Diagnostic and therapeutic challenges of primary autoimmune haemolytic anaemia in children

Autoimmune haemolytic anaemias (AIHAs) are extracorpuscular haemolytic anaemias produced by antierythrocyte autoantibodies which cause a shortened red blood cell life span. There are several reasons why the diagnosis and treatment of AIHAs in children represent a bigger challenge than in adult patients, including the presence of particular AIHA types, the uncertainty of serological tests and the limited clinical experience. All these facts have added up to a poor understanding and management of some topics in childhood AIHA. We discuss some of these questions, for example, the occurrence of AIHA with negative direct antiglobulin (Coombs) test, the correct diagnosis and actual incidence of paroxysmal cold haemoglobinuria, the most appropriate second-line therapy of AIHA in childhood or the management of transfusion procedures in these patients. This review takes a practical point of view, providing with some ground rules on how to identify and deal with these paediatric patients.