|Classification and external resources|
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.
It was characterized in 1975.
- 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:10.1111/j.1365-2141.1975.tb02740.x. PMID 1191563.
- King MJ, Jepson MA, Guest A, Mushens R (April 2011). "Detection of hereditary pyropoikilocytosis by the eosin-5-maleimide (EMA)-binding test is attributable to a marked reduction in EMA-reactive transmembrane proteins". Int J Lab Hematol 33 (2): 205–11. doi:10.1111/j.1751-553X.2010.01270.x. PMID 21054813.
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