Hemoglobin disorders in South India.
Hemoglobin disorders in South India.
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DOI：
10.5402/2011/748939
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发表时间：
2011-01-01
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影响因子：
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通讯作者：
Soni, Mamta
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作者：
Chandrashekar, Vani;Soni, Mamta
阳离子交换-高效液相色谱法（CE-HPLC）越来越多地被用作血红蛋白病和地中海贫血的第一线研究。与全血细胞计数一起，CE-HPLC可有效地将血红蛋白病分类为性状、纯合子疾病和复合杂合子疾病。我们进行了为期一年的研究，在阿波罗医院，钦奈（泰米尔纳德邦，印度南部），在此期间，543异常色谱图。我们遇到的最常见的疾病是β-地中海贫血性状（37.9%），其次是HbE性状（23.2%），纯合子HbE疾病（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%），重型β-地中海贫血（2.3%），HbH（1.6%），纯合子HbS（1.4%），HbD性状（0.7%）。轻型β地中海贫血患者HbA 2平均值为5.4%。重型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，HbA 2 + HbE在杂合状态下为30.1%，在纯合状态下为90.8%，在HbE β-地中海贫血中为54.8%。在镰状细胞疾病中，HbS从性状的30.9%到纯合状态的79.9%到HbS β-地中海贫血的65.6%不等。
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 beta-thalassemia trait (37.9%), followed by HbE trait (23.2%), homozygous HbE disease (18.9%), HbS trait (5.3%), HbE beta-thalassemia (4.6%), HbS beta-thalassemia (2.5%), beta-thalassemia major (2.3%), HbH (1.6%), homozygous HbS (1.4%), HbD trait (0.7%). The average value of HbA2 in beta-thalassemia minor was 5.4%. beta-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 beta-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 beta-thalassemia.