title: Cold agglutinin-mediated autoimmune hemolytic anemia.
abstract: Cold antibody types account for about 25% of autoimmune hemolytic anemias. Primary chronic cold agglutinin disease (CAD) is characterized by a clonal lymphoproliferative disorder. Secondary cold agglutinin syndrome (CAS) complicates specific infections and malignancies. Hemolysis in CAD and CAS is mediated by the classical complement pathway and is predominantly extravascular. Not all patients require treatment. Successful CAD therapy targets the pathogenic B-cell clone. Complement modulation seems promising in both CAD and CAS. Further development and documentation are necessary before clinical use. We review options for possible complement-directed therapy.
authors.affiliation: Department of Medicine, Haugesund Hospital, Karmsundgata 120, Haugesund NO-5504, Norway. Electronic address: sigbjorn.berentsen@haugnett.no.
authors.forename: Sigbjørn
authors.initials: S
authors.lastname: Berentsen
authors.affiliation: Department of Pathology, Oslo University Hospital, Ullernchausseen 70, NO-0310 Oslo, Norway.
authors.forename: Ulla
authors.initials: U
authors.lastname: Randen
authors.affiliation: Department of Haematology, Oslo University Hospital, Institute of Clinical Medicine, University of Oslo, Sognsvannsveien 20, NO-0372 Oslo, Norway.
authors.forename: Geir E
authors.initials: GE
authors.lastname: Tjønnfjord
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology*; D003171:Complement Pathway, Classical / Q000276:immunology; D003165:Complement System Proteins / Q000276:immunology; D003450:Cryoglobulins / Q000276:immunology; D006461:Hemolysis / Q000276:immunology; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000276:immunology*; D018448:Models, Immunological
chemical_list: D003450:Cryoglobulins; C029443:cold agglutinins; D003165:Complement System Proteins
keywords: Autoimmune hemolytic anemia; B lymphocytes; Cold agglutinin; Cold agglutinin disease; Cold agglutinin syndrome; Complement; Lymphoproliferative disorders; Therapy