Genetic mutation and early onset of T-cell leukemia in pediatric patients infected at birth with HTLV-I

Genetic mutation and early onset of T-cell leukemia in pediatric patients infected at birth with HTLV-I

Autor Pombo-de-Oliveira, M. S. Google Scholar
Dobbin, J. A. Google Scholar
Loureiro, P. Google Scholar
Borducchi, D. Google Scholar
Maia, R. C. Google Scholar
Fernandes, M. A. Google Scholar
Cavalcanti, G. B. Google Scholar
Takemoto, S. Google Scholar
Franchini, G. Google Scholar
Instituição Inst Nacl Canc
Universidade Federal de São Paulo (UNIFESP)
Resumo T-cell leukemia/lymphoma (T-c LL) associated with prior infection with HTLV-I is rarely described in children. We present herein, the clinical, morphological, and virologic features of T-c LL, which occurred in eight pediatric cases with similar features of ATLL described in adults. There were three girls and five boys with age ranging from 2 to 18 years. Lymphoadenopathy, hepatosplenomegaly and marked skin lesions were presented in all cases. Five patients had hypercalcemia. the diagnostic criteria of T-c LL were based on both morphological and immunophenotypical analyses characterized by T-cell markers positively. Seven cases were cCD3 +, CD4/CD25 +, whereas CD1a and TdT were negative in all cases tested. HTLV-I antibodies were detected in all cases. HTLV-I provirus integration of at least one provirus was seen in all cases tested by molecular analysis. Mother-to-child transmission of HTLV-I was demonstrated in six cases. Interestingly, a homozygous deletion in p16 gene locus was observed in all four cases studied, while exons 7 and 8 of p53 were deleted in one child. the deletion of the p16(INK4A)/p14(ARF) or mutation of p53, key regulatory protein of cell cycle checkpoint in G1/S progression, found in five of the eight pediatric patients suggests that in these cases genetic lesions associated with HTLV-I infection may predispose for an early onset of leukemia. (C) 2002 Elsevier B.V. All rights reserved.
Assunto T-cell leukemia
pediatric patients
Idioma Inglês
Data 2002-02-01
Publicado em Leukemia Research. Oxford: Pergamon-Elsevier B.V., v. 26, n. 2, p. 155-161, 2002.
ISSN 0145-2126 (Sherpa/Romeo, fator de impacto)
Editor Elsevier B.V.
Extensão 155-161
Direito de acesso Acesso restrito
Tipo Artigo
Web of Science WOS:000173774400004

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