Chansai Siriyakorn, Fucharoen Supan, Fucharoen Goonnapa, Jetsrisuparb Arunee, Chumpia Worawan
Medical Science Program, Graduate School, Khon Kaen University, Khon Kaen, Thailand.
Centre for Research and Development of Medical Diagnostic Laboratories, Faculty of Associated Medical Sciences, Khon Kaen University, Khon Kaen, Thailand.
Acta Haematol. 2018;139(1):47-51. doi: 10.1159/000486157. Epub 2018 Jan 18.
Thalassemia is a group of hereditary hemoglobinopathies caused by decreased or absent synthesis of α and/or β globin chains. Studies have shown that hypercoagulability and thrombosis are common clinical symptoms in β-thalassemia, especially β-thalassemia intermedia, but little is known about in α-thalassemia. This study aims to examine phosphatidylserine (PS) levels, platelet activation, and coagulation markers in splenectomized (S) and nonsplenectomy (NS) patients with hemoglobin (Hb) H disease.
The NS group comprised 20 patients (median age 15.0 years, range, 14-16.5 years), and the S group consisted of 11 patients (median age 16.4 years, range, 14-19.9 years) with Hb H disease; the control group consisted of 20 normal subjects. Hematological parameters were collected. Flow cytometry was used to measure PS exposure on red blood cells. The levels of intercellular adhesive molecule (ICAM)-1, tumor necrosis factor α (TNFα), β-thromboglobulin (TG) and prothrombin fragment 1 + 2 (F1.2) were determined using ELISA test kits.
Significant increases in the levels of PS, ICAM-1, TNFα, β-TG, and F1.2 were observed in both patient groups compared to normal controls (p < 0.01).
This observation indicates blood coagulation, endothelial injury, chronic low-grade inflammation, platelet activation, and thrombin generation are present in Hb H disease; these findings merit further assessment in a larger prospective cohort to establish possible links with thrombotic manifestations.
地中海贫血是一组由于α和/或β珠蛋白链合成减少或缺乏引起的遗传性血红蛋白病。研究表明，高凝状态和血栓形成是β地中海贫血常见的临床症状，尤其是中间型β地中海贫血，但关于α地中海贫血的情况知之甚少。本研究旨在检测血红蛋白H病脾切除（S）和非脾切除（NS）患者的磷脂酰丝氨酸（PS）水平、血小板活化和凝血标志物。
NS组包括20例血红蛋白H病患者（中位年龄15.0岁，范围14 - 16.5岁），S组由11例血红蛋白H病患者组成（中位年龄16.4岁，范围14 - 19.9岁）；对照组由20名正常受试者组成。收集血液学参数。采用流式细胞术检测红细胞上PS的暴露情况。使用酶联免疫吸附测定试剂盒测定细胞间黏附分子（ICAM）-1、肿瘤坏死因子α（TNFα）、β-血小板球蛋白（TG）和凝血酶原片段1 + 2（F1.2）的水平。
与正常对照组相比，两组患者的PS、ICAM-1、TNFα、β-TG和F1.2水平均显著升高（p < 0.01）。
该观察结果表明血红蛋白H病存在凝血、内皮损伤、慢性低度炎症、血小板活化和凝血酶生成；这些发现值得在更大的前瞻性队列中进行进一步评估，以确定与血栓形成表现的可能联系。
