Division of Hematology, Department of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Thromb Res. 2014 Jan;133(1):5-9. doi: 10.1016/j.thromres.2013.09.030. Epub 2013 Sep 26.
Blood coagulation activation is frequently found in patients with malaria. Clinically apparent bleeding or disseminated intravascular coagulation (DIC) is associated with very severe disease and a high mortality. Protein C, protein S, and antithrombin levels were found to be low in P. falciparum, but were normal in P. vivax infection. Plasma levels of plasminogen activator inhibitor-1 were high in cases of P. falciparum infection whereas tissue plasminogen activator levels were low. Elevated plasma levels of von Willebrand factor (vWF) and vWF propeptide, thrombomodulin, endothelial microparticles have been reported in P. falciparum-infected patients. It has been demonstrated that severe P. falciparum infection is associated with acute endothelial cell (EC) activation, abnormal circulating ultralarge vWF multimers, and a significant reduction in plasma ADAMTS13 function. These changes may result in intravascular platelet aggregation, thrombocytopenia, and microvascular disease. It has also been shown that P. falciparum-parasitized red blood cells (pRBCs) induce tissue factor (TF) expression in microvascular ECs in vitro. Recently, loss of endothelial protein C receptor (EPCR) localized to sites of cytoadherent pRBCs in cerebral malaria has been demonstrated. Severe malaria is associated with parasite binding to EPCR. The cornerstone of the treatment of coagulopathy in malaria is the use of effective anti-malarial agents. DIC with spontaneous systemic bleeding should be treated with screened blood products. Study in Thailand has shown that for patients who presented with parasitemia >30% and severe systemic complications such as acute renal failure and ARDS, survival was superior in the group who received exchange transfusion. The use of heparin is generally restricted to patients with DIC and extensive deposition of fibrin, as occurs with purpura fulminans or acral ischemia. Antiplatelet agents interfere with the protective effect of platelets against malaria and should be avoided.
血液凝固激活在疟疾患者中经常被发现。临床上明显的出血或弥漫性血管内凝血(DIC)与非常严重的疾病和高死亡率相关。研究发现,疟原虫感染时蛋白 C、蛋白 S 和抗凝血酶水平降低,但间日疟原虫感染时正常。疟原虫感染时血浆纤溶酶原激活物抑制剂-1 水平升高,而组织型纤溶酶原激活物水平降低。研究报道,疟原虫感染患者血浆血管性血友病因子(vWF)和 vWF 前肽、血栓调节蛋白、内皮微颗粒水平升高。研究表明,严重疟原虫感染与急性内皮细胞(EC)激活、循环中超大 vWF 多聚体异常和血浆 ADAMTS13 功能显著降低有关。这些变化可能导致血管内血小板聚集、血小板减少和微血管疾病。研究还表明,疟原虫感染的红细胞(pRBC)在体外诱导微血管 EC 表达组织因子(TF)。最近,研究表明在脑型疟疾中,失去内皮蛋白 C 受体(EPCR)定位于黏附 pRBC 的部位。严重疟疾与寄生虫结合 EPCR 有关。疟疾凝血功能障碍治疗的基石是使用有效的抗疟药物。DIC 伴有自发性全身出血应使用筛选的血液制品治疗。泰国的研究表明,对于寄生虫血症>30%且有严重全身并发症(如急性肾衰竭和 ARDS)的患者,接受换血治疗的患者存活率更高。肝素的使用一般限于 DIC 患者和广泛纤维蛋白沉积的患者,如暴发性紫癜或肢端缺血。抗血小板药物会干扰血小板对疟疾的保护作用,应避免使用。