Congenital Hemolytic Anemia

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10 Scopus citations

Abstract

Red blood cell (RBC) destruction can be secondary to intrinsic disorders of the RBC or to extrinsic causes. In the congenital hemolytic anemias, intrinsic RBC enzyme, RBC membrane, and hemoglobin disorders result in hemolysis. The typical clinical presentation is a patient with pallor, anemia, jaundice, and often splenomegaly. The laboratory features include anemia, hyperbilirubinemia, and reticulocytosis. For some congenital hemolytic anemias, splenectomy is curative. However, in other diseases, avoidance of drugs and toxins is the best therapy. Supportive care with transfusions are also mainstays of therapy. Chronic hemolysis often results in the formation of gallstones, and cholecystectomy is often indicated.

Original languageEnglish (US)
Pages (from-to)361-374
Number of pages14
JournalMedical Clinics of North America
Volume101
Issue number2
DOIs
StatePublished - Mar 1 2017

Keywords

  • Aplastic crisis
  • Hemolysis
  • Jaundice
  • Splenectomy

ASJC Scopus subject areas

  • Medicine(all)

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