Contemporary management of patients with BCR-ABL1-negative myeloproliferative neoplasms during pregnancy.

INTRODUCTION: The management of pregnancy during the course of BCR-ABL1-negative myeloproliferative neoplasms (MPN) is an increasingly relevant problem. This is mostly due to earlier and better diagnosis of MPN together with the trend in modern society toward delaying pregnancy until later life. Areas Covered: The present review aims to provide an overview of the available literature data concerning outcome of pregnancy in MPN. Possible therapeutic modalities are discussed and a management algorithm is suggested. Expert Commentary: Most data are available for women with essential thrombocythemia and we present 793 published pregnancies. Live birth rate is 68.5% with 31.5% miscarriages. Spontaneous abortion is the most frequent complication with 26.5%, followed by stillbirth with 4.8%. Maternal complications are relatively low with 1.8% major thrombotic and 2.4% major bleeding events. In polycythemia vera the situation is clinically more complex and roughly 150 pregnancy reports are available. There is very limited information in primary myelofibrosis with less than 20 reported pregnancies. With active management including control of blood counts, aspirin, low molecular weight heparin and in higher risk cases interferon alpha pregnancy in MPN is manageable with a success rate not far below the normal situation with 80%.