Hereditary pyropoikilocytosis is a rare autosomal recessive condition, more common in blacks, which is characterised by:
Although the genetics of HPP differ from those of HE, the two are believed to be related because HPP patients often have relatives with mild HE, and the spectrin defect in HPP is qualitatively similar to that in mild HE.
Haemolysis but not heat sensitivity responds to splenectomy.
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