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 × 109/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.
Keywords: Acquired von Willebrand syndrome; Bleeding; Myeloproliferative neoplasms; Pregnancy; Von Willebrand factor.