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Pronunciation: pi'ro-poy-ki'lo-si-to-sis

Definition: A rare recessive disorder manifested by severe hemolysis, marked poikilocytosis, and a characteristic sensitivity of the red cells to heat-induced fragmentation in vitro; apparently due to a defect in spectrin self-association.

Synonym(s): hereditary pyropoikilocytosis

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.