Please use this identifier to cite or link to this item: https://ninho.inca.gov.br/jspui/handle/123456789/5921
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dc.contributor.authorNoronha, Elda Pereira-
dc.contributor.authorMarques, Luísa Vieira Codeço-
dc.contributor.authorAndrade, Francianne Gomes-
dc.contributor.authorSardou-Cezar, Ingrid-
dc.contributor.authorBueno, Filipe Vicente dos Santos-
dc.contributor.authorZampier, Carolina da Paz-
dc.contributor.authorPina, Eugênia Terra Granado-
dc.contributor.authorPombo-de-Oliveira, Maria do Socorro-
dc.date.accessioned2022-03-22T17:08:55Z-
dc.date.available2022-03-22T17:08:55Z-
dc.date.issued2019-05-
dc.identifier.issn1179-1322-
dc.identifier.other10.2147/CMAR.S196574-
dc.identifier.urihttp://sr-vmlxaph03:8080/jspui/handle/123456789/5921-
dc.description.abstractLuísa Vieira Codeço Marques Francianne Gomes Andrade Ingrid Sardou-Cezar FilipeVicentedosSantos-Bueno CarolinaDa PazZampier Eugênia Terra-Granado Maria SPombo-de-Oliveira Paediatric Haematology-Oncology Program, Research Centre, Instituto Nacional de Câncer, Rio de Janeiro, RJ, Brazil Purpose: T-lymphoid/Myeloid Mixed phenotype acute leukemia (T/M-MPAL) is ambiguous leukemia which overlaps with early T-cell precursor lymphoblastic leukemia (ETPALL). We have revisited the immunophenotyping profile of T/M-MPAL and ETP-ALL to identify differences and/or similarities, as these entities represent a therapeutic challenge in clinical practice. Patients and methods: A total of 26 ETP-ALL and 10 T/M-MPAL cases were identified among 857 cases of childhood leukemia (T-ALL, n=266 and AML, n=591) before any treatment decisions. The variables analyzed were age strata, sex, clinical features, immunophenotyping, and molecular aberrations. Immunophenotyping was performed in all samples using a panel of cytoplasm and membrane antibodies to identify the lineage and blast differentiation. The mutational status of STIL-TAL1, TLX3, RUNX1, NOTCH1, FBXW7, FLT3, IL7R, RAS, KTM2A, and CDKN2A/B was tested using RT-PCR, FISH, and PCR sequencing methods. The outcomes were assessed in terms of overall survival (OS). Results: The immunophenotypes were similar in ETP-ALL and T/M-MPAL, regarding the cellular expression of CD34, CD117, CD13/CD33, and CD11b, although CD2 and HLA-DR were more frequent in T/M-MPAL (p<0.01). aMPO positivity and myelomonocyte differentiation were definitive in separating both entities. NOTCH1, FLT3-ITD, and N/KRAS mutations as well as TLX3 and KMT2A rearrangements were found in both ETP-ALL and T/M-MPAL. Thirty-one patients received ALL protocol whereas five had AML therapy. The overall 5-year survival rate (pOS) was 56.4% for patients treated using ALL protocols. No differences were observed between T/M-MPAL (pOS of 57%) and ETP-ALL (pOS of 56%) patients. The prognostic value of NOTCH1mut was associated with significantly better OS (pOS 90%) than NOTCH1wt (pOS 37%) (p=0.017). Conclusion: This research can potentially contribute to NOTCH1 as targeted therapy and prognostic assessment of T-cell mixed phenotype leukemia.en
dc.publisherCancer Management and Researchpt_BR
dc.subjectPrecursor T-Cell Lymphoblastic Leukemia-Lymphomapt_BR
dc.subjectReceptor, Notch1pt_BR
dc.subject.otherT-lymphoid/myeloid mixed phenotype acute leukemiaen
dc.subject.otherNOTCH1 mutationsen
dc.titleT-lymphoid/myeloid mixed phenotype acute leukemia and early T-cell precursor lymphoblastic leukemia similarities with NOTCH1 mutation as a good prognostic factorpt_BR
dc.TypeArticlept_BR
Appears in Collections:Artigo de Periódicos da Pesquisa Clínica



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