pyropoikilocytosis

py·ro·poi·ki·lo·cy·to·sis

(pī'rō-pōy-ki'lō-si-tō-sis),
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.

pyropoikilocytosis

[pī′rōpoi′kilō′sītō′sis]
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.
References in periodicals archive ?
The family history included a father and sister with similar symptoms of anemia, both of which had been diagnosed with hereditary pyropoikilocytosis.
A diagnosis of hereditary pyropoikilocytosis (HPP) was rendered based on laboratory studies, family history, erythrocyte osmotic fragility testing, and erythrocyte morphology.
Effect of heat on the circular dichroism of spectrin in hereditary pyropoikilocytosis.
Molecular determinants of clinical expression of hereditary elliptocytosis and pyropoikilocytosis.
Nonsense mutations of the alpha-spectrin gene in hereditary pyropoikilocytosis.
Hereditary spherocytosis RBC spectrin RBC ankyrin Hereditary elliptocytosis RBC spectrin Hereditary pyropoikilocytosis RBC spectrin Hemolytic anemia - Glucose- G-6-PD 6-phosphate dehydrogenase deficiency Hereditary nonspherocytic PDH hemolytic anemia - Pyruvate kinase deficiency Porphyrias Various genes encoding enzymes of the heme biosynthesis pathway