What leads to an increased production of bilirubin in unconjugated hyperbilirubinemia?

Updated: May 21, 2019
  • Author: Hisham Nazer, MBBCh, FRCP, DTM&H; Chief Editor: BS Anand, MD  more...
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Answer

Hemolysis generally induces a modest elevation in the plasma levels of unconjugated bilirubin (1-4 mg/dL). During acute hemolytic crises, such as those occurring in sickle cell disease or paroxysmal nocturnal hemoglobinuria, bilirubin production and plasma bilirubin may transiently exceed these levels. Although the plasma bilirubin level increases linearly in relation to bilirubin production, the bilirubin concentration may still be near the reference range in patients with a 50% reduction in red blood cell survival if hepatic bilirubin clearance is within the reference range.

Ineffective erythropoiesis (early labeled bilirubin [ELB] production) that is markedly increased is the basis of a rare disorder known as primary shunt hyperbilirubinemia, or idiopathic dyserythropoietic jaundice.


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