Pregnancy outcomes in myeloproliferative neoplasms: A Mayo Clinic report on 102 pregnancies

Gangat, Naseema; Joshi, Maansi; Shah, Sahrish; Yogarajah, Meera; Patnaik, Mrinal M.; Pardanani, Animesh; Wolanskyj, Alexandra P.; Tefferi, Ayalew

doi:10.1002/ajh.25748

Cited by 15 publications

(25 citation statements)

References 8 publications

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“…[55][56][57][58] First-trimester spontaneous miscarriage rate in ET or PV (>30%) is significantly higher than the 15% rate expected in the control population and does not appear to be influenced by specific treatment. 59,60 Late obstetric complications as well as maternal thrombohemorrhagic events are relatively infrequent and platelet count usually decreases substantially during the second and third trimesters. 60 Neither platelet count nor cytoreductive therapy appears to affect either maternal morbidity or pregnancy outcome.…”

Section: Bothmentioning

confidence: 99%

“…59,60 Late obstetric complications as well as maternal thrombohemorrhagic events are relatively infrequent and platelet count usually decreases substantially during the second and third trimesters. 60 Neither platelet count nor cytoreductive therapy appears to affect either maternal morbidity or pregnancy outcome. 60 Therefore, cytoreductive treatment is currently not recommended for low-risk women with ET or PV that are either pregnant or wish to be pregnant.…”

Section: Bothmentioning

confidence: 99%

“…60 Neither platelet count nor cytoreductive therapy appears to affect either maternal morbidity or pregnancy outcome. 60 Therefore, cytoreductive treatment is currently not recommended for low-risk women with ET or PV that are either pregnant or wish to be pregnant.…”

Section: Bothmentioning

confidence: 99%

“…Aspirin therapy is also considered to be adequate, and potentially useful in preventing complications during pregnancy, especially in JAK2 V617F‐positive cases 55‐58 . First‐trimester spontaneous miscarriage rate in ET or PV (>30%) is significantly higher than the 15% rate expected in the control population and does not appear to be influenced by specific treatment 59,60 . Late obstetric complications as well as maternal thrombohemorrhagic events are relatively infrequent and platelet count usually decreases substantially during the second and third trimesters 60 .…”

Section: Risk‐adapted Therapymentioning

confidence: 99%

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Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk‐stratification and management

2020

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Disease overview: Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms (MPN) respectively characterized by clonal erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus and risk of leukemic or fibrotic transformation. Diagnosis: Bone marrow morphology remains the cornerstone of diagnosis. In addition, the presence of JAK2 mutation is expected in PV while approximately 90% of patients with ET express mutually exclusive JAK2, CALR or MPL mutations (so called driver mutations). In ET, it is most important to exclude the possibility of prefibrotic myelofibrosis. Survival: Median survivals are approximately 15 years for PV and 18 years for ET; the corresponding values for patients age 40 or younger were 37 and 35 years. Certain mutations (mostly spliceosome) and abnormal karyotype might compromise survival in PV and ET. Life-expectancy in ET is inferior to the control population. Driver mutations have not been shown to affect survival in ET but risk of thrombosis is higher in JAK2 mutated cases. Leukemic transformation rates at 10 years are estimated at <1% for ET and 3% for PV. Thrombosis risk: In PV, two risk categories are considered: high (age > 60 years or thrombosis history present) and low (absence of both risk factors). In ET, four risk categories are considered: very low (age ≤ 60 years, no thrombosis history, JAK2 wildtype), low (same as very low but JAK2 mutation present), intermediate (age > 60 years, no thrombosis history, JAK2 wild-type) and high (thrombosis history present or age > 60 years with JAK2 mutation). Risk-adapted therapy: The main goal of therapy in both PV and ET is to prevent thrombohemorrhagic complications. All patients with PV require phlebotomy to keep hematocrit below 45% and once-daily or twice-daily aspirin (81 mg), in the absence of contraindications. Very low risk ET might not require therapy while aspirin therapy is advised for low risk disease. Cytoreductive therapy is recommended for high-risk ET and PV, but it is not mandatory for intermediate-risk ET. First-line drug of choice for cytoreductive therapy, in both ET and PV, is hydroxyurea and second-line drugs

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Section: Bothmentioning

confidence: 99%

Section: Bothmentioning

confidence: 99%

Section: Bothmentioning

confidence: 99%

Section: Risk‐adapted Therapymentioning

confidence: 99%

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Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk‐stratification and management

2020

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“…Amongst patients with MPN, ET is the most prevalent in young women; accordingly, course of pregnancy in the context of ET has been well described over the last several decades through numerous retrospective reports 26‐46 . The only prospective observational study of pregnancies in MPN was conducted in the UK (2010‐2012) describing a total of 58 pregnancies in MPN, of which 47 pregnancies occurred in the context of ET, five each with PV and PMF, and one patient with MPN‐unclassified 47 .…”

Section: Pregnancy Outcomes In Etmentioning

confidence: 99%

Myeloproliferative neoplasms and pregnancy: Overview and practice recommendations

Gangat

Tefferi

2020

American J Hematol

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Pregnancy in the context of myeloproliferative neoplasms (MPN) poses unique fetal and maternal challenges. Current literature in this regard mostly involves essential thrombocythemia (ET) and less so polycythemia vera (PV) or myelofibrosis. In ET, live birth rate is estimated at 70% with first trimester fetal loss (˜ 30%) as the major complication. Risk of pregnancy‐associated complications is higher in PV, thus mandating a more aggressive treatment approach. Herein, we appraise the relevant literature, share our own experience and propose management recommendations. Aspirin therapy may offer protection against fetal loss; however the additive benefit of systemic anticoagulation or cytoreductive therapy, in the absence of high risk disease, is unclear. We recommend cytoreductive therapy in the form of interferon alpha in all high risk and select low‐risk ET and PV patients with history of recurrent fetal loss, prominent splenomegaly or suboptimal hematocrit control with phlebotomy. In addition, all women with PV should maintain strict hematocrit control <45% with the aid of phlebotomy. Systemic anticoagulation with low molecular weight heparin is advised in patients with history of venous thrombosis. Further clarification awaits prospective clinical trials that implement risk adapted therapeutic interventions.

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The international consensus classification of myeloid neoplasms and acute Leukemias: myeloproliferative neoplasms

Thiele

Kvasnicka²,

Orazi

et al. 2022

American J Hematol

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A group of international experts, including hematopathologists, oncologists, and geneticists were recently summoned (September 2021, Chicago, IL, USA) to update the 2016/17 World Health Organization classification system for hematopoietic tumors. After careful deliberation, the group introduced the new International Consensus Classification (ICC) for Myeloid Neoplasms and Acute Leukemias. This current in‐depth review focuses on the ICC‐2022 category of JAK2 mutation‐prevalent myeloproliferative neoplasms (MPNs): essential thrombocythemia, polycythemia vera, primary myelofibrosis, and MPN, unclassifiable. The ICC MPN subcommittee chose to preserve the primary role of bone marrow morphology in disease classification and diagnostics, while also acknowledging the complementary role of genetic markers for establishing clonality, facilitating MPN subtype designation, and disease prognostication.

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Pregnancy outcomes in myeloproliferative neoplasms: A Mayo Clinic report on 102 pregnancies

Cited by 15 publications

References 8 publications

Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk‐stratification and management

Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk‐stratification and management

Myeloproliferative neoplasms and pregnancy: Overview and practice recommendations

The international consensus classification of myeloid neoplasms and acute Leukemias: myeloproliferative neoplasms

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