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

Abstract

UNLABELLED:

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.

• Authors: L Braunert, M Bruegel, C Pfrepper, J Thiery, D Niederwieser
• Journal: Hamostaseologie (Hamostaseologie) Vol. 30 Suppl 1 Pg. S40-3 (Nov 2010) ISSN: 0720-9355 [Print] Germany
• Vernacular Title: Rituximab in der Behandlung der erworbenen Hämophilie A bei einem Patienten mit Polymyalgia rheumatica.
• PMID: 21042682 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Inflammatory Agents, Non-Steroidal
• Antibodies, Monoclonal, Murine-Derived
• Isoxazoles
• Rituximab
• Cyclophosphamide
• Leflunomide
• Prednisone

Topics

• Aged
• Anti-Inflammatory Agents, Non-Steroidal (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Cyclophosphamide (therapeutic use)
• Hemophilia A (drug therapy, etiology)
• Humans
• Isoxazoles (therapeutic use)
• Leflunomide
• Male
• Polymyalgia Rheumatica (complications, etiology)
• Prednisone (therapeutic use)
• Rituximab
• Treatment Outcome