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Hereditary pyropoikilocytosis

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Hereditary pyropoikilocytosis is a rare autosomal recessive condition, more common in blacks, which is characterised by:

  • severe haemolytic anaemia
  • marked red cell fragmentation
  • microspherocytosis
  • poikilocytosis
  • MCV less than or equal to 50
  • abnormal red cell heat sensitiviy

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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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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