title: SIL-TAL1 fusion gene negative impact in T-cell acute lymphoblastic leukemia outcome.
abstract: SIL-TAL1 fusion gene and the ectopic expression of HOX11L2 are common molecular abnormalities in T-cell acute lymphoblastic leukemia (T-ALL). To verify their influence on outcome, we analyzed a Brazilian pediatric T-ALL series of cases. One hundred and ninety two children, age ranged 0-21 years old, were consecutively diagnosed and treated. Reverse transcriptase-polymerase chain reaction (RT-PCR) technique was used to identify the molecular alterations. Kaplan-Meyer method was applied to estimate overall survival. The most frequent maturation stage was T-IV (40.1%), and 30.7% of cases were CD10(+). SIL-TAL1(+) and HOX11L2(+) accounted for 26.7% and 10.3% of the cases, respectively. The overall survival (OS) was 74% in 80-month follow-up. HOX11L2(+) was not predictive factor for outcome. Considering patients younger than nine years-old, those with SIL-TAL1(+) presented a poorer outcome (p = 0.02). The results of this study suggest that in the Brazilian population only the presence of SIL-TAL1 can predict outcome in a restricted group of patients.
authors: Programa de Hematologia-Oncologia Pediátricas - Coordenação de Pesquisa, Instituto Nacional de Câncer, Rio de Janeiro, Brazil.
authors: Marcela Braga
authors: MB
authors: Mansur
authors: Mariana
authors: M
authors: Emerenciano
authors: Lilian
authors: L
authors: Brewer
authors: Mariana
authors: M
authors: Sant'Ana
authors: Núbia
authors: N
authors: Mendonça
authors: Luiz Claudio Santos
authors: LC
authors: Thuler
authors: Sérgio
authors: S
authors: Koifman
authors: Maria S
authors: MS
authors: Pombo-de-Oliveira
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018398:Homeodomain Proteins / Q000032:analysis* / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000097:blood* / Q000235:genetics; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood / Q000188:drug therapy / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D020133:Reverse Transcriptase Polymerase Chain Reaction; D055815:Young Adult
chemical_list: D018398:Homeodomain Proteins; D015514:Oncogene Proteins, Fusion; C547584:SIL-TAL1 fusion protein, human; C489494:TLX3 protein, human