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
a recessive inherited disorder characterized by severe hemolysis, irregular shapes of red blood cells, and sensitivity of blood cells to fragmentation in vitro after minor temperature variations.
pyropoikilocytosis(pī″rō-poy″ki-lŏ-sī-tō′sĭs) [ pyro- + poikilocytosis]
An inherited form of hemolytic anemia in which cells have defective spectrin in their cytoskeleton, resulting in cellular breakdown during exposure to heat.