Document Detail

Genetic mutation and early onset of T-cell leukemia in pediatric patients infected at birth with HTLV-I.
MedLine Citation:
PMID:  11755465     Owner:  NLM     Status:  MEDLINE    
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.
Maria S Pombo-de-Oliveira; Jane A Dobbin; Paula Loureiro; Davimar Borducchi; Raquel C Maia; Marcia A Fernandes; Geraldo B Cavalcanti; Shigeki Takemoto; Genoveffa Franchini
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Leukemia research     Volume:  26     ISSN:  0145-2126     ISO Abbreviation:  Leuk. Res.     Publication Date:  2002 Feb 
Date Detail:
Created Date:  2001-12-28     Completed Date:  2002-03-27     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  7706787     Medline TA:  Leuk Res     Country:  England    
Other Details:
Languages:  eng     Pagination:  155-61     Citation Subset:  IM    
Laboratorio de Marcadores Celulares and Hematology Service, Instituto Nacional de Cancer, Praca Cruz Vermelha 23, CEP 20230-130 Rio de Janeiro, Brazil.
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MeSH Terms
Age of Onset
Blotting, Southern
Child, Preschool
Enzyme-Linked Immunosorbent Assay
Exons / genetics
Gene Deletion
Genes, p16*
Genes, p53*
HTLV-I Infections / complications,  congenital*,  virology
Human T-lymphotropic virus 1 / isolation & purification
Hypercalcemia / etiology
Leukemia-Lymphoma, Adult T-Cell / complications,  epidemiology*,  genetics,  virology
Polymerase Chain Reaction
Proviruses / isolation & purification
Skin / pathology
Splenomegaly / etiology
Survival Analysis

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