Department of Obstetrics and Gynecology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
Braun School of Public Health and Community Medicine, Faculty of Medicine of the Hebrew University and Hadassah, Jerusalem, Israel.
J Thromb Thrombolysis. 2018 Oct;46(3):304-309. doi: 10.1007/s11239-018-1663-9.
To investigate the course of acquired type 2A von Willebrand syndrome (AVWS) in relation to patient management and outcomes among pregnant patients with essential thrombocytosis (ET). A review of pregnant women with ET evaluated for AVWS at the beginning of pregnancy and at the third trimester. Eighteen women with 24 pregnancies were included in this study. A history of bleeding was noted in 8 (44%) patients. In 20 (83%) pregnancies AVWS was evident at the initial testing. Following initial testing, antithrombotic therapy was administered in 22 (92%) pregnancies (aspirin, n = 20 and low-molecular-weight heparin, n = 2). In the remaining two pregnancies, VWF:RCo levels were below 30%; thus, aspirin was given only after repeat testing at 14-16 weeks. At third trimester testing, median VWF:RCo levels were significantly higher than at the initial testing (86 vs. 48%, P < 0.001), with no evidence of AVWS in any of the patients. Significant increases were also observed in the VWF:Ag level (127 vs. 84%, P < 0.001), the VWF:RCo/VWF:Ag ratio (0.75 vs. 0.54, P < 0.001) and the FVIII level (103 vs. 68%, P < 0.001); while platelet count (359 vs. 701 × 10/l, P < 0.001) and hemoglobin level (11.6 vs. 13.4 g/dl, P < 0.001) decreased. Neuraxial anesthesia was safely performed in 17 (71%) pregnancies. No significant bleeding events occurred during pregnancy and delivery. AVWS-related abnormalities in women with ET mostly improved during pregnancy, with favorable maternal and fetal outcomes. VWF parameters should be tested at early pregnancy and repeated at the third trimester, to guide pregnancy and delivery management.
探讨与妊娠患者特发性血小板增多症（ET）相关的获得性 2A 型血管性血友病（AVWS）病程、患者管理和结局。对妊娠早期和妊娠晚期接受 AVWS 评估的 ET 孕妇进行了回顾性分析。本研究共纳入 18 例 ET 孕妇的 24 例妊娠。8 例（44%）患者有出血史。20 例（83%）妊娠在初次检测时出现 AVWS。初次检测后，22 例（92%）妊娠接受了抗血栓治疗（阿司匹林 20 例，低分子肝素 2 例）。在其余两例妊娠中，VWF:RCo 水平低于 30%；因此，仅在 14-16 周重复检测后才给予阿司匹林。在妊娠晚期检测时，VWF:RCo 中位数明显高于初次检测（86%比 48%，P<0.001），所有患者均无 AVWS 证据。VWF:Ag 水平（127%比 84%，P<0.001）、VWF:RCo/VWF:Ag 比值（0.75 比 0.54，P<0.001）和 FVIII 水平（103%比 68%，P<0.001）也显著升高；而血小板计数（359 比 701×10/l，P<0.001）和血红蛋白水平（11.6 比 13.4 g/dl，P<0.001）下降。17 例（71%）妊娠中安全实施了脊麻。妊娠和分娩期间未发生明显出血事件。ET 患者的 AVWS 相关异常在妊娠期间大多得到改善，母婴结局良好。VWF 参数应在妊娠早期进行检测，并在妊娠晚期重复检测，以指导妊娠和分娩管理。
