Background and objectives: Polycythemia vera (PV) is rare in women of childbearing age with only 20 previous pregnancies reported.
Design and methods: We report a series of 18 pregnancies (19 fetuses) in eight women with PV managed prior to or following implementation of management guidelines tailored to PV in pregnancy, and review the literature.
Results: Seven of these pregnancies were managed by standard antenatal care (group A) without specific attention to the women's PV. All remaining 11 pregnancies (group B) were managed following a formal protocol and received tailored management principally comprising tight control of the hematocrit by venesection, and the use of interferon ? in three patients, in addition to aspirin 75 mg, and prophylactic low molecular weight heparin (LMWH). Each pregnancy was monitored with uterine artery Doppler examinations and regular fetal scanning. In group A (n=7) there was one live birth, which required delivery at 34 weeks due to placental insufficiency, three first trimester miscarriages, two stillbirths and one combined stillbirth and neonatal death (twins) associated with placental dysfunction. All 11 patients in group B received aspirin and post-partum LMWH; four also received venesection (during pregnancy), three interferon-a and three antenatal LMWH. There were ten live births, nine at term, one first trimester miscarriage and no intrauterine growth retardation.
Interpretation and conclusions: Pregnancy in PV without meticulous attention to hematocrit is associated with poor fetal outcome. Aggressive intervention with control of hematocrit, aspirin and some LMWH appears to be associated with significantly better outcome (p=0.0017).