Pregnancy Management in Patients with Ph-Negative Myeloproliferative Neoplasms: A Literature Review and Case Report

Myeloproliferative neoplasms (MPNs) refer to a range of chronic oncohematologic clonal diseases characterized by excessive proliferation of myeloid lineage cells and/or excessive fibrosis of the bone marrow. Although MPNs are commonly diagnosed in subjects over 60 years of age, it is now increasingly detected at a young age. It is not uncommon for practitioners to encounter young pregnant patients with Ph-negative MPNs. Both pregnancy and Ph-negative MPNs are characterized by hypercoagulation increasing the risk of thrombotic complications and other adverse events associated with placental insufficiency. Pregnant MPN patients are treated with antiaggregants (aspirin) and anticoagulants (low molecular weight heparin), erythrocytapheresis or bloodletting; interferon-α is used if cytoreduction is required. This paper reports a case of management and outcomes of four pregnancies in a patient with masked polycythemia vera marked by portal vein thrombosis, portal hypertension, splenomegaly as well as esophageal and gastric fundus varices. Her first pregnancy ended in miscarriage at the early stage of gestation, her second pregnancy resulted in a premature birth, whereas her third and fourth pregnancies were successful — she delivered full-term babies. During all pregnancies, the patient received interferon-α.