[Autoimmune hemolytic anemia].
BACKGROUND
Knowledge of autoimmune hemolytic anemia is rapidly increasing, especially on pathogenesis, associated disorders and aspects of transfusion immunology. New treatment options have emerged.


METHODS
This review is based on selected publications, a non-systematic search in PubMed and the authors' own research.


RESULTS
Autoimmune hemolytic anemia is a heterogeneous group of diseases. The warm-antibody type is frequently associated with chronic lymphocytic leukemia or autoimmune systemic disease. Corticosteroids are still the first-line therapy and splenectomy is second-line, while rituximab has become an option in refractory disease. Blood transfusions require specific precautions such as restrictive use, extensive phenotyping of antigens and testing for alloantibodies and biological compatibility. Primary chronic cold agglutinin disease, a subgroup of cold-antibody autoimmune hemolytic anemia, is a clonal lymphoproliferative bone marrow disease. This subgroup is usually refractory to corticosteroids and patients with few clinical symptoms and slight anemia may not require drug therapy. However, in a majority, effective drugs are needed. The best documented therapy is infusions with rituximab, but new options are being tested. Immune hemolytic anemia associated with drugs occurs less frequently now than before.


CONCLUSIONS
Subgroups and associated or underlying disease should be identified in patients with autoimmune hemolytic anemia. The therapeutic implications of subclassification are important. Patients who have cold agglutinin disease and need therapy should be included in prospective trials.
Berentsen
Sigbjørn
S
Medisinsk klinikk, Haugesund sjukehus, Helse Fonna, 5504 Haugesund, Norway. sigbjorn.berentsen@haugnett.no
Sundic
Tatjana
T
Hervig
Tor
T
Tjønnfjord
Geir E
GE
D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000628:therapy; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000097:blood; D001803:Blood Transfusion; D002648:Child; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D000069283:Rituximab
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D000069283:Rituximab; D011239:Prednisolone