南印度的血红蛋白紊乱。

ISRN Hematology Pub Date : 2011-01-01 Epub Date: 2011-06-28 DOI:10.5402/2011/748939
Vani Chandrashekar, Mamta Soni
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引用次数: 53

摘要

阳离子交换高效液相色谱(CE-HPLC)越来越多地被用作血红蛋白病和地中海贫血的一线研究。结合全血细胞计数,CE-HPLC可以有效地将血红蛋白病分为性状、纯合性疾病和复合杂合性疾病。我们在金奈(南印度泰米尔纳德邦)的阿波罗医院进行了为期一年的研究,在此期间发现了543例异常色谱模式。最常见的疾病是β-地中海贫血性状(37.9%),其次是HbE性状(23.2%)、纯合子HbE病(18.9%)、HbS性状(5.3%)、HbE β-地中海贫血(4.6%)、HbS β-地中海贫血(2.5%)、β-重度地中海贫血(2.3%)、HbH(1.6%)、纯合子HbS(1.4%)、HbD性状(0.7%)。β-地中海贫血患者HbA2平均值为5.4%。β-地中海贫血的平均HbF为88%,HbH的平均A2为1.4%。在HbE疾病中,HbA2 + HbE为杂合状态的占30.1%,纯合状态的占90.8%,HbE β-地中海贫血的占54.8%。在镰状细胞疾病中,HbS从30.9%的性状变化到纯合子状态的79.9%,再到HbS β-地中海贫血的65.6%。
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Hemoglobin disorders in South India.

Cation exchange-high performance liquid chromatography (CE-HPLC) is increasingly being used as a first line of investigation for hemoglobinopathies and thalassemias. Together with a complete blood count, the CE-HPLC is effective in categorizing hemoglobinopathies as traits, homozygous disorders and compound heterozygous disorders. We carried out a one year study in Apollo Hospitals, Chennai (Tamil Nadu, South India) during which 543 abnormal chromatogram patterns were seen. The commonest disorder we encountered was β-thalassemia trait (37.9%), followed by HbE trait (23.2%), homozygous HbE disease (18.9%), HbS trait (5.3%), HbE β-thalassemia (4.6%), HbS β-thalassemia (2.5%), β-thalassemia major (2.3%), HbH (1.6%), homozygous HbS (1.4%), HbD trait (0.7%). The average value of HbA2 in β-thalassemia minor was 5.4%. β-thalassemia major had an average HbF of 88% and in HbH the mean A2 was 1.4%. Among the HbE disorders the HbA2 + HbE was 30.1% in the heterozygous state, 90.8% in the homozygous state and 54.8% in HbE β-thalassemia. In the sickle cell disorders, HbS varied from 30.9% in the trait to 79.9% in the homozygous state to 65.6% in HbS β-thalassemia.

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