Myeloproliferative neoplasms in the young: Mayo Clinic experience with 361 patients age 40 years or younger

American Journal of Hematology
Natasha SzuberAyalew Tefferi

Abstract

Between 1967 and 2017, 361 patients with myeloproliferative neoplasms (MPN), age ≤ 40 years, were seen at our institution, constituting 12% of all MPN patients (n = 3023) seen during the same time period; disease-specific incidences were 12% in polycythemia vera (PV; n = 79), 20% in essential thrombocythemia (ET; n = 219) and 5% in primary myelofibrosis (PMF; n = 63). Compared to their older counterparts, younger patients were more likely to present with low risk disease (P < .001) and display female preponderance in ET (P = .04), lower incidence of arterial events overall (P < .001), and higher incidence of venous thrombosis in PV (P = .01). Younger patients were also more likely to express CALR mutations, in ET and PMF, normal karyotype, in PV and PMF, and lower incidence of high molecular risk mutations in PMF (P significant in all instances). Over median follow-up of 11.3, 13, and 7.1 years for PV, ET, and PMF, leukemic transformations were respectively documented in 4%, 2%, and 10% (P values 0.1-0.9) while incidences of fibrotic progression in PV (22%) and ET (16%) were expectedly higher in young patients, because of their longer survival (P < .001). Median survival in young patients was 37 years for PV, 35 for ET and 20 f...Continue Reading

References

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Citations

Oct 4, 2018·American Journal of Hematology·Ayalew Tefferi, Tiziano Barbui
Nov 15, 2018·Leukemia & Lymphoma·Ayalew Tefferi, Animesh Pardanani
Apr 4, 2019·European Journal of Haematology·Merav BarzilaiNoa Lavi
Mar 31, 2019·Expert Review of Hematology·Maria Luigia RandiMaria Caterina Putti
May 24, 2019·European Journal of Haematology·Vincenzo AccursoSergio Siragusa
Sep 14, 2019·Therapeutic Advances in Hematology·Prithviraj Bose, Srdan Verstovsek
Feb 6, 2020·American Journal of Hematology·Naseema GangatAyalew Tefferi
May 28, 2019·BMC Research Notes·Jonathan HeppnerFariborz Rashid-Kolvear
Mar 17, 2020·Current Hematologic Malignancy Reports·Nicole Kucine
Sep 26, 2020·American Journal of Hematology·Ayalew Tefferi, Tiziano Barbui
Dec 10, 2020·American Journal of Hematology·Naseema Gangat, Ayalew Tefferi
Feb 2, 2021·American Journal of Hematology·Naseema GangatAyalew Tefferi
Apr 29, 2021·Blood Cancer Journal·Paola GuglielmelliAyalew Tefferi
Apr 22, 2021·HemaSphere·Andrew J Robinson, Anna L Godfrey
Jun 12, 2021·Seminars in Thrombosis and Hemostasis·Omri CohenWalter Ageno
Jun 15, 2021·British Journal of Haematology·Naseema GangatAyalew Tefferi
Jun 25, 2021·BMJ Case Reports·Maria VetluzhskayaValery Podzolkov
Sep 4, 2021·Current Hematologic Malignancy Reports·Joan How, Gabriela Hobbs

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