New insights in diagnostics and treatment of autoimmune haemolytic anaemia

Abstract

Autoimmune haemolytic anaemia is a rare disease of the immune system characterized by haemolysis of patient’s own red blood cells caused by autoantibodies and/or activated components of the complement. Depending on the underlying disease, autoimmune haemolytic anaemia can be primary or secondary, and according to the serological characteristics of autoantibodies, it is divided into warm, cold and mixed. The diagnosis of the disease is made on the basis of a positive result of a direct antiglobulin test, anaemia and the presence of laboratory parameters for haemolysis. In very rare cases, direct antiglobulin test negative-autoimmune haemolytic anaemias are possible. For the proper treatment, in addition to precise immunohaematological diagnostics to distinguish between certain types of autoimmune haemolytic anaemia, the diagnosis of the underlying diseases is required. Corticosteroids, and in severe cases, rituximab are usually used to treat warm autoimmune haemolytic anaemia. In cold autoimmune haemolytic anaemia warming up a patient is important and avoiding exposure to cold temperatures is recommended, but in severe cases administration of rituximab, with or without bendamustine is required. In refractory cases of paroxysmal cold haemoglobinuria rituximab is used with immunosuppressive drugs. Corticosteroids with early administration of rituximab are recommended for the treatment of mixed autoimmune haemolytic anaemia. In the treatment of symptomatic anaemia blood transfusion is applied, and for the treatment of life-threatened patient it is possible to apply intravenous immunoglobulins, plasmapheresis and in rare cases of reticulocytopenia also erythropoietin. New drugs that are offered in clinical trials are promising for the treatment of severe forms of autoimmune haemolytic anaemias, such as complement-mediated autoimmune haemolytic anaemias or refractory forms of cold autoimmune haemolytic anaemias. For monitoring the therapeutic effect of autoimmune haemolytic anaemias, it is important to know the criteria which define the response to therapy and the outcome of the disease. The aim of this review paper is to present the current knowledge in the diagnostics and treatment of autoimmune haemolytic anaemias, and monitoring the therapeutic effect in autoimmune haemolytic anaemias.

Keywords

• anemia
• hemolytic
• autoimmune – classification
• diagnosis
• therapy; autoantibodies – blood
• immunology; erythrocytes – analysis