Essential Thrombocythemia/Polycythemia Vera and Pregnancy: The Need for an Observational Study in Europe

Abstract

The management of pregnant patients with essential thrombocythemia (ET) and polycythemia vera (PV) may be problematic. In the literature there are approximately 300 cases of pregnancies reported in ET and less than 50 pregnancies reported in PV. To reduce the effect of reporting bias, we selected articles with either > 10 pregnancies or at least six patients, and here report on the outcome of 195 pregnancies in ET and 36 pregnancies in PV patients. The live birth rate was approximately 60% in ET and 58% in PV. Spontaneous abortion during the first trimester was the most frequent fetal complication, occurring in 31% of ET pregnancies and in 22% of PV pregnancies, respectively. Major maternal complications were more frequent in PV compared with ET (44.4 versus 7.7%). Treatment with low-dose aspirin during pregnancy in ET seemed to reduce complications and also seemed beneficial during pregnancy in PV. In high-risk pregnancies, the additional use of low molecular weight heparin and/or interferon alpha should be considered. This article also describes a registry for an observational study concerning pregnancy in chronic Philadelphia chromosome-negative myeloproliferative disorders within the European LeukemiaNet. A potential management algorithm for pregnancies in ET or PV is also provided.

Polycythemia vera and essential thrombocythemia: 2015 update on diagnosis, risk‐stratification and management

“…First-trimester spontaneous miscarriage rate in ET or PV (>30%) [79][80][81] is significantly higher than the 15% rate expected in the control population and does not appear to be influenced by specific treatment [79]. Late obstetric complications as well as maternal thrombohemorrhagic events are relatively infrequent and platelet count usually decreases substantially during the second and third trimesters [82].…”

Patients with essential thrombocytosis during pregnancy: Challenges and therapeutic dilemmas

“…Pregnant women who are candidates for platelet-lowering therapy according to general guidelines of ET treatment should receive IFNa which seems to be the safest option for all patients while trying conceiving. Treatment with peg-IFN is more attractive due to the longer half-life and the similar efficacy in controlling platelet count; nevertheless, there is limited data concerning safety profile [18,29,36].…”

“…The addition of LMWH is not recommended even if there are published reports of successful pregnancies after the combination of both drugs in women with history of severe pregnancy complications including fetal death. However, LMWH is reasonable to be considered in women with history of thromboembolic events in the past and high-risk pregnancies for thrombotic potential (for instance immobilization, twin-pregnancies, high body weight of the patient) or presence of antiphosphilipid syndrome [36]. Inherited thrombophilia like presence of heterozygous F-Leiden mutation, in the absence of positive thrombotic family history, is not a definite indication for anticoagulation in all trimesters.…”

“…Griesshammer et al presented a large case series and patients with ET were under treatment with ASA which stopped about two weeks before labour was expected and started with LMWH in prophylactic dosage. LMWH was continued until delivery, with a dose stopped immediately about 12 hours before the expected delivery and was restarted on the first postpartum day and continued for 6 weeks [36].…”

Long-term management of thrombocytosis in essential thrombocythaemia

“…During pregnancy it is not unusual for platelet levels to fall, but if platelet-reducing therapy is needed, interferon-α is the only recommended drug [111].…”