Hereditary pyropoikilocytosis (HPP) is an autosomal recessive form of 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. However, the hemolysis can lead to rapid sequestration and destruction of red cells. Splenectomy is curative when this occurs.
HPP has been associated with a defect of the erythrocyte membrane protein spectrin and with spectrin deficiency.
See also
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. doi:. PMID 1191563.
- Gallagher PG (2005). "Red cell membrane disorders". Hematology (Am Soc Hematol Educ Program): 13–8. PMID 16304353. Full text
- {{cite journal | author Cameron, John L | Current Surgical Therapy 9th edition pp547. | year 2008
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