Two consecutive pregnancies in a patient with paroxysmal nocturnal haemoglobinuria treated with anticoagulant therapy at different doses.
Two consecutive pregnancies in a patient with paroxysmal nocturnal haemoglobinuria treated with anticoagulant therapy at different doses.
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阵发性睡眠性血红蛋白尿症患者连续两次妊娠，接受不同剂量的抗凝治疗。
DOI：
10.1097/mbc.0000000000000386
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发表时间：
2016
期刊：
影响因子：
--
通讯作者：
Kimura T ,et al.
中科院分区：
文献类型：
--
作者：
Sasano T; Matsumura I;Kimura T ,et al.
阵发性夜间血红蛋白尿（PNH）是一种罕见的获得性造血干细胞疾病，其特征是血管内溶血、细胞减少和亲血栓症。血栓形成是PNH患者死亡的主要原因。由于血栓形成的风险在妊娠期和产后进一步增加，抗凝治疗通常被推荐用于PNH孕妇。然而，目前还没有确定抗凝治疗适当剂量的标准。我们描述的情况下，PNH患者谁是管理在不同剂量的抗凝治疗在两个连续怀孕。在她第一次怀孕期间给予预防性剂量的肝素，在她第二次怀孕期间给予治疗剂量。两次妊娠均无并发症阴道分娩，无血栓形成。有趣的是，不仅d -二聚体（作为血栓标志物），而且乳酸脱氢酶（作为溶血标志物）水平在她第二次怀孕时使用治疗剂量的肝素时更低。
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.
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DOI：
10.3324/haematol.2010.037531
发表时间：
2011-09-01
期刊：
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
影响因子：
--
作者：
de Guibert, Sophie;de Latour, Regis Peffault;Lamy, Thierry
通讯作者：
Lamy, Thierry
影响因子：
7.5
作者：
Oberkersch, Roxana;Attorresi, Alejandra I.;Calabrese, Graciela C.
通讯作者：
Calabrese, Graciela C.
依库丽单抗治疗阵发性睡眠性血红蛋白尿期间平滑肌肌张力障碍症状的改善。
影响因子：
10.1
作者：
A. Hill;R. Rother;P. Hillmen
通讯作者：
P. Hillmen
DOI：
10.1016/0019-2791(76)90223-8
发表时间：
1976-01-01
期刊：
IMMUNOCHEMISTRY
影响因子：
--
作者：
RAEPPLE, E;HILL, HU;LOOS, M
通讯作者：
LOOS, M
肝素或低分子肝素抑制阵发性睡眠性血红蛋白尿中补体介导的溶血
影响因子：
6.5
作者：
H. Ninomiya;Y. Kawashima;T. Nagasawa
通讯作者：
T. Nagasawa