Hematological Observations on Arabian SS Patients with a Homozygosity or Heterozygosity for aβSChromosome with Haplotype #31
作者:
KutlarA.,
HattoriY.,
BakiogluI.,
KutlarF.,
HuismanT. H. J.,
KamelK.,
期刊:
Hemoglobin
(Taylor Available online 1985)
卷期:
Volume 9,
issue 6
页码: 545-557
ISSN:0363-0269
年代: 1985
DOI:10.3109/03630268508997037
出版商: Taylor&Francis
数据来源: Taylor
摘要:
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 aβSchromosome 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 sameβSchromosome (#31) and for aβSchromosome with another haplotype (mainly #19). These data offer additional evidence indicating that the increasedγchain production is specific for theβSchromosome 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 withβSchromosome (#31) support the suggestion that the increased Hb F production mainly occurs in response to the anemia of the sickle cell disease.
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