Shamoon Mohib, Mahmood Rafia, Bozdar Manzar, Yousof Saad
Armed Forces Institute of Pathology, Department of Hematology, Rawalpindi, Pakistan.
Hemoglobin. 2025 May;49(3):195-199. doi: 10.1080/03630269.2025.2510442. Epub 2025 Jun 1.
This prospective cross-sectional study was conducted at the Department of Hematology at Armed Forces Institute of Pathology Rawalpindi, Pakistan, from July 2023 to February 2025 after approval from Ethical Review Committee of the institute. Individuals being investigated for hemoglobinopathies in whom Hemoglobin D was detected, were included in the study. After detailed history and examination, investigations were performed including Complete Blood Counts (on Sysmex XN-3000), Capillary Zone Electrophoresis (on Sebia Capillarys 2 Flex-Piercing), High Performance Liquid Chromatography (on Bio-Rad D10) for differentiating Hb D-Punjab and D-Iran. Molecular studies (using PCR) were performed on samples in which a co-existing β thalassemia mutation was suspected on hemoglobin electrophoresis. Data collected was analyzed on Jamovi v2.4. Over 18 months, 2,171 individuals were tested for hemoglobinopathies, and Hb D, after excluding concomitant iron deficiency anemia, was detected in 106. Among these, 76 were found to have Hb D trait, 3 with homozygous Hb D disease, and 27 with compound heterozygous conditions. The compound heterozygous group included 21 patients of Hb D/β-thalassemia, 4 patients of Hb S/D, and 2 patients of Hb D/E. Hb D-Punjab accounted for 71% of the Hb D patients, and Hb D-Iran for the remaining 29%. Linear regression analysis revealed that MCH and RBC count showed significant positive correlations with Hb D levels. Molecular analysis identified specific β-thalassemia mutations in the Hb D/β-thalassemia cases, with IVS1-5 and FR 8-9 being the most common.
这项前瞻性横断面研究于2023年7月至2025年2月在巴基斯坦拉瓦尔品第武装部队病理研究所血液科进行,该研究获得了该研究所伦理审查委员会的批准。对检测到血红蛋白D的血红蛋白病患者进行调查,并纳入研究。在详细询问病史和体格检查后,进行了多项检查,包括全血细胞计数(使用Sysmex XN-3000)、毛细管区带电泳(使用Sebia Capillarys 2 Flex-Piercing)、高效液相色谱(使用Bio-Rad D10)以区分血红蛋白D-旁遮普型和D-伊朗型。对血红蛋白电泳怀疑存在共存β地中海贫血突变的样本进行分子研究(使用聚合酶链反应)。收集的数据使用Jamovi v2.4进行分析。在18个月的时间里,对2171名个体进行了血红蛋白病检测,排除合并缺铁性贫血后,检测到106例血红蛋白D。其中,76例为血红蛋白D性状,3例为纯合子血红蛋白D病,27例为复合杂合子情况。复合杂合子组包括21例血红蛋白D/β地中海贫血患者、4例血红蛋白S/D患者和2例血红蛋白D/E患者。血红蛋白D-旁遮普型占血红蛋白D患者的71%,其余29%为血红蛋白D-伊朗型。线性回归分析显示,平均红细胞血红蛋白含量(MCH)和红细胞计数与血红蛋白D水平呈显著正相关。分子分析在血红蛋白D/β地中海贫血病例中鉴定出特定的β地中海贫血突变,其中IVS1-5和FR 8-9最为常见。