Singer Sylvia T, Kuypers Frans A, Styles Lori, Vichinsky Elliott P, Foote Drucella, Rosenfeld Howard
Children's Hospital and Research Center at Oakland, Hematology/Oncology Department, Oakland, CA 94609-1808, USA.
Am J Hematol. 2006 Sep;81(9):670-5. doi: 10.1002/ajh.20640.
The pathogenesis of pulmonary hypertension (PAH), a serious complication in thalassemia, is not well understood. Thromboembolism has been postulated as one of the causative factors; however, there are currently limited specific data on its role. To examine whether increased platelet activation and hypercoagulability are linked to PAH, 25 beta-thalassemia major and beta-thalassemia intermedia patients were evaluated with Doppler echocardiograms for estimation of pulmonary artery pressure and with laboratory assays for indications of a prothrombotic state. The association of clinical variables and abnormal coagulation assays with PAH was determined. PAH was identified in 17 (68%) patients; mean pulmonary artery systolic pressure was 39.8 +/- 5.4 mm Hg. PAH was significantly associated with prior splenectomy, older age, and evidence for chronic hemolysis, diagnosed in both transfused (n = 10) and nontransfused (n = 7) patients. Increased platelet activation, measured by P-selectin, was significantly associated with PAH (P = 0.001). Increased thrombin-antithrombin III level was more prevalent in the presence of PAH, but increased fibrinolysis or low protein C levels were not. This study underscores the role of platelet activation in the development of PAH and stresses its occurrence even among patients who are regularly transfused, especially those who are older and have had splenectomies.
肺动脉高压（PAH）是地中海贫血的一种严重并发症，其发病机制尚未完全明确。血栓栓塞被认为是致病因素之一；然而，目前关于其作用的具体数据有限。为了研究血小板活化增加和高凝状态是否与PAH相关，对25例重型β地中海贫血和中间型β地中海贫血患者进行了多普勒超声心动图检查以评估肺动脉压力，并进行了实验室检测以评估血栓前状态指标。确定了临床变量和异常凝血检测与PAH的关联。17例（68%）患者被诊断为PAH；平均肺动脉收缩压为39.8±5.4mmHg。PAH与既往脾切除术、年龄较大以及慢性溶血证据显著相关，在输血患者（n = 10）和未输血患者（n = 7）中均有诊断。通过P-选择素测量的血小板活化增加与PAH显著相关（P = 0.001）。凝血酶-抗凝血酶III水平升高在PAH患者中更为普遍，但纤维蛋白溶解增加或蛋白C水平降低则不然。本研究强调了血小板活化在PAH发生发展中的作用，并强调即使在定期输血的患者中也会出现，尤其是年龄较大且已行脾切除术的患者。
