Autoimmune Hemolytic Anemia in Children: Diagnostic Approach and Management

Autoimmune Haemolytic Anaemia (AIHA) is not very uncommon in childhood and mainly caused by the presence of auto-antibodies directed against antigens on the surface of red blood cells, leading to premature destruction of the cells. Many children are hospitalized every year due to sequelae of this heterogeneous group of disorders to which pediatric hematologist take a variety of diagnostic and therapeutic approaches. AIHA is generally categorized as "warm" or "cold" based on the thermal reactivity of the autoantibodies and is further classified as primary (idiopathic) or secondary. Early recognition and diagnosis are very important for successful management and outcome of hemolytic anemia. The evaluation of suspected immune hemolytic anemia includes a thorough history, physical examination and laboratory workup assessing for evidence of acute hemolytic anemia, possible diagnosis of an acute event, to evaluate for concurrent causes and to rule out alternative causes.

In this review article we will discuss the classification, diagnostic approach and general management of hemolytic anemia in children especially focusing on newly diagnosed autoimmune hemolytic anemia. Specific recommendations for difficult to diagnosed cases, definitions of response assessment and treatment i.e. dosage and tapering plan of steroid therapy; indications of rituximab, supportive therapy and second-line therapy are discussed.