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Immune tolerance with rituximab in congenital haemophilia with inhibitors: a systematic literature review based on individual patients’ analysis
Author(s) -
FRANCHINI M.,
MENGOLI C.,
LIPPI G.,
TARGHER G.,
MONTAGNANA M.,
SALVAGNO G. L.,
ZAFFANELLO M.,
CRUCIANI M.
Publication year - 2008
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/j.1365-2516.2008.01839.x
Subject(s) - medicine , rituximab , immunosuppression , haemophilia , cd20 , concomitant , haemophilia a , adverse effect , oncology , immunology , pediatrics , antibody
Summary. Rituximab, a monoclonal antibody against the pan B‐cell antigen CD20, has been successfully used in both adults and children for the management of malignant and non‐malignant immune‐mediated disorders including acquired haemophilia. On the basis of this positive experience, a number of investigators have recently used this agent in patients with congenital haemophilia and inhibitors refractory to first‐line treatments. After a careful electronic and hand search, we have collected 29 studies that included 49 cases. A durable complete remission was obtained in 53% of the cases and no severe adverse events related to rituximab were recorded. A multivariate analysis applied to individual patients’ data identified the diagnosis of a mild/moderate haemophilia and the concomitant treatment with factor VIII concentrates and immunosuppression agents as covariates associated with an increased response to rituximab. Large prospective randomized studies with an adequate follow‐up are needed to confirm these preliminary findings.