Hereditary pyropoikilocytosis

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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.

See also

• Erytrocyte
• Hereditary elliptocytosis
• Poikilocytosis

References

• 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. PMID 1191563.
• Gallagher PG (2005). "Red cell membrane disorders". Hematology (Am Soc Hematol Educ Program): 13-8. PMID 16304353. Full text

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Evidence Based Medicine Regarding Hereditary pyropoikilocytosis	Cochrane Collaboration on Hereditary pyropoikilocytosis • Bandolier on Hereditary pyropoikilocytosis • TRIP on Hereditary pyropoikilocytosis
Cost Effectiveness of Hereditary pyropoikilocytosis	Cost Effectiveness of Hereditary pyropoikilocytosis
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Guidelines / Policies / Government Resources (FDA/CDC) Regarding Hereditary pyropoikilocytosis	US National Guidelines Clearinghouse on Hereditary pyropoikilocytosis • NICE Guidance on Hereditary pyropoikilocytosis • NHS PRODIGY Guidance • FDA on Hereditary pyropoikilocytosis • CDC on Hereditary pyropoikilocytosis
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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .