What is the pathophysiology of ineffective erythropoiesis 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

In a review of 11 cases of ineffective erythropoiesis (early labeled bilirubin [ELB] production), evidence existed of rapid heme and hemoglobin turnover in the bone marrow, possibly due to the premature destruction of red blood cell precursors. These patients also had erythroid hyperplasia of bone marrow, reticulocytosis, increased iron turnover with diminished red blood cell incorporation, and hemosiderosis of hepatic parenchymal cells and Kupffer cells. The underlying defect responsible for the high rate of heme turnover within the marrow remains unknown.


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