Hereditary pyropoikilocytosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Hereditary pyropoikilocytosis (HPP) is a hemolytic anemia characterized by an abnormal sensitivity of red blood cells to heat and erythrocyte morphology similar to that seen in thermal burns. Patients with HPP tend to experience severe haemolysis and anaemia in infancy that gradually improves, evolving toward typical elliptocytosis later in life.

HPP has been associated with a defect of the erythrocyte membrane protein spectrin and with spectrin deficiency.

See also

• Erytrocyte
• Hereditary elliptocytosis
• Poikilocytosis

References

• Zarkowsky HS, Mohandas N, Speaker CB, Shohet SB (1975). "A congenital haemolytic anaemia with thermal sensitivity of the erythrocyte membrane". Br J Haematol. 29 (4): 537–43. PMID 1191563.CS1 maint: Multiple names: authors list (link)
• Gallagher PG (2005). "Red cell membrane disorders". Hematology (Am Soc Hematol Educ Program): 13–8. PMID 16304353. Full text