Biosynthetic studies and y-chain composition in the greek type of hereditary persistence of fetal hemoglobin and in its association with β-thalassemia

C. Camaschella, M. A. Ciocca-Vasino, A. Guerrasio, G. Balegno, E. Barberis, D. Delponte, G. Saglio

Research output: Contribution to journalArticlepeer-review

Abstract

Hematological data, biosynthetic studies and γ-chain structure of three heterozygotes for HPFH Greek type and of two heterozygotes for both HPFH and β-thalassemia are reported. In the HPFH heterozygotes, hematological data were normal and globin chain synthesis balanced, while subjects carrying both HPFH and β-thalassemia presented a thalassemic picture and the same degree of α/non-α-chain imbalance as the β-thalassemia carrier belonging to the same family. The γ-chain composition studies showed only the presence of Aγ-chains in HPFH; in the association HPFH/β-thalassemia also some Gγ and Tγ were found. The mechanisms determining the high production of Hb F in the association HPFH/β-thalassemia are discussed.

Original languageEnglish
Pages (from-to)272-277
Number of pages6
JournalActa Haematologica
Volume61
Issue number5
DOIs
Publication statusPublished - 1979

Keywords

  • Globin synthesis
  • HbF
  • Hereditary persistence of fetal hemoglobin
  • β-thalassemia
  • γ-chains

ASJC Scopus subject areas

  • Hematology

Fingerprint Dive into the research topics of 'Biosynthetic studies and y-chain composition in the greek type of hereditary persistence of fetal hemoglobin and in its association with β-thalassemia'. Together they form a unique fingerprint.

Cite this