Autoimmune haemolytic anaemias: Diagnosis and treatment

A. Zanella, F. Rossi

Research output: Contribution to journalArticle

Abstract

Autoimmune haemolytic anaemias (AIHA) are a heterogeneous group of disorders characterised by the presence of red cell autoantibodies. In a review covering the last 35 years of clinical data on a total of 7,085 cases, the distribution percentage of various clinical and serological types of AIHA was the following: 72.3% warm antibody AIHA, 22.6% cold haemagglutinin disease, 1.9% paroxysmal cold haemoglobinuria and 3.2% mixed-type AIHA. The overall ratio of primary to secondary cases was 1.04. A positive Coombs' test in the presence of haemolysis is the cornerstone of diagnosis of AIHA; however, Coombs' negative cases have rarely been reported. The main therapeutic strategy in secondary forms is to treat the associated disorder, as this may lead to remission of the haemolytic process. In primary warm antibody AIHA, the standard therapy is based on corticosteroids, followed by splenectomy and cytotoxic drugs. Other treatment modalities include intravenous infusion of immunoglobulins, danazol, plasmapheresis, vinca alkaloids and cyclosporine. In cold antibody AIHA the most efficient treatment is to avoid exposure to low temperatures. Chlorambucil or cyclophosphamide, particularly if combined with plasmapheresis, may be useful in some patients with severe anaemia. interferon α and danazol have been also successfully used in some patients. Blood transfusions should be avoided as far as possible in all types of AIHA; however, if they are necessary, the co-existence of clinically significant alloantibodies must be investigated utilising appropriate techniques.

Original languageEnglish
Pages (from-to)589-604
Number of pages16
JournalFORUM - Trends in Experimental and Clinical Medicine
Volume4
Issue number5
Publication statusPublished - 1994

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Keywords

  • Autoimmune haemolytic anaemias
  • Red cell antibodies
  • Treatment

ASJC Scopus subject areas

  • Medicine(all)

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