Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
권호 표지

Hemolytic anemia in pediatrics

소아 용혈빈혈(Hemolytic anemia in pediatrics)

  • Hah, Jeong Ok (Department of Pediatrics, College of Medicine, Yeungnam University)
  • 하정옥 (영남대학교 의과대학 소아과학교실)
  • Received : 2007.04.08
  • Accepted : 2007.04.29
  • Published : 2007.06.15
Download PDF
⟨ Previous Next ⟩

Abstract

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, ${\beta}$ 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.

Keywords

References

  1. Ahn DH, Sohn KC, Kang IJ, et al. Statistical analysis of hemolytic anemia in Korea. Korean J Hematol 1991;26:445-61
  2. The Korean Society of Hematology. Hematology. Seoul, Korea: E$\ast$PUBLIC; 2006:101-24
  3. Lee YK, Cho HI, Park SS, Lee YJ, Ra EK, Ahn HS, et al. Abnormalities of erythrocyte membrane proteins in Korean patients with hereditary spherocytosis. J Korean Med Sci 2000;15:284-8 https://doi.org/10.3346/jkms.2000.15.3.284
  4. Park SS, Lee YJ, Kim JY, Joo SI, Hattori Y, Cho HI, et al. $\beta$-Thalassemia in the Korean population. Hemoglobin 2002;26:135-45 https://doi.org/10.1081/HEM-120005451
  5. Lee GB, Lee SJ, Kim YJ, Kim SY, Kim HH, Cho B, .et al. A case of G-6-PD Guadalajara. Korean J Pediatr 2004;47: 210-3
  6. Park-Hah JO, Kanno H, Kim WD, Fujii H. A novel homozygous mutation of PKLR gene in a pyruvate-kinase-deficient Korean family. Acta Haematologica 2005;113:208-11 https://doi.org/10.1159/000084453
  7. Gallagher PG, Lux SE. Disorders of the erythrocyte membrane.In: Nathan DG, Orkin SH, Ginsburg D, Look AT, eds.Hematology of Infancy and Childhood. 6th ed. Philadelphia,PA: Saunders;2003:560-684
  8. Segel GB. Hereditary spherocytosis. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PA:Saunders;2004: 1620-3
  9. Quirolo K, Vichinsky E. Hemoglobin disorders. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PA: Saunders;2004:1623-34
  10. Borgna-Pignatti C, Galanello R. Thalassemias and related disoders: Quantitative disorders of hemoglobin synthesis. In: Greer JP, Foerster J, Lukens JN, Rodgers GM, Paraskevas F, Glader B, eds. Wintrobe's Clinical Hematology. 11th ed. Philadelphia, PA: Lippincott; 2004:1319-65.
  11. Weatherall DJ. The thalassemias. In: Stamatoyannopoulus G. Majerus PW, Perlmutter RM, Varmus H, eds. The Molecular Basis of Blood Diseases. 3rd ed. Philadelphia, PA: saunders; 2001:183-226
  12. Hemoglobin mutation database: http://globin.cse.psu.edu/
  13. Beutler E. Disorders of hemoglobin structure: sickle cell anemia and related abnormalities. In: Lichtman MA, Beutler E, Kipps TJ, Seligsohn U, Kaushansky K, Prchal JT, eds. Williams Hematology. 7th ed. New York: McGraw-Hill; 2006:667-700
  14. Hirono A, Kanno H, Miwa S, Beutler E. Pyruvate kinase deficiency and other enzymopathies of the erythrocyte. Luzzatto L, Mehta A, Vulliamy T. Glucose 6-phosphate dehydrogenase. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 8th ed. McGraw-Hill;2001: 4637-64, 4517-54
  15. Mentzer WC. Pyruvate kinase deficiency and disorders of glycolysis. Luzzatto L. Glucose 6-phosphate dehydrogenase deficiency and hemolytic anemia. In: Nathan DG, Orkin SH, Ginsburg D, Look AT, eds. Hematology of Infancy and Childhood. 6th ed. Philadelphia, PA: Saunders;2003:687-720, 721-42
  16. The Korean Society of Hematology. Hematology. Seoul, Korea: E$\ast$PUBLIC; 2006: 125-35
  17. Packman CH. Hemolytic anemia resulting from immune injury. In: Lichtman MA, Beutler E, Kipps TJ, Seligsohn U, Kaushansky K, Prchal JT, eds. Williams Hematology. 7th ed. New York: McGraw-Hill;2006:729-50
  18. Segel GB. Hemolytic anemias resulting from extracellular factors. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PA: Saunders;2004:1638-40
  19. Flores G, Cunninham-Rundles C, Newland AC, et al. Efficacy of intravenous immunoglobulin in the treatment of autoimmune hemolytic anemia: Results in 73 patients. Am J Hematol 1993;44:237
  20. Sparling TM, Andricevic M, Wass H. Remission of cold hemagglutinin disease induced by rituximab therapy. Can Med Assoc J 2001;164:1405
  21. Segel GB. Hemolytic anemias secondary to other extracellular factors. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 17th ed. Philadelphia, PA: Saunders;2004:1640