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Article

Molecular basis of spectrin deficiency in hereditary pyropoikilocytosis.

Blood - 1 Sept 1993

Hanspal M, Hanspal J S, Sahr K E, Fibach E, Nachman J, Palek J

Abstract excerpt

Hereditary pyropoikilocytosis (HPP) is a recessively inherited hemolytic anemia characterized by severe poikilocytosis and red blood cell fragmentation. HPP red blood cells are partially deficient in spectrin and contain a mutant alpha or beta-spectrin that is defective in terms of spectrin self-association. Although the nature of the latter defect has been studied in considerable detail and many mutations of...

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PMID: 8364214

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

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