Acquired autoimmune haemophilia: Where is the place of rituximab in the treatment strategy? Reflection from a monocentric series of 8 patients and literature review

• Published in: La revue de medecine interne 2019-09, Vol.40 (9), p.574-580
• Main Authors: Grossin, D, Broner, J, Arnaud, E, Goulabchand, R, Gris, J C
• Format: Article
• Language: fre
• Subjects: Adult; Aged; Aged, 80 and over; Autoimmune Diseases - drug therapy; Female; Hemophilia A - drug therapy; Hemophilia A - immunology; Humans; Immunologic Factors - therapeutic use; Male; Middle Aged; Retrospective Studies; Rituximab - therapeutic use; Treatment Outcome
• ISSN: 1768-3122
• DOI: 10.1016/j.revmed.2019.02.008

Autoimmune acquired haemophilia is a rare autoimmune disease. The purpose of immunosuppressive therapy is to stop the production of autoantibodies that inhibit clotting factors VIII or IX. A corticosteroids-cyclophosphamide combination is recommanded as first-line therapy. From our experience at the University Hospital of Nîmes, we discuss the place of rituximab in the therapeutic arsenal. We report a monocentric observational retrospective study. Our data are discussed in light of literature data, in particular cohorts EACH2 and SACHA. Eight patients (7 with FVIII anibodies) were consecutively included from 2005. The average age was 68.5 years with a male predominance (62.5%). Bleeding manifestations were usually spontaneous and superficial. A pathology, mostly autoimmune or neoplastic, was associated in 5/8 patients. A "by-pass" haemostatic treatment was prescribed for 3/8 patients. Rituximab was prescribed for 5/8 patients, three times as first-line therapy, and always associated with corticosteroids. Three patients received a cyclophosphamid/cortisone combination, two were treated exclusively with oral corticosteroids. Remission was obtained in all patients, without subsequent relapse. The average time to obtain remission under rituximab (after the first injection) was 32.5 days (10-143). The results observed in our series of patients are consistent with the data from the literature. Rituximab appears to be an effective and well-tolerated treatment for autoimmune acquired haemophilia. However, its place remains to be specified.