Clinical variability and molecular characterization of Hbs/Gγ (Aγδβ)0-thal and Hbs/HPFH in Indian sickle cell dia patients: AIIMS experience 期刊名称: Hematology/oncology & Stem Cell Therapy
作者: Pandey, Hareram,Singh, Kanwaljeet,Ranjan, Ravi,Pandey, Sanjay
Kumar,Sharma, Amit,Kishor, Kamal,Seth, Tulika,Mahapatra, Manoranjan,Saxena,
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威德尔海Renu
年份: 2019年
万步炎期号: 第1期
关键词: SCD;Coinheritance;HbF;Delta beta thal;HPFH;HBB;Hemoglobin;HPFH-3
摘要:Introduction: In sickle cell dia (SCD) patients, among the predictors of survival, HbF levels play a significant role in lowering the morbidity and mortality. Coinheritance of δβ thalasmia and hereditary persistence of fetal hemoglobin (HPFH) may contribute to variable HbF levels in SCD patien
ts, thus influencing their clinicopathological profile. Such cas are sparly documented in the literature
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and thus, we screened the prence of δβ thalasmia and HPFH in 126 cas of
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SCD with high HbF. Material and methods: A total 126 SCD individuals with raid
HbF levels were the study subject. Capillary zone electrophoresis (CZE) was done
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一个半小时的英文for the quantitative asssment of hemoglobin variants. HbSC, HbSD, HbAS and HbSE cas were excluded. Asian Indian Gγ(Aγδβ)0-thal, δβ0-thal (Sicilian, 13.4 kb), (Chine, 100 kb),...
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