title: Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients.
abstract: Patients with common variable immunodeficiency (CVID) are at high risk of developing immune thrombocytopenia (ITP) and/or autoimmune haemolytic anaemia (AHA). Given their underlying immunodeficiency, immunosuppressive treatment of these manifestations may increase the risk of infection. To assess efficacy and safety of rituximab in patients with CVID-associated ITP/AHA, a multicentre retrospective study was performed. Thirty-three patients, 29 adults and four children, were included. Patients received an average of 2·6 treatments prior to rituximab including steroids, intravenous immunoglobulin and splenectomy (21%). The median ITP/AHA duration at time of first rituximab administration was 12 months [range 1-324] and the indication for using rituximab was ITP (22 cases), AHA (n = 5) or both (n = 7); 1 patient was treated sequentially for ITP and then AHA. The overall initial response rate to rituximab was 85% including 74% complete responses. After a mean follow-up of 39 ± 30 months after rituximab first administration, 10 of the initial responders relapsed and re-treatment with rituximab was successful in 7/9. Severe infections occurred after rituximab in eight adults (24%), four of whom were not on immunoglobulin replacement therapy. In conclusion, rituximab appears to be highly effective and relatively safe for the management of CVID-associated severe immune cytopenias.
authors: Department of Internal Medicine, Hôpital Henri Mondor, Créteil, Paris, France.
authors: Delphine
authors: D
authors: Gobert
authors: James B
authors: JB
authors: Bussel
authors: Charlotte
authors: C
authors: Cunningham-Rundles
authors: Lionel
authors: L
authors: Galicier
authors: Agnès
authors: A
authors: Dechartres
authors: Alice
authors: A
authors: Berezne
authors: Bernard
authors: B
authors: Bonnotte
authors: Thierry
authors: T
authors: DeRevel
authors: Christophe
authors: C
authors: Auzary
authors: Roland
authors: R
authors: Jaussaud
authors: Claire
authors: C
authors: Larroche
authors: Alain
authors: A
authors: LeQuellec
authors: Marc
authors: M
authors: Ruivard
authors: Pascal
authors: P
authors: Seve
authors: Amar
authors: A
authors: Smail
authors: Jean-François
authors: JF
authors: Viallard
authors: Bertrand
authors: B
authors: Godeau
authors: Olivier
authors: O
authors: Hermine
authors: Marc
authors: M
authors: Michel
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000188:drug therapy* / Q000276:immunology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D017074:Common Variable Immunodeficiency / Q000097:blood / Q000188:drug therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D000069283:Rituximab; D013921:Thrombocytopenia / Q000188:drug therapy* / Q000276:immunology; D055815:Young Adult
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab