Autoimmune hemolytic anemia is a relatively uncommon pediatric clinical condition. As such, the evaluation and management of these cases can be challenging for even the most seasoned pediatrician. In this review, the 3 major forms of autoimmune hemolytic anemia in children will be discussed: warm autoimmune hemolytic anemia, cold agglutinin disease, and paroxysmal cold hemoglobinuria. After a general description of the laboratory approach to these entities, the pathophysiology of these disease processes, including important disease associations, will be described, and treatment strategies will be discussed. This will provide the reader with a rational approach to identifying and managing pediatric patients with these uncommon autoimmune conditions.
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