IMEX   05356
INSTITUTO DE MEDICINA EXPERIMENTAL
Unidad Ejecutora - UE
congresos y reuniones científicas
Título:
Age, performance status and lenalidomide therapy independently influence the outcome of patients with Myelodysplastic Syndrome with isolated del(5q) from South America
Autor/es:
BELLI, CAROLINA; FEITOSA PINHEIRO RONALD; ARAUJO SCHUSTER, SERGIO; PERUSINI, MARIA AGUSTINA; MAGALHAES, SILVIA; ROCHA, VANDERSON; AZEVEDO, ROSANA; FERRI, L; TRAIANA, FABIOLA
Lugar:
Campiñas
Reunión:
Congreso; Congreso Brasileño de Hematología, Hemoterapia y Terapia Celular (HEMO 2020; 2020
Institución organizadora:
Sociedad Brasilera de Hematología y Hemoterapia
Resumen:
Background: MDS are heterogeneous clonal disorders of hematopoietic stem cells. MDS with isolated deletion 5q is the most distinct group of MDS, comprises 3- 4.5% of MDS cases in Latin America and seems to have a favorable prognosis, with a lower risk of AML progress and longer overall survival (OS). Some factors have been correlated with a poor prognosis, such as dysgranulopoiesis, age over 70 years, transfusion dependency and TP53 mutation. This study aims to describe clinical, laboratory and outcome of patients with MDS with isolated del(5q) in South America.Methods: We retrospectively reviewed patients with MDS with isolated del(5q) who met WHO 2016 criteria from 4 Brazilian (1 in the Northeast and 3 in the Southeast) diagnosed between 1999 and 2009 and from 4 Argentinean centers diagnosed between 2003 and 2019. OS and leukemia progression were estimated using the Kaplan-Meier method and compared according to the Cox model. All variables with a p-value lower than 0.05 were included in the final multivariate model.Results: The 58 patients (16 Argentinean and 42 Brazilian) included presented a median age of 67.3 (61-75) years old, predominance of females (70.7%) and transfusion dependency in 60.3% of patients. The median Hb level was 8.0 (3.5-17.6) g/dL, while thrombocytosis was present in 27.5% and neutropenia in 44.4%. Bone marrow (BM) trephine was predominantly hypercellular (43.1%). Dysplasia >1 lineage was found in 33 (56.9%) patients and 22 (37.9%) had > 2% of blasts in BM aspirate. Fibrosis greater than grade 1 was documented in 6.9% (4/34 samples). The deletion 5q was isolated in 65.5%. p53>1% by immunohistochemistry was found in 26.3% (5/19 BM studies at diagnosis) and only one patient (from 18 samples) showed del17p by FISH. Most patients were treated with ESA, 17 received lenalidomide and 15 thalidomide (only Brazilian). With a median follow-up of 7.6 (range 3.2-10.5) years, 56.8% died including 6 related to AML progression and one patient with concomitant AML and DLBC Lymphoma. The median OS was 43.2 (36-78) months and to AML progression of 6.9 (range, 6.5-7.3) years with a probability of 18.4% over 8 years. Univariate analysis showed that age > 67.3 years old, ECOG ≥ 2, percentage of BM blasts > 2% and fibrosis in BM biopsy > 1 were associated with lower OS, while lenalidomide treatment was the only factor associated with its improvement. Multivariate analysis confirmed the independence of ECOG ≥ 2 (HR 6.88 [CI95% 2.25 ? 21.09], p ≤0.001), > 2% of BM blasts (HR 3.66 [CI95% 1.09 ? 12.30], p=0.04) and lenalidomide treatment (HR 0.31 [CI95% 0.12-0.80], p=0.016) in terms of OS. Conclusion: The median OS of our series seems slightly lower than previously published. Some factors that can be easily assessed at diagnosis as performance status and the percentage of BM blasts are associate to prognosis. And, the use of lenalidomide was associated with a better outcome, with a heterogeneity of access among patients due to the retrospective characteristic of the study. The assessment of the impact of TP53 requires that more centers implements its detection by immunohistochemistry, molecular biology and FISH, which are not routinely performed in Brazilian and Argentinean services.