[Rituximab in the treatment of acquired haemophilia A in a patient with polymyalgia rheumatica].
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.


METHODS
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&reg;, 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.


CONCLUSIONS
Rituximab appears an effective and well-tolerated treatment for patients with acquired haemophilia.
Department für Innere Medizin, selbstständige Abteilung für Hämatologie, Internistische Onkologie und Hämostaseologie, Johannisallee 32 A, 04103 Leipzig. Leanthe.braunert@medizin.uni-leipzig.de
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D000368:Aged; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007555:Isoxazoles / Q000627:therapeutic use; D000077339:Leflunomide; D008297:Male; D011111:Polymyalgia Rheumatica / Q000150:complications* / Q000209:etiology; D011241:Prednisone / Q000627:therapeutic use; D000069283:Rituximab; D016896:Treatment Outcome
D000894:Anti-Inflammatory Agents, Non-Steroidal; D058846:Antibodies, Monoclonal, Murine-Derived; D007555:Isoxazoles; D000069283:Rituximab; D003520:Cyclophosphamide; D000077339:Leflunomide; D011241:Prednisone