rituximab in der Behandlung der erworbenen Hämophilie A bei einem Patienten mit Polymyalgia rheumatica Rituximab in the treatment of acquired haemophilia A in a patient with polymyalgia rheumaticaAuthors Publication History Publication Date: 23 December 2017 (online) Summary Acquired hemophilia A is a rare but potentially life-threatening bleeding disorder. It is caused by the development of autoantibodies directed against coagulation factor VIII in adults or elderly patients, who do not have a personal or family history of bleeding. Case: A man (age: 76 years) on prednisone and leflunomide for polymyalgia rheumatica developed spontaneous severe haematomas. The patient was diagnosed with acquired factor VIII deficiency (FVIII activity 1.2%, FVIII inhibitor 31.7 BU). Due to the active bleeding diathesis, treatment was administered with activated prothrombin complex concentrates (FEIBA®, Baxter). Immunosuppressive treatment with a combination of oral prednisone (1 mg/kg daily) and cyclophosphamide (1,5 mg/kg daily) was administered to reduce the FVIII inhibitor. However, after two weeks of treatment, FVIII was only 3% and no clinical improvement was observed. Treatment with the anti CD20 monoclonal antibody rituximab intravenously at 375 mg/m2 once weekly for four consecutive weeks was started. The patient showed rapid clinical improvement following rituximab treatment. He achieved a complete remission defined as return to normal FVIII activity and undetectable FVIII inhibitor titer. After a follow-up of six months no relapse occurred. Conclusion: Rituximab appears an effective and well-tolerated treatment for patients with acquired haemophilia. Zusammenfassung: Die erworbene Hämophilie A ist eine seltene mit einer lebensbedrohlichen Blutungsneigung einhergehende Gerinnungsstörung. Sie wird verursacht durch Autoantikörper gegen den Gerinnungsfaktor VIII und tritt bei bisher gerinnungsunauffälligen erwachsenen Patienten auf. Kasuistik: Ein 76-jähriger Patient mit Polymyalgia rheumatica entwickelt plötzlich eine spontane Blutungsneigung. In der Gerinnungsdiagnostik fällt eine verminderte Faktor-VIII-Aktivität (1,2%) auf, bei Nachweis eines Faktor-VIII-Inhibitors (31,7 BU) im Bethesda-Assay. Damit wird die Diagnose einer erworbenen Hämophilie A gestellt. Zur Therapie der akuten Blutungssituation erfolgt die Gabe von aktiviertem Prothrombinkomplexkonzentrat (FEIBA®, Baxter). Die immunsuppressive Behandlung wird mit Prednisolon (pro Tag 1 mg/kg) und Cyclophosphamid (pro Tag 1,5 mg/kg) begonnen. Bei fehlendem Ansprechen wird nach zwei Wochen eine Behandlung mit dem CD20-Antikörper Rituximab eingeleitet (375 mg/m2 i. v. wöchentlich für 4 Wochen). Der Patient zeigte rasche klinische Besserung der Blutungsneigung und erreicht eine komplette Remission mit normaler Faktor-VIII-Aktivität bei fehlendem Nachweis eines Hemmkörpers. Sechs Monate nach Ende der Therapie befindet sich der Patient in anhaltender kompletter Remission. Schlussfolgerung: Rituximab ermöglicht eine effektive und gut verträgliche Behandlung der auf Steroide und Cyclophosphamid resistenten erworbenen Hemmkörperhämophilie. - Literatur - 1 Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. J Thromb Haemost 2004; 02: 899-909. - 2 Aggarwal A, Grewal R, Green RJ. et al. Rituximab for autoimmune haemophilia: a proposed treatment algorithm. Haemophilia 2005; 11: 13-19. - 3 Baxter Corp. FEIBA VH Anti-inhibitor coagulant complex Vapor Heated Summary of Product Characteristics. 2005 - 4 Borg JY, Levesque H. for the SACHA study group. 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