Autoimmune haemolytic anaemia

Autoimmune haemolytic anaemia (AIHA) can be classified as warm or cold type and may be either idiopathic (primary) or secondary to haematological malignancy, infection or other conditions that result in immune dysregulation. Presentation can vary from the gradual onset of symptomatic anaemia, to an acute fulminant episode of life‐threatening haemolysis. Management, which is not evidence based, largely entails the use of initial corticosteroids for primary warm‐type AIHA with either splenectomy, rituximab or alternative immunosuppression for those requiring second‐line therapy. Rituximab is now considered a first‐line therapy for primary cold haemagglutinin disease. Transfusion may be needed as a supportive measure particularly with severe acute haemolysis and requires close liaison between clinical and laboratory teams to ensure appropriate selection of red cell units in a timely manner.