Hereditary pyropoikilocytosis
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.
Create an account to add page annotations
Annotations allow you to add information to this page that would be handy to have on hand during a consultation. E.g. a website or number. This information will always show when you visit this page.