title: Diagnosis and treatment of cold agglutinin mediated autoimmune hemolytic anemia.
abstract: Exact diagnosis of the subtype has essential therapeutic consequences in autoimmune hemolytic anemia. Cold-antibody types include primary chronic cold agglutinin disease (CAD) and rare cases of cold agglutinin syndrome (CAS) secondary to cancer or acute infection. Primary CAD is a clonal lymphoproliferative disorder. Not all patients require pharmacological therapy, but treatment seems indicated more often than previously thought. Corticosteroids should not be used to treat primary CAD. Half of the patients respond to rituximab monotherapy; median response duration is 11 months. The most efficient treatment to date is fludarabine and rituximab in combination, resulting in responses in 75%, complete responses in 20% and median response duration of more than 66 months. Toxicity may be a concern, and an individualized approach is discussed. Erythrocyte transfusions can be given provided specific precautions are undertaken. No evidence-based therapy exists in secondary CAS, but optimal treatment of the underlying disorder is essential when feasible.
authors.affiliation: Department of Medicine, Haugesund Hospital, Norway. sigbjorn.berentsen@haugnett.no
authors.forename: Sigbjørn
authors.initials: S
authors.lastname: Berentsen
authors.forename: Geir E
authors.initials: GE
authors.lastname: Tjønnfjord
mesh_terms: D000305:Adrenal Cortex Hormones; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000744:Anemia, Hemolytic, Autoimmune / Q000145:classification / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D058846:Antibodies, Monoclonal, Murine-Derived / Q000009:adverse effects / Q000627:therapeutic use*; D001323:Autoantibodies / Q000276:immunology*; D000075202:Contraindications; D003450:Cryoglobulins / Q000276:immunology; D004338:Drug Combinations; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D008875:Middle Aged; D057285:Precision Medicine; D012074:Remission Induction; D000069283:Rituximab; D014740:Vidarabine / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D003450:Cryoglobulins; D004338:Drug Combinations; C029443:cold agglutinins; D000069283:Rituximab; D014740:Vidarabine; C024352:fludarabine