Age, Blasts, Performance Status and Lenalidomide Therapy Influence the Outcome of Myelodysplastic Syndrome With Isolated Del(5q): A Study of 58 South American Patients.


Journal

Clinical lymphoma, myeloma & leukemia
ISSN: 2152-2669
Titre abrégé: Clin Lymphoma Myeloma Leuk
Pays: United States
ID NLM: 101525386

Informations de publication

Date de publication:
01 2022
Historique:
received: 14 06 2021
revised: 14 07 2021
accepted: 19 07 2021
pubmed: 26 8 2021
medline: 1 4 2022
entrez: 25 8 2021
Statut: ppublish

Résumé

Myelodysplastic Syndrome (MDS) with isolated deletion 5q is associated with a low risk to leukemic evolution and long overall survival (OS); it comprises 3%-4.5% of MDS cases in Latin America classified according to the World Health Organization 2008. This study aims to describe clinical, laboratory and the outcome of patients according to the newest World Health Organization 2016 proposal. We retrospectively reviewed patients from four Brazilian (BR) and four Argentinean (AR) centers diagnosed between 1999 and 2019. The 58 patients (16-AR and 42-BR) presented a median age of 67 (IQR 61-75) years old, women predominance (70.7%) and transfusion dependency (62.5%) at diagnosis. Median hemoglobin level was 8.1g/dL, 27.5% and 44.4% presented thrombocytosis and neutropenia, respectively. Bone marrow (BM) was predominantly hypercellular (43.1%) with 66% showing dysplasia >1 lineage and 37.9% with >2% of blasts. Deletion 5q was mostly isolated (79.3%) and a variety of abnormalities were observed in remaining cases. Most patients were treated with erythropoietin-stimulating agents (ESA), 18 with lenalidomide and 15 with thalidomide. Median follow-up was 7.6 years, with a median OS of 3.5 years and an 8-years leukemic evolution rate of 18.4%. Multivariate analysis showed that age >75 years (HR 2.19), ECOG ≥2 (HR 5.76), BM blasts >2% (HR 2.92) and lenalidomide treatment (HR 0.25) independently influenced the OS. Older age, worse performance status and higher percentage of blasts, that can be easily assessed, were associated to a worse prognosis. Also, our results corroborate the protective influence of lenalidomide in terms of OS in this South American series.

Sections du résumé

BACKGROUND
Myelodysplastic Syndrome (MDS) with isolated deletion 5q is associated with a low risk to leukemic evolution and long overall survival (OS); it comprises 3%-4.5% of MDS cases in Latin America classified according to the World Health Organization 2008. This study aims to describe clinical, laboratory and the outcome of patients according to the newest World Health Organization 2016 proposal.
METHODS
We retrospectively reviewed patients from four Brazilian (BR) and four Argentinean (AR) centers diagnosed between 1999 and 2019.
RESULTS
The 58 patients (16-AR and 42-BR) presented a median age of 67 (IQR 61-75) years old, women predominance (70.7%) and transfusion dependency (62.5%) at diagnosis. Median hemoglobin level was 8.1g/dL, 27.5% and 44.4% presented thrombocytosis and neutropenia, respectively. Bone marrow (BM) was predominantly hypercellular (43.1%) with 66% showing dysplasia >1 lineage and 37.9% with >2% of blasts. Deletion 5q was mostly isolated (79.3%) and a variety of abnormalities were observed in remaining cases. Most patients were treated with erythropoietin-stimulating agents (ESA), 18 with lenalidomide and 15 with thalidomide. Median follow-up was 7.6 years, with a median OS of 3.5 years and an 8-years leukemic evolution rate of 18.4%. Multivariate analysis showed that age >75 years (HR 2.19), ECOG ≥2 (HR 5.76), BM blasts >2% (HR 2.92) and lenalidomide treatment (HR 0.25) independently influenced the OS.
CONCLUSION
Older age, worse performance status and higher percentage of blasts, that can be easily assessed, were associated to a worse prognosis. Also, our results corroborate the protective influence of lenalidomide in terms of OS in this South American series.

Identifiants

pubmed: 34429274
pii: S2152-2650(21)00308-6
doi: 10.1016/j.clml.2021.07.026
pii:
doi:

Substances chimiques

Lenalidomide F0P408N6V4

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e1-e6

Informations de copyright

Copyright © 2021 Elsevier Inc. All rights reserved.

Auteurs

R S Azevedo (RS)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil. Electronic address: r.sazevedo@hotmail.com.

C Belli (C)

Laboratorio de Genética Hematológica, Instituto de Medicina Experimental (IMEX-CONICET)/Academia Nacional de Medicina; On behalf of the Grupo de Estudio de SMD, Sociedad Argentina de Hematología, Buenos Aires, Argentina.

L Bassolli (L)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil.

L Ferri (L)

Department of Pathology, University of Sao Paulo Medical School, Sao Paulo, Brazil.

M A Perusini (MA)

On behalf of the Grupo de Estudio de SMD, Sociedad Argentina de Hematología, Buenos Aires, Argentina; Hematology Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina.

A Enrico (A)

On behalf of the Grupo de Estudio de SMD, Sociedad Argentina de Hematología, Buenos Aires, Argentina; Hematology Department, Hospital Italiano de La Plata, Buenos Aires, Argentina.

Tdm Pereira (T)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil.

Wfs Junior (W)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil.

V Buccheri (V)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil.

R F Pinheiro (RF)

Federal University of Ceara, Departament of Internal Medicine, Ceara, Brazil.

S M Magalhaes (SM)

Federal University of Ceara, Departament of Internal Medicine, Ceara, Brazil.

S Schuster (S)

Hematology Department, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil.

J B Castelli (JB)

Laboratory of Pathology of the Heart Institute (InCor), Hospital das Clinicas (HC-FMUSP), University of Sao Paulo, Sao Paulo, Brazil; The Fleury Group, Sao Paulo/SP, Brazil.

F Traina (F)

Department of Imaging, Hematology and Oncology, University of Sao Paulo at Ribeirao Preto Medical School, Ribeirao Preto, Sao Paulo, Brazil.

V Rocha (V)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil.

Edrp Velloso (E)

Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil; Genetics Laboratory, Hospital Israelita Albert Einstein, Sao Paulo, Brazil.

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