Essential thrombocythaemia in pregnancy

• Essential thrombocythaemia (ET) is classified as a chronic myeloproliferative disorder ( with polycythaemia vera (PV) and myelofibrosis with myeloid metaplasia (MMM))
  • ET is characterized by microvascular symptoms (headache, lightheadedness, paresthesias, erythromelalagia)
  • patients have a paradoxical predisposition to both bleeding and thrombotic complications, and the potential of transformation into acute myeloid leukaemia (AML) or MMM
  • in the first decade of the disease
    • median survival is close to that of an age-adjusted normal population and the greatest morbidity and mortality stems from thrombohaemorrhagic complications
  • ET in pregnancy:
    • treating women with ET during pregnancy is challenging due to the increased risk for first trimester spontaneous abortion, and thrombotic and obstetric complications (2)
    • treatment is similar to that for non-pregnant patients. Aspirin can be be effective in reducing pregnancy complications, especially in patients with JAK2-mutated ET (3)
    • cytoreductive therapy with peginterferon alfa-2a can be considered for high-risk patients who are pregnant (2)

Reference:

1. Tefferi A, Vannucchi AM, Barbui T. Essential thrombocythemia: 2024 update on diagnosis, risk stratification, and management. Am J Hematol. 2024 Apr;99(4):697-718.
2. Gangat N, Tefferi A. Myeloproliferative neoplasms and pregnancy: overview and practice recommendations. Am J Hematol. 2021 Mar 1;96(3):354-66.
3. Passamonti F, Rumi E, Randi ML, et al. Aspirin in pregnant patients with essential thrombocythemia: a retrospective analysis of 129 pregnancies. J Thromb Haemost. 2010 Feb;8(2):411-3.