title: Beneficial effect of rituximab in combination with oral cyclophosphamide in primary chronic cold agglutinin disease.
abstract: Cold agglutinin disease (CAD) is an uncommon autoimmune hemolytic anemia characterized by B-cell proliferation. Conventional therapies for primary CAD such as corticosteroids, oral alkylating agents, splenectomy, interferon alpha, and plasma exchange are often ineffective at controlling the disease. The anti-CD20 monoclonal antibody rituximab (MabThera) depletes B-lymphocytes and thereby interferes with the production of cold agglutinin. We describe an elderly patient with primary (idiopathic) chronic CAD refractory to steroids who was successfully treated with 4 weekly infusions (375 mg/m2) of rituximab and 6 months of oral cyclophosphamide at a dosage of 60 mg/m2 per day. The increase in hemoglobin level and the decline in the plasma cold agglutinin titer were rapid (from the second rituximab infusion). The hematologic remission persisted for at least 8 months after treatment start, with no adverse effects. Rituximab and cyclophosphamide may be supplementary therapeutic modalities whose combination warrants further clinical investigation.
authors.affiliation: Department of Hematology, Ioannina University Hospital, Panepistimiou Ave, 45110 Ioannina, Greece. penther@ukonline.co.uk
authors.forename: A
authors.initials: A
authors.lastname: Vassou
authors.forename: V
authors.initials: V
authors.lastname: Alymara
authors.forename: A
authors.initials: A
authors.lastname: Chaidos
authors.forename: K L
authors.initials: KL
authors.lastname: Bourantas
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D012074:Remission Induction; D000069283:Rituximab; D016879:Salvage Therapy
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D003520:Cyclophosphamide