The prognostic scoring system for chronic myelomonocytic leukemia (cpss) in the context of the new classification of the world health organization 2016
Vol. 23 No. 1 (2019), Original articles
Vol. 23 No. 1 (2019)

The prognostic scoring system for chronic myelomonocytic leukemia (cpss) in the context of the new classification of the world health organization 2016

Original articles
Published 2019-06-27
Jacqueline Gonzalez
A Perusini
Hospital Italiano de Buenos Aires
- Romagnoli
HGA “C Durand”; Buenos Aires
Dorotea Fantl
Hospital Italiano de Buenos Aires
W Correa
Hospital Italiano de Buenos Aires - En representación del Grupo de SMD de la SAH
M Rosenhain
Hematología, Hospital General de Agudos “E Tornú”
N Pintos
Hematología, Sanatorio Municipal “J Mendez”
W Macedo Lima
Hospital das Clínicas, Facultad de Medicina, Universidad de Sao Paulo, Brasil
Irene Larripa
6- Laboratorio de Genética Hematológica, Instituto de Medicina Experimental (IMEX-CONICET)/Academia Nacional de Medicina (ANM); Buenos Aires
Jorge Arbelbide
Hospital Italiano de Buenos Aires
E Velloso
Hospital das Clínicas, Facultad de Medicina, Universidad de Sao Paulo, Brasil
Carolina Belli
6- Laboratorio de Genética Hematológica, Instituto de Medicina Experimental (IMEX-CONICET)/Academia Nacional de Medicina (ANM); Buenos Aires
Revista Hematología ENERO - ABRIL 2019
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Keywords

Chronic Myelomonocytic Leukemia, CMML-Prognostic-Scoring-System, WHO-2016, prognosis, survival, leukemic evolution

How to Cite

Gonzalez, J., Perusini, A., Romagnoli, .-., Fantl, D., Correa, W., Rosenhain, M., Pintos, N., Macedo Lima, W., Larripa, I., Arbelbide, J., Velloso, E., & Belli, C. (2019). The prognostic scoring system for chronic myelomonocytic leukemia (cpss) in the context of the new classification of the world health organization 2016. Journal of Hematology, 23(1), 13–23. Retrieved from https://revistahematologia.com.ar/index.php/Revista/article/view/69
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Abstract

Chronic Myelomonocytic Leukemia (CMML) is a clonal disorder sharing features of myelodysplastic syndromes (MD) and myeloproliferative (MP) disorders. The CMML-Prognostic-Scoring-System (CPSS) was designed specifically for these patients. Recently, the WHO committee proposed classifying them into: CMML-0, -1 and 2. Since both proposals have not been widely reproduced, our aim was to validate them and to evaluate different prognostic factors in a series of 277 patients with CMML from South America (197-Argentina and 80-Brazil).

CMML patients showed a median age of 72.6 years and a gender ratio M/F: 2.1. With a median overall survival of 32.6 months, 61(22.0%) evolved to AML and 157(56.7%) died. All evaluated variables, including the WHO-2016 proposal and all prognostic scoring systems, were useful to predict the outcome in our population (Kaplan-Meier and log-rank test, p<0.05). The CPSS-Hb (hemoglobin threshold of 10g/dL) sustained its independence for survival (p<0.001, HR1.856) and evolution to AML (p<0.001, HR 2.275) in a multivariate model including the other CPSS variants (Cox-Regression, Backward-Stepwise method). Afterward, variables not originally considered into the CPSS were included in multivariate analyses. The WHO proposal sustained its independency as an adverse prognostic factor to predict survival and evolution to AML (p=0.005 and p=0.001, HR1.420 and 1.964) when compared with the CPSS-Hb variant (p<0.001, HR1.741 and 1.890). Also, IPSS-R platelet cut-points were relevant to predict leukemic evolution (p=0.003, HR 2.929).

Our results showed that the new WHO-2016 proposal adds prognostic information to the CPSS, and that platelet count is also important to predict leukemic evolution in CMML patients.

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