Congenital Hemolytic Anemia

Research output: Contribution to journalReview articlepeer-review

35 Scopus citations


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
Issue number2
StatePublished - Mar 1 2017


  • Aplastic crisis
  • Hemolysis
  • Jaundice
  • Splenectomy

ASJC Scopus subject areas

  • General Medicine


Dive into the research topics of 'Congenital Hemolytic Anemia'. Together they form a unique fingerprint.

Cite this