A Globally Applicable 'Triple AAA' Risk Model for Essential Thrombocythemia Based on Age, Absolute Neutrophil Count, and Absolute Lymphocyte Count
In: Blood, Jg. 138 (2021-11-05), S. 238-238
Online
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Zugriff:
Background The detrimental effect of leukocytosis on survival in myeloproliferative neoplasms (MPN) has been well established for primary myelofibrosis (PMF), polycythemia vera (PV) and essential thrombocythemia (ET ) (JCO. 2018;36:310; BJH. 2020;189:291) Previous studies have also implicated leukocytosis as a risk factor for leukemic transformation (Mayo Clin Proc. 2017;92:1118) and thrombosis in MPN (Blood Adv. 2019;3:1729). However, it is currently not clear as to which component(s) of white blood cells is responsible for these observations. In the current study, we sought to examine the individual prognostic contribution of absolute neutrophil (ANC), lymphocyte (ALC) and monocyte (AMC) counts, on overall (OS), leukemia-free (LFS), and myelofibrosis-free (MFFS) survival and in ET. Methods Study patients (n=349) were retrospectively recruited from the Mayo Clinic MPN database of 1,249 WHO-defined ET patients, evaluated over five decades (1967-2021), based on availability of information on ANC, ALC and AMC. Conventional criteria were used for diagnosis and definitions of major complications, including leukemic or fibrotic transformation (Blood 2016;127:2391). Conventional statistical methods were applied using JMP Pro 14.0.0 software package, SAS Institute, Cary, NC. Multivariable analyses included previously established risk factors for survival. Results 349 patients (median age 57 years, range 18-89; females 61%) with ET were included in the study: 46% JAK2, 34% CALR, 16% triple-negative and 4% MPL mutated; IPSET risk category high 24%, intermediate 41%, and low 35%; presenting median (range) values were 13.8 g/dL (11.1-16.4) for hemoglobin, 8.2 x 10(9)/L (3.2-52) for leukocyte count, and 859 x 10(9)/L (451-3460) for platelet count; palpable splenomegaly was present in 48 (14%); median followup was 10 years (range 0-47), during which time 118 deaths, 52 fibrotic progressions, and 14 leukemic transformations were documented. Multivariable analysis identified older age (p60 years, 3.1 (2.1-4.6; p60 years), 2 for increased ANC (≥8 x 10(9)/L) and 1 for ALC ( An external validation cohort from the University of Florence (n=485) confirmed the independent survival risk contribution from age >60 years (p Conclusions: The current study identifies increased ANC and decreased ALC as age-independent risk factors for survival in ET, thus allowing the development of a globally applicable simple to use "Triple A" risk model that is based on Age, ANC and ALC. Decreased ALC also predicted fibrotic and leukemic progression. Our observations suggest potential value for immune profiling as an additional prognostic tool in MPN. Figure 1 Figure 1. Disclosures Szuber: Novartis: Honoraria. Vannucchi: BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees; Incyte: Honoraria, Membership on an entity's Board of Directors or advisory committees; AbbVie: Membership on an entity's Board of Directors or advisory committees.
Titel: |
A Globally Applicable 'Triple AAA' Risk Model for Essential Thrombocythemia Based on Age, Absolute Neutrophil Count, and Absolute Lymphocyte Count
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Autor/in / Beteiligte Person: | Gangat, Naseema ; Szuber, Natasha ; Mannelli, Francesco ; Vannucchi, Alessandro M. ; Pardanani, Animesh ; Farrukh, Faiqa ; Barbui, Tiziano ; Tefferi, Ayalew ; Giuseppe Gaetano Loscocco ; Guglielmelli, Paola ; Valerio De Stefano ; Hanson, Curtis A. |
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Zeitschrift: | Blood, Jg. 138 (2021-11-05), S. 238-238 |
Veröffentlichung: | American Society of Hematology, 2021 |
Medientyp: | unknown |
ISSN: | 1528-0020 (print) ; 0006-4971 (print) |
DOI: | 10.1182/blood-2021-149140 |
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