Fayed Mahmoud Alhosiny, Abdel-Hady Hesham El-Sayed, Hafez Mona Mohammed, Salama Osama Saad, Al-Tonbary Youssef Abdelhalim
Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt.
Neonatal Intensive Care Unit, Pediatric Department, Mansoura Faculty of Medicine, Mansoura University Children Hospital, Mansoura University, Al-Mansoura, Dakahlia 35516, Egypt.
Hematol Oncol Stem Cell Ther. 2018 Jun;11(2):65-74. doi: 10.1016/j.hemonc.2017.05.028. Epub 2017 Jun 15.
The increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered.
Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia.
36 Egyptian children with β-thalassemia with a mean age of 9.9years (±4.7 SD). In addition, 20 healthy Egyptian children matched for age and sex were enrolled as a control group. Both were subjected to clinical and laboratory assessments. Echocardiography was done to the patient group and PH was diagnosed based on calculated mean pulmonary artery pressure [MPAP] >25mmHg.
We found that, mean±SD serum P-selectin level (platelet activator marker) was significantly higher in thalassemic patients (2337±566pg/ml) in comparison to controls (1467±247pg/ml) (P<0.001). Mean serum protein-C and antithrombin-III levels were significantly lower in thalassemic patients (1.2±1.3µg/ml, 27.3±7.5mg/dl) in comparison to controls (2.3±1.3µg/ml, 35.1±4.1mg/dl) (P=0.003 and <0.001) respectively. PH was detected in 17 (47.2%) patients and it was significantly associated with splenectomy (P=0.01) and non-transfusion dependent thalassemia (NTDT) (P=0.04). PH was positively correlated with serum levels of P-selectin (r=0.38, P=0.02), fibrinogen (r=0.41, P=0.01) and negatively correlated with serum protein-C level (r=-0.48, P=0.003).
A chronic hypercoagulable state and platelet activation is present in children with β-thalassemia. Splenectomy and transfusion infrequency are the main risk factors noted to be associated with such hypercoagulable state and platelet activation and consequently the PH among our thalassemic patients.
地中海贫血患者生存率的提高导致了一些之前很少遇到的与管理相关并发症的暴露。
研究β地中海贫血患儿凝血和血小板活化增加的情况，分析导致这种高凝状态的因素，并结合β地中海贫血患儿的血小板活化和高凝状态研究肺动脉高压（PH）。
36名埃及β地中海贫血患儿，平均年龄9.9岁（±4.7标准差）。此外，招募20名年龄和性别匹配的健康埃及儿童作为对照组。两组均接受临床和实验室评估。对患者组进行超声心动图检查，并根据计算出的平均肺动脉压[MPAP]＞25mmHg诊断PH。
我们发现，地中海贫血患者的平均±标准差血清P选择素水平（血小板活化标志物）（2337±566pg/ml）显著高于对照组（1467±247pg/ml）（P＜0.001）。地中海贫血患者的平均血清蛋白C和抗凝血酶III水平（1.2±1.3µg/ml，27.3±7.5mg/dl）显著低于对照组（2.3±1.3µg/ml，35.1±4.1mg/dl）（分别为P=0.003和＜0.001）。17名（47.2%）患者检测到PH，且其与脾切除术（P=0.01）和非输血依赖型地中海贫血（NTDT）（P=0.04）显著相关。PH与血清P选择素水平呈正相关（r=0.38，P=0.02）、与纤维蛋白原呈正相关（r=0.41，P=0.01），与血清蛋白C水平呈负相关（r=-0.48，P=0.003）。
β地中海贫血患儿存在慢性高凝状态和血小板活化。脾切除术和输血不频繁是与这种高凝状态和血小板活化相关的主要危险因素，因此也是我们地中海贫血患者发生PH的主要危险因素。
