title: Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients.
abstract: Conventional therapies for primary chronic cold agglutinin disease (CAD) are ineffective, but remissions after treatment with the anti-CD20 antibody rituximab have been described in a small, prospective trial and in some case reports. In this study we report on 37 courses of rituximab administered prospectively to 27 patients. Fourteen of 27 patients responded to their first course of rituximab, and 6 of 10 responded to re-treatment. In both groups combined, responses were achieved after 20 of 37 courses, giving an overall response rate of 54%. We observed 1 complete and 19 partial responses. Two nonresponders and 3 patients who experienced relapse received second-line therapy with interferon-alpha combined with a new course of rituximab, and 1 nonresponder and 2 patients who experienced relapse achieved partial responses. Responders achieved a median increase in hemoglobin levels of 40 g/L (4 g/dL). Median time to response was 1.5 months, and median observed response duration was 11 months. We conclude that rituximab is an effective and well-tolerated therapy for CAD. Histologic and flow cytometric findings suggest that some of the effect may be mediated by mechanisms other than the elimination of clonal lymphocytes. We were unable to predict responses from the hematologic, immunologic, or histologic parameters before therapy.
authors.affiliation: Department of Medicine, Haugesund Hospital, Haugesund, Norway. s.beren@online.no
authors.forename: Sigbjørn
authors.initials: S
authors.lastname: Berentsen
authors.forename: Elling
authors.initials: E
authors.lastname: Ulvestad
authors.forename: Bjørn Tore
authors.initials: BT
authors.lastname: Gjertsen
authors.forename: Henrik
authors.initials: H
authors.lastname: Hjorth-Hansen
authors.forename: Ruth
authors.initials: R
authors.lastname: Langholm
authors.forename: Håvar
authors.initials: H
authors.lastname: Knutsen
authors.forename: Waleed
authors.initials: W
authors.lastname: Ghanima
authors.forename: Fuad Victor
authors.initials: FV
authors.lastname: Shammas
authors.forename: Geir E
authors.initials: GE
authors.lastname: Tjønnfjord
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000188:drug therapy* / Q000276:immunology; D000911:Antibodies, Monoclonal / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D004361:Drug Tolerance; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007370:Interferon Type I / Q000008:administration & dosage / Q000627:therapeutic use; D011446:Prospective Studies; D011994:Recombinant Proteins; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D006454:Hemoglobins; D007075:Immunoglobulin M; D007370:Interferon Type I; D011994:Recombinant Proteins; D000069283:Rituximab