To distinguish the similarities or differences between T-cell acute lymphoblastic leukaemia (T-ALL) and T-cell lymphoblastic lymphoma (T-LBL), we retrospectively analyzed the clinical, immunophenotypic, cytogenetic, and molecular characteristics in 37 children diagnosed between December 1990 and December 2003. Comparative Expressed Sequence Hybridisation (CESH) was used to determine gene expressing profile in both diseases. Twenty two patients suffered from T-ALL and 15 patients were diagnosed as T-LBL. Immunophenotyping demonstrated a more immature phenotype in T-ALL and a more mature phenotype in T-LBL. Cytogenetic and molecular genetic aberrations were found in 82% of T-ALL compared with 73% of T-LBL. By CESH gene expression profiling, the investigated cases were segregated into two groups that largely corresponded with T-ALL and T-LBL. The clinical presentation and cytogenetic characteristics are largely similar for T-ALL and T-LBL supporting the concept that both represent a spectrum of one single disease. The differences that were found between both neoplasms, in particular in their phenotype and in their expression profile may suggest that most T-ALL derive from a T-cell progenitor of the bone marrow, while thymocytes represent the normal counterpart of T-LBL.
"*P < 0AE003. T-LBL: current study (JPLSG ALB-NHL03) combined with three published reports(Burkhardt et al, 2006; Lones et al, 2007; Uyttebroeck et al, 2007). T-ALL: combined two published reports (Heerema et al, 1998; Schneider et al, 2000). "
[Show abstract][Hide abstract] ABSTRACT: T-cell acute lymphoblastic leukaemia (T-ALL) and T-cell lymphoblastic lymphoma (T-LBL) are combined into one category as T lymphoblastic leukaemia/lymphoma in the current World Health Organization (WHO) classification. However, there is still ongoing discussion on whether T-ALL and T-LBL are two separate entities or represent two variant phenotypes of the same disease. Cytogenetic analysis has been used to identify the molecular background of haematological malignancies. To compare the distribution of chromosomal abnormalities of T-ALL and T-LBL, large series of cytogenetic data are required, but are absent in T-LBL in contrast to the abundant data in T-ALL. Among 111 T-LBL cases in our clinical trial, we obtained complete cytogenetic data from 56 patients. The comparison between our cytogenetic findings and those from three published T-LBL studies revealed no significant difference. However, meta-analysis showed that translocations involving chromosome region 9q34 were significantly more common in T-LBL than in T-ALL. In particular, four out of the 92 T-LBL cases, but none of the 523 paediatric T-ALL cases, showed translocation t(9;17)(q34;q22-23) (P=0·0004). Further studies are needed for the possible linkage between abnormal expression of genes located at 9q34 and/or 17q22-23 and the unique 'lymphoma phenotype' of T-LBL.
British Journal of Haematology 06/2011; 154(5):612-7. DOI:10.1111/j.1365-2141.2011.08788.x · 4.71 Impact Factor
"In a second series, 10 paediatric T-ALL and eight T-LBL were available for comparative expressed sequence hybridization (CESH), which identified chromosomal regions corresponding to differential gene expression (Uyttebroeck et al, 2007). RNA was isolated from BM in T-ALL or lymph node biopsies in T-LBL cases and cohybridized with a reference sample of a RNA mixture isolated from five reactive lymph node biopsies. "
[Show abstract][Hide abstract] ABSTRACT: There is ongoing discussion on whether paediatric acute T-cell lymphoblastic leukaemia (T-ALL) and paediatric lymphoblastic T-cell lymphoma (T-LBL) are two distinct entities or whether they represent two variant manifestations of one and the same disease and the distinction is arbitrary. Both show overlapping clinical, morphological and immunophenotypic features. Many clinical trials use the amount of blast infiltration of the bone marrow as the sole criterion to distinguish between T-ALL and T-LBL. The current World Health Organization classification designates both malignancies as T lymphoblastic leukaemia/lymphoma. However, subtle immunophenotypic, molecular and cytogenetic differences suggest that T-ALL and T-LBL might be biologically different in certain aspects. The current review summarizes and discusses the recent advances and understanding of the molecular profile of paediatric T-ALL and T-LBL.
British Journal of Haematology 11/2009; 149(5):653-68. DOI:10.1111/j.1365-2141.2009.08006.x · 4.71 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: T-cell neoplasms encompass a heterogeneous group of relatively rare disease entities. This review, focused on lymphoblastic tumors (T-ALL/LBL) and nodal-based peripheral T-cell lymphomas (PTCL), summarizes recent advances in the molecular characterization of these diseases. In T-ALL/LBL, molecular subgroups delineated by gene expression profiling correlate with leukemic arrest at specific stages of normal thymocyte development and different oncogenic pathways, and seem to be of interest for prognosis prediction. Angioimmunoblastic T-cell lymphoma (AITL), one of the most common PTCL entities, comprises neoplastic cells with a molecular signature similar to normal follicular helper T cells, and this cellular derivation might account for several of the peculiar aspects of this disease. Except in ALK-positive anaplastic large cell lymphoma, defined by ALK gene fusions, chromosomal translocations are otherwise rare in PTCLs, but some recurrent rearrangements might be associated with distinct lymphoma subtypes. In PTCL, not otherwise specified (PTCL, NOS), novel molecular biomarkers of potential therapeutic interest have been recently identified.
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