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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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