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:
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.
Gangat N, Tefferi A. Myeloproliferative neoplasms and pregnancy: overview and practice recommendations. Am J Hematol. 2021 Mar 1;96(3):354-66.
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.
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