Successful response to rituximab in two cases of acquired haemophilia refractory to standard-therapy

• Published in: International journal of hematology Vol. 87; no. 5; pp. 545 - 549
• Main Authors: Machado, P., Raya, J. M., Martín, T., Morabito, L., Brito, M. L., Rodríguez-Martín, J. M.
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 01.06.2008; Springer; Springer Nature B.V
• Subjects: Adult; Antibodies, Monoclonal - administration & dosage; Antibodies, Monoclonal, Murine-Derived; Autoantibodies - blood; Autoantibodies - immunology; Biological and medical sciences; Blood Coagulation Factor Inhibitors - blood; Blood Coagulation Factor Inhibitors - immunology; Case Report; Factor VIII - antagonists & inhibitors; Factor VIII - immunology; Female; Hematologic and hematopoietic diseases; Hematology; Hemophilia A - blood; Hemophilia A - drug therapy; Hemophilia A - immunology; Humans; Immunologic Factors - administration & dosage; Male; Medical sciences; Medicine; Medicine & Public Health; Middle Aged; Oncology; Platelet diseases and coagulopathies; Rituximab; Rituximab; Factor VIII inhibitor; Acquired haemophilia; Coagulation inhibitor; Antineoplastic agent; Blood coagulation; Treatment resistance; Hematology; Factor VIII inhibitor, Rituximab; Acquired; Factor VIII; Hemopathy; Monoclonal antibody; Standards; Genetic disease; Treatment; Immunotherapy; Hemophilia; Inhibitor; Acquired haemophilia, Coagulation inhibitor; Coagulation factor; Coagulopathy
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-008-0077-4

Acquired autoantibodies against coagulation factors (acquired haemophilia) frequently constitute a life-threatening bleeding situation requiring a prompt therapeutic intervention, including control of bleeding and secondarily an attempt of eradication of the inhibitor by prolonged immunosuppressive therapy. The combination of oral corticosteroids and cyplophosphamide seems to be effective to eradicate the autoantibody, but some patients may be resistant. Another therapeutic approach, recently described, observes treatment with the chimeric anti-CD20 monoclonal antibody rituximab. We report two consecutively treated patients whose acquired FVIII inhibitors did not respond to standard immunosuppressive regimens, and only when rituximab was added to therapy, complete response and prolonged remission were obtained.