How is paroxysmal nocturnal hemoglobinuria (PNH) differentiated from aplastic anemia?

Updated: Jan 29, 2021
  • Author: Sameer Bakhshi, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

FLAER is a highly sensitive flow cytometry test for PNH that uses whole blood and binds specifically to glycophosphatidylinositol (GPI) anchor proteins in peripheral blood granulocytes. [59, 60] In PNH, mutation of the PIGA anchor protein gene results in a lack of the GPI anchors. Thus, the lack of FLAER binding to granulocytes is sufficient for the diagnosis of PNH. [59, 60] The disadvantage of the test is in measuring binding in the absence of adequate granulocytes—such as in severe aplastic anemia, when the number of circulating granulocytes is extremely low.


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