Hematological observations on arabian SS patients with a homozygosity or heterozygosity for a βs chromosome with haplotype 31

A. Kutlar, Y. Hattori, I. Bakioglu, F. Kutlar, T. H.J. Huisman, K. Kamel

Research output: Contribution to journalArticle

37 Scopus citations

Abstract

Hematological and hemoglobin composition data are presented for seven Arabian SS patients with mild disease and with high Hb F levels varying between 21 and 34% Four patients were homozygous for a βS chromosome with a specific haplotype (31). The data for these four patients were similar to those for three other SS patients (and for five patients reported earlier, Ref. 2) who were heterozygous for the same βS chromosome (31) and for a βS chromosome with another haplotype (mainly 19). These data offer additional evidence indicating that the increased γ chain production is specific for the βS chromosome with haplotype 31. The similarities in hematological data and Hb F levels between these two groups of SS patients and the normal Hb F value in Hb S heterozygotes with βS chromosome (31) support the suggestion that the increased Hb F production mainly occurs in response to the anemia of the sickle cell disease.

Original languageEnglish (US)
Pages (from-to)545-557
Number of pages13
JournalHemoglobin
Volume9
Issue number6
DOIs
StatePublished - Jan 1 1985

ASJC Scopus subject areas

  • Hematology
  • Clinical Biochemistry
  • Genetics(clinical)
  • Biochemistry, medical

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