Korean Journal of Pediatrics 2007;50(6):511-518.
Published online June 15, 2007.
Hemolytic anemia in pediatrics
Jeong Ok Hah
Department of Pediatrics, College of Medicine, Yeungnam University, Daegu, Korea
소아 용혈빈혈(Hemolytic anemia in pediatrics)
영남대학교 의과대학 소아과학교실
Jeong Ok Hah, Email: johah@med.yu.ac.kr
To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea, HHA due to the RBC membrane defect, hereditary spherocytosis had been reported often but HHA due to hemoglobinopathies and RBC enzymopathies had been thought to be relatively rare. With recent development in the molecular diagnosis, β thalassemia, mostly heterozygote, G6PD and pyruvate kinase deficiency have been reported with gene characterization. If the patients with microcytic hypochromic anemia show unproportionally low MCV or MCH or refractory to the iron therapy, hemoglobin electrophoresis and gene analysis for thalassemia or other unstable hemoglobinopathies need to be done accordingly. The global movement of the population especially from the region prevalent of hemoglobinopathies or enzymopathies to Korea warrants considering broad spectrum of etiology for the diagnosis of HHA. Aquired HA resulting from extracellular factors such as autoimmune HA from warm antibody, cold agglutinin and paroxysmal cold hemoglobinuria as well as nonimmune HA are described briefly.
Key Words: Hereditary hemolytic anemia, RBC membrane defect, Hemoglobinopathy, RBC enzymopathy, Autoimmune hemolytic anemia

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