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Congenital Hemolytic Anemias

  • Xylina Gregg
  • Archana Agarwal
  • Josef T. PrchalEmail author
Chapter

Abstract

Congenital hemolytic anemias are due to defects affecting the red cell membrane, red cell enzymes, hemoglobin structural properties, or hemoglobin synthesis. The clinical picture depends upon the severity of the defects, the duration of hemolysis, and the exposure to a hemolysis-precipitating or hemolysis-augmenting event and ranges from asymptomatic to life-threatening anemia. Even fully compensated non-anemic congenital hemolytic conditions increase the risk of gallstones and erythroferrone/hepcidin-mediated iron overload and its associated complications.

Keywords

Hemolysis Anemia Splenomegaly Spherocytosis Elliptocytosis Pyropoikilocytosis Glucose-6-phosphate dehydrogenase Pyruvate kinase 5′ Nucleotidase Thalassemias Sickle cell disease 

Suggested Reading

  1. 1.
    Kaushansky K, Lichtman MA, Prchal JT, Levi M, Press OW, Burns LJ, Caligiuri MA, editors. Williams hematology. 9th ed. New York: McGraw Hill; 2015. ISBN 978-0-07-183300-4Google Scholar
  2. 2.
    Kaushansky K, Lichtman MA, Prchal JT, Levi M, Press OW, Burns LJ, Caligiuri MA, editors. Williams manual of hematology. 9th ed. New York: McGraw Hill; 2016. ISBN 978-1-259-64247-0Google Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Xylina Gregg
    • 1
  • Archana Agarwal
    • 2
  • Josef T. Prchal
    • 3
    Email author
  1. 1.Hematology and Medical OncologyUtah Cancer SpecialistsSalt Lake CityUSA
  2. 2.Department of PathologyUniversity of Utah HealthSalt Lake CityUSA
  3. 3.Department of Medicine/HematologyUniversity of UtahSalt Lake CityUSA

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