Myeloproliferative neoplasms are rare at a young age, and few reports have described the disease characteristics and outcomes in this group. This study aimed to elucidate the clinical course of essential thrombocythemia (ET) and polycythemia vera (PV) in children and young adults aged <39 years focusing on thromboembolic events (TE) and second primary malignancies (SPMs). A total of 990 patients who were diagnosed from 2008 to 2017 were included by analyzing the Health Insurance Review and Assessment Service database in Korea. The incidence was 2.53 per 1,000,000 for ET (643 patients; 276 male patients; median 31 years) and 1.37 per 1,000,000 for PV (347 patients; 309 male patients; median 32 years). Three ET patients developed secondary acute myelogenous leukemia and three developed secondary myelofibrosis. The 5-year cumulative incidence of TE was 14.2% in ET and 21.3% in PV. Thus, the incidence was higher in PV; in particular, arterial TE (ATE) was evidently higher in PV than in ET. The 5-year cumulative incidence of SPMs was 2.5% in ET and 2.6% in PV. While the use of both aspirin and hydroxyurea reduced the incidence of ATE, hydroxyurea significantly increased the incidence of SPMs. The incidence of ET and PV was very low, and ET was more common than PV in children and young adults. The high incidence of TE in young patients suggests the importance of thrombosis prevention. However, hydroxyurea appears to increase the incidence of SPMs; therefore, the risks and benefits should be considered.
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The data that support the findings of this study are available from the corresponding author upon reasonable request.
Acute lymphoblastic leukemia
Arterial thromboembolic events
Acute coronary syndrome
Adolescent and young adults
Cerebral vein thrombosis
Deep vein thrombosis
Health Insurance Review and Assessment Service
National Health Insurance Service
Secondary acute myeloid leukemia
Second primary malignancies
Venous thromboembolic events
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This work was supported by a research fund from Seoul National University Bundang Hospital (grant number: SNUBH 13-2018-018).
This study was exempted by the Institutional Review Board of Seoul National University Bundang Hospital (IRB Approval No. X-1803/459-905), and the need for informed consent from each patient was waived, as the authors did not have access to any identifying information. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008.
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Choi, H.S., Hong, J., Hwang, S.M. et al. Evaluation of the need for cytoreduction and its potential carcinogenicity in children and young adults with myeloproliferative neoplasms. Ann Hematol 100, 2567–2574 (2021). https://doi.org/10.1007/s00277-021-04527-7
- Essential thrombocythemia
- Polycythemia vera
- Second primary malignancy