Blood Res 2023; 58(S1):
Published online April 30, 2023
https://doi.org/10.5045/br.2023.2023038
© The Korean Society of Hematology
Correspondence to : Junshik Hong, M.D., Ph.D.
Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea
E-mail: hongjblood@snu.ac.kr
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Increasing knowledge of the molecular features of myeloproliferative neoplasms (MPNs) is being combined with existing prognostic models based on clinical, laboratory, and cytogenetic information. Mutation-enhanced international prognostic systems (MIPSS) for polycythemia vera (PV) and essential thrombocythemia (ET) have improved prognostic assessments. In the case of overt primary myelofibrosis (PMF), the MIPSS70 and its later revisions (MIPSS70+ and MIPSS70+ version 2.0) effectively predicted the overall survival (OS) of patients. Because post-PV and post-ET myelofibrosis have different biological and clinical courses compared to overt PMF, the myelofibrosis secondary to PV and ET-prognostic model was developed. Although these molecular-inspired prognostic models need to be further validated in future studies, they are expected to improve the prognostic power in patients with MPNs in the molecular era. Efforts are being made to predict survival after the use of specific drugs or allogeneic hematopoietic stem cell transplantation. These treatment outcome prediction models enable the establishment of personalized treatment strategies, thereby improving the OS of patients with MPNs.
Keywords Myeloproliferative neoplasms, Prognosis, Prognostic models, Myelofibrosis, Mutations
Blood Res 2023; 58(S1): S37-S45
Published online April 30, 2023 https://doi.org/10.5045/br.2023.2023038
Copyright © The Korean Society of Hematology.
Junshik Hong
Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
Correspondence to:Junshik Hong, M.D., Ph.D.
Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea
E-mail: hongjblood@snu.ac.kr
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Increasing knowledge of the molecular features of myeloproliferative neoplasms (MPNs) is being combined with existing prognostic models based on clinical, laboratory, and cytogenetic information. Mutation-enhanced international prognostic systems (MIPSS) for polycythemia vera (PV) and essential thrombocythemia (ET) have improved prognostic assessments. In the case of overt primary myelofibrosis (PMF), the MIPSS70 and its later revisions (MIPSS70+ and MIPSS70+ version 2.0) effectively predicted the overall survival (OS) of patients. Because post-PV and post-ET myelofibrosis have different biological and clinical courses compared to overt PMF, the myelofibrosis secondary to PV and ET-prognostic model was developed. Although these molecular-inspired prognostic models need to be further validated in future studies, they are expected to improve the prognostic power in patients with MPNs in the molecular era. Efforts are being made to predict survival after the use of specific drugs or allogeneic hematopoietic stem cell transplantation. These treatment outcome prediction models enable the establishment of personalized treatment strategies, thereby improving the OS of patients with MPNs.
Keywords: Myeloproliferative neoplasms, Prognosis, Prognostic models, Myelofibrosis, Mutations
Table 1 . Risk stratification of polycythemia vera: the classic risk model and the Molecular International Prognostic Scoring System for polycythemia vera (MIPSS-PV)..
Classical risk stratification for PV | MIPSS-PV | |
---|---|---|
Age ≥60 yr | Thrombosis history | 1 point |
Thrombosis history | WBC ≥15×109/L | 1 point |
Age >67 | 2 points | |
Mutated | 3 points |
Stratification and treatment | Sum of the points and interpretation | ||
---|---|---|---|
Low risk | None of them; no cytoreduction | Low risk | 0–1; mOS 24 yr |
High risk | Any of them; cytoreduction needed | Intermediate risk | 2–3; mOS 13.1 yr |
High risk | ≥4; mOS 3.2 yr |
Abbreviations: MIPSS-PV, Molecular International Prognostic Scoring System for polycythemia vera; mOS, median overall survival; PV, polycythemia vera; WBC, white blood cell count..
Table 2 . Risk stratification of essential thrombocythemia: the revised IPSET-thrombosis and the Molecular International Prognostic Scoring System for essential thrombocythemia (MIPSS-ET)..
Revised IPSET-thrombosis for ET | MIPSS-ET | |
---|---|---|
Thrombosis history | Male sex | 1 point |
Age >60 yr | WBC ≥11×109/L | 1 point |
Adverse mutationsb) | 2 points | |
Age >60 | 4 points |
Stratification and treatment | Sum of the points and interpretation | ||
---|---|---|---|
Very low risk | None of them; observationa) | Low risk | 0–1; mOS 34.4 yr |
Low risk | Intermediate risk | 2–5; mOS 14.1 yr | |
Intermediate risk | Age >60 yr only; aspirin | High risk | ≥6; mOS 7.9 yr |
High risk | Any others; cytoreduction |
a)Aspirin, if any cardiovascular risk factors are present. b)Mutations in
Abbreviations: ET, essential thrombocythemia; IPSET, International Prognostic Score for Essential Thrombocythemia; MIPSS-ET, Molecular International Prognostic Scoring System for essential thrombocythemia; mOS, median overall survival; WBC, white blood cell count..
Table 3 . Risk stratification of post-polycythemia vera or post-essential thrombocythemia myelofibrosis: the Myelofibrosis Secondary to PV and ET-Prognostic Model (MYSEC-PM)..
Risk variables and points | |
---|---|
Age at diagnosis | 0.15 points per yr |
Hemoglobin <11 g/dL | 2 points |
Circulating blast ≥3% | 2 points |
Absence of | 2 points |
Platelet count <150×109/L | 1 point |
Constitutional symptoms | 1 point |
Risk group and interpretation | |
---|---|
Low risk | <11 point; mOS not reached |
Intermediate-1 risk | ≥11 and <14 points; mOS 9.3 yr |
Intermediate-2 risk | ≥14 and <16 points; mOS 4.4 yr |
High risk | ≥16 points; mOS 2.0 yr |
Abbreviation: mOS, median overall survival..
Table 4 . Prognostication in myeloproliferative neoplasms according to mutational abnormalities..
Genes | Polycythemia vera | Essential thrombocythemia | Myelofibrosis | |
---|---|---|---|---|
Driver mutations | -. Associated with younger age, higher hemoglobin, lower leukocytes and platelet counts, but no difference in LFS, MFFS, and OS, compared to | |||
- | ||||
- | - | |||
Triple negativityb) | - | - | ||
Non-driver mutations | “Adverse variants/mutations” [24, 25] -. All: inferior OS. -. -. | - | Inferior LFS, OS [52] | |
Inferior PFS after HSCT [53] | ||||
- | Inferior LFS [52, 53] | |||
“Adverse variants/mutations” [24, 25] -. All: inferior OS. -. -. -. | Inferior PFS after HSCT [52, 53] | |||
- | ||||
Inferior LFS and OS [52] | ||||
- | Inferior LFS [54] | |||
- | : Inferior OS compared to | |||
: inferior OS post allogeneic HSCT | ||||
- | Inferior OS [52] | |||
- | - | |||
- | - | |||
- | Inferior OS [57] |
a)A>B: A has a higher thrombosis rate (or superior survival) than B. b)Triple negativity: no mutation in
Abbreviations: HSCT, hematopoietic stem cell transplantation; LFS, leukemia-free survival; MFFS, myelofibrosis-free survival; OS, overall survival..
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