TY - JOUR
T1 - Successful response to rituximab in two cases of acquired haemophilia refractory to standard-therapy
AU - Machado, P.
AU - Raya, J. M.
AU - Martín, T.
AU - Morabito, L.
AU - Brito, M. L.
AU - Rodríguez-Martín, J. M.
PY - 2008/6
Y1 - 2008/6
N2 - 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.
AB - 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.
KW - Acquired haemophilia
KW - Coagulation inhibitor
KW - Factor VIII inhibitor
KW - Rituximab
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U2 - 10.1007/s12185-008-0077-4
DO - 10.1007/s12185-008-0077-4
M3 - Article
C2 - 18414981
AN - SCOPUS:46149105198
VL - 87
SP - 545
EP - 549
JO - International Journal of Hematology
JF - International Journal of Hematology
SN - 0925-5710
IS - 5
ER -