Al Huneini M, Alkindi S, Panjwani V, Al Falahi K, Al Balushi B, Gravell D, Ho C H, Krishnamoorthy R, Pathare A V
Sultan Qaboos University Hospital, Muscat, OMAN.
Sultan Qaboos University, College of Medicine and Health Sciences, Muscat, OMAN.
Mediterr J Hematol Infect Dis. 2017 Apr 20;9(1):e2017028. doi: 10.4084/MJHID.2017.028. eCollection 2017.
To explore the incidence of vaso-occlusive crisis (VOC) in Blood Group "O" sickle cell disease (SCD) patients, and correlate it with the blood group and thrombospondin (TSP) levels.
In 89 consecutive SCD patients, blood samples were obtained for von Williebrand factor (vWF:Ag) antigen, collagen binding activity (CBA), ristocetin binding activity (RCo), blood group typing, C-reactive protein (CRP), high performance liquid chromatography (HPLC), Serum TSP 1 and TSP 2 levels, complete blood counts (CBC), lactic dehydrogenase (LDH) levels, liver function (LFT) and renal function tests (RFT) during VOC episodes and in steady state conditions.
In steady state SCD patients (n=72), "O" blood group patients (n=37) showed a significantly higher median serum TSP 1 and TSP 2 levels as compared to non-O blood group patients [n=35] [p <0.05, Mann-Whitney test]; with an inverse relation between vWF:Ag, Factor VIII:C and TSP levels. Furthermore, the serum TSP 1 and TSP 2 levels were significantly higher in patients presenting with acute VOC [n=17], as well as in those with repeated VOC's (group 1, n=16), especially amongst blood group "O" patients [p, <0.05, Mann-Whitney test].
The study demonstrates an inverse relation between TSP and vWF levels, in blood group "O" SCD patients, with an upregulation of the TSP levels. Expectedly, during active VOC crisis, the TSP 1 and TSP 2 levels were significantly elevated.
探讨血型为“O”的镰状细胞病（SCD）患者血管闭塞性危机（VOC）的发生率，并将其与血型和血小板反应蛋白（TSP）水平相关联。
在89例连续的SCD患者中，采集血样检测血管性血友病因子（vWF:Ag）抗原、胶原结合活性（CBA）、瑞斯托霉素结合活性（RCo）、血型分型、C反应蛋白（CRP）、高效液相色谱法（HPLC）、血清TSP 1和TSP 2水平、全血细胞计数（CBC）、乳酸脱氢酶（LDH）水平、肝功能（LFT）和肾功能测试（RFT），检测时间为VOC发作期间和病情稳定状态。
在病情稳定的SCD患者（n = 72）中，血型为“O”的患者（n = 37）血清TSP 1和TSP 2的中位数水平显著高于非“O”血型患者[n = 35] [p <0.05，曼-惠特尼检验]；vWF:Ag、凝血因子VIII:C与TSP水平呈负相关。此外，急性VOC患者（n = 17）以及反复发生VOC的患者（第1组，n = 16）血清TSP 1和TSP 2水平显著更高，尤其是在血型为“O”的患者中[p <0.05，曼-惠特尼检验]。
该研究表明，在血型为“O”的SCD患者中，TSP与vWF水平呈负相关，且TSP水平上调。不出所料，在活跃的VOC危机期间，TSP 1和TSP 2水平显著升高。
