MARCELA CAVALCANTE DE ANDRADE SILVA

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Autor e Coautores FMUSP-HC Normalizados: VANDERSON GERALDO ROCHA ×

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  • article 2 Citação(ões) na Scopus
    Prognostic assessment for chronic myelomonocytic leukemia in the context of the World Health Organization 2016 proposal: a multicenter study of 280 patients
    (2021) GONZALEZ, Jacqueline S.; PERUSINI, Maria Agustina; BASQUIERA, Ana L.; ALFONSO, Graciela; FANTL, Dorotea; LIMA, Walter Macedo; NUCIFORA, Elsa; LAZZARINO, Carolina; NOVOA, Viviana; SILVA, Marcela Cavalcanti de Andrade; LARRIPA, Irene B.; ROCHA, Vanderson; ARBELBIDE, Jorge; VELLOSO, Elvira D. R. P.; BELLI, Carolina B.
    Knowledge on chronic myelomonocytic leukemia (CMML) patients from Argentina and Brazil is limited. Our series of 280 patients depicted an older age at diagnosis (median 72 years old), 26% of aberrant karyotypes, and a prevalence of myelodysplastic (60%) and CMML-0 subtypes (56%). The median overall survival (OS) was 48.2 months for patients in CMML-0 (Ref.), 24.7 months for those in CMML-1 (HR = 2.0, p = 0.001), and 8.8 months for patients in CMML-2 (HR = 4.6, p < 0.001). In the CMML-0 category, median OS were different between myelodysplastic and myeloproliferative subtypes (63.7 vs 21.2 months, p < 0.001); however, no differences were observed within CMML-1 and CMML-2 subtypes (24.7 vs 23.7 months, p = 0.540, and 9.1 vs 8.2 months, p = 0.160). The prognostic impact of 24 variables and 7 prognostic systems was adjusted to the WHO 2016 after validating their usefulness. Multivariate analysis were performed, and the final model revealed Hb >= 8 -< 10g/dL (HR 1.7), Hb < 8g/dL (HR 2.8), poor karyotypes (HR 2.1), WHO 2016-CMML-1 (HR 2.1), and CMML-2 (HR 3.5) as independent adverse clinical parameters in our cohort with a borderline influence of platelets count < 50 x 10(9)/L (HR 1.4). We could validate several scoring systems, the WHO 2016 proposal and its prognostic capability, along with accessible covariates, on predicting the outcome in our series of CMML patients from Latin America.
  • article 19 Citação(ões) na Scopus
    Deletion of RUNX1 exons 1 and 2 associated with familial platelet disorder with propensity to acute myeloid leukemia
    (2018) SILVA, Marcela Cavalcante de Andrade; KREPISCHI, Ana Cristina Victorino; KULIKOWSKI, Leslie Domenici; ZANARDO, Evelin Aline; NARDINELLI, Luciana; LEAL, Aline Medeiros; COSTA, Silvia Souza; MUTO, Nair Hideki; ROCHA, Vanderson; VELLOSO, Elvira Deolinda Rodrigues Pereira
    Familial platelet disorder with propensity to acute myeloid leukemia (FPD/AML) associated with RUNX1 mutations is an autosomal dominant disorder included in the group of the myeloid neoplasms with germ line predisposition. We describe two brothers who were diagnosed with hematological malignancies (one with AML and the other with T-cell lymphoblastic lymphoma). There was a history of leukemia in the paternal family and two of their siblings presented with low platelet counts and no history of significant bleeding. A microdeletion encompassing exons 1-2 of RUNX1 (outside the cluster region of the Runt Homology domain and the transactivation domain) was detected in six family members using array-CGH and MLPA validation. A low platelet count was not present in all deletion carriers and, therefore, it should not be used as an indication for screening in suspected families and family members.