Hereditary pyropoikilocytosis

Hereditary pyropoikilocytosis (HPP) is a 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.

HPP has been associated with a defect of the erythrocyte membrane protein spectrin and with spectrin deficiency.