Giovanni D'arena1, Elvira Grandone2, Matteo N D Di Minno3,4, Pellegrino Musto5, Giovanni Di Minno3. 1. Haematology and Stem Cell Transplantation Unit, IRCCS Cancer Referral Centre of Basilicata, Rionero in Vulture, Italy. 2. Haemostasis and Thrombosis Unit, IRCCS "Casa Sollievo della Sofferenza" Hospital, San Giovanni Rotondo, Italy. 3. Department of Clinical Medicine and Surgery, Regional Reference Centre for Coagulation Disorders, Federico II University, Naples, Italy. 4. Unit of Cell and Molecular Biology in Cardiovascular Diseases. Monzino Cardiology Centre, IRCCS, Milan, Italy. 5. Scientific Direction, IRCCS Cancer Referral Centre of Basilicata, Rionero in Vulture, Italy.
Abstract
BACKGROUND: Acquired haemophilia A (AHA) is a rare bleeding disorder caused by the development of specific autoantibodies against naturally occurring factor VIII (FVIII). Although about half of cases are idiopathic, AHA may be associated with several non-neoplastic conditions, autoimmune disorders, as well as haematological malignancies, such as chronic lymphocytic leukaemia and lymphoma. The long-term suppression of inhibitors is one of the mainstays of the treatment of AHA. Apart from standard immunosuppressive treatments, rituximab has been proven to be effective in AHA. MATERIALS AND METHODS: The aim of this review is to provide a systematic description of data available in the literature on this topic. To do so, we performed a search using the indexed online database Medline/PubMed, without temporal limits, matching the words "rituximab" and "acquired h(a)emophilia". Furthermore, additional published studies were identified in the reference list of the publications found in PubMed. RESULTS: The review of the literature confirms that rituximab may be a safe and useful treatment for AHA. DISCUSSION: Although rituximab is not a standard therapy for AHA, it may be useful in resistant cases. However, the definitive place of this monoclonal antibody in the therapeutic strategy for AHA (first or second-line, alone or in combination with other drugs) remains to be determined more precisely and warrants further investigation.
BACKGROUND: Acquired haemophilia A (AHA) is a rare bleeding disorder caused by the development of specific autoantibodies against naturally occurring factor VIII (FVIII). Although about half of cases are idiopathic, AHA may be associated with several non-neoplastic conditions, autoimmune disorders, as well as haematological malignancies, such as chronic lymphocytic leukaemia and lymphoma. The long-term suppression of inhibitors is one of the mainstays of the treatment of AHA. Apart from standard immunosuppressive treatments, rituximab has been proven to be effective in AHA. MATERIALS AND METHODS: The aim of this review is to provide a systematic description of data available in the literature on this topic. To do so, we performed a search using the indexed online database Medline/PubMed, without temporal limits, matching the words "rituximab" and "acquired h(a)emophilia". Furthermore, additional published studies were identified in the reference list of the publications found in PubMed. RESULTS: The review of the literature confirms that rituximab may be a safe and useful treatment for AHA. DISCUSSION: Although rituximab is not a standard therapy for AHA, it may be useful in resistant cases. However, the definitive place of this monoclonal antibody in the therapeutic strategy for AHA (first or second-line, alone or in combination with other drugs) remains to be determined more precisely and warrants further investigation.
Authors: M Franchini; C Mengoli; G Lippi; G Targher; M Montagnana; G L Salvagno; M Zaffanello; M Cruciani Journal: Haemophilia Date: 2008-07-28 Impact factor: 4.287
Authors: Giovanni D'Arena; Roberto Guariglia; Francesco La Rocca; Stefania Trino; Valentina Condelli; Laura De Martino; Vincenzo De Feo; Pellegrino Musto Journal: Clin Dev Immunol Date: 2013-04-16
Authors: Jennifer Nardella; Domenico Comitangelo; Renato Marino; Giuseppe Malcangi; Marco Damiano Barratta; Carlo Sabba; Antonio Perrone Journal: J Med Cases Date: 2022-04-12