Sasano Tomoyuki, Tomimatsu Takuji, Nishimura Jun-Ichi, Matsumura Itaru, Kanakura Yuzuru, Kimura Tadashi
aDepartment of Obstetrics and Gynecology bDepartment of Hematology and Oncology, Osaka University Graduate School of Medicine cDivision of Hematology and Rheumatology, Department of Internal Medicine, Kinki University Faculty of Medicine, Osaka, Japan.
Blood Coagul Fibrinolysis. 2016 Jan;27(1):109-12. doi: 10.1097/MBC.0000000000000386.
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare acquired disorder of haematopoietic stem cells characterized by intravascular haemolysis, cytopenias and thrombophilia. Thrombophilia is the leading cause of mortality in patients with PNH. As the risk of thrombogenesis further increases during pregnancy and the postpartum period, an anticoagulant therapy is generally recommended for pregnant women with PNH. However, there are no standardized criteria for determining the appropriate dose of anticoagulant therapy. We describe the case of a PNH patient with who was managed with anticoagulant therapy at different doses during two consecutive pregnancies. A prophylactic dose of heparin was administered during her first pregnancy and a therapeutic dose, during her second pregnancy. Both pregnancies resulted in uncomplicated vaginal deliveries without thrombosis. Interestingly, not only D-dimer (as a thrombotic marker) but also lactate dehydrogenase (as a haemolytic marker) levels were lower during her second pregnancy when a therapeutic dose of heparin was used.
阵发性睡眠性血红蛋白尿（PNH）是一种罕见的造血干细胞获得性疾病，其特征为血管内溶血、血细胞减少和易栓症。易栓症是PNH患者死亡的主要原因。由于妊娠和产后血栓形成风险进一步增加，一般建议对患有PNH的孕妇进行抗凝治疗。然而，对于确定抗凝治疗的合适剂量尚无标准化标准。我们描述了一例PNH患者的病例，该患者在连续两次妊娠期间接受了不同剂量的抗凝治疗。她第一次妊娠期间给予预防性剂量的肝素，第二次妊娠期间给予治疗性剂量的肝素。两次妊娠均顺利经阴道分娩，未发生血栓形成。有趣的是，在使用治疗性剂量肝素的第二次妊娠期间，不仅D-二聚体（作为血栓形成标志物）水平，而且乳酸脱氢酶（作为溶血标志物）水平均较低。
