Is there a difference in childhood T-cell acute lymphoblastic leukemia and T-cell lymphoblastic lymphoma?

Department of Laboratory medicine, Universitair Ziekenhuis Leuven, Louvain, Flemish, Belgium
Leukemia and Lymphoma (Impact Factor: 2.89). 10/2007; 48(9):1745-54. DOI: 10.1080/10428190701509772
Source: PubMed


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.

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    • "*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). "
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    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.
    Full-text · Article · Jun 2011 · British Journal of Haematology
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    • "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. "
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    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.
    Full-text · Article · Nov 2009 · British Journal of Haematology
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    ABSTRACT: 25% of all adult ALLs belong to the T-cell subtype; T-lymphoblastic lymphoma are a rare subgroup (< 2 %) of Non-Hodgkin's Lymphoma. Immunophenotyping has the most important prognostic impact for T-ALL. Thymic T-ALL has a cure rate of 60 to 70 % with chemotherapy alone, whereas for early and mature T-ALL the outcome is inferior (20-30 %), but can be improved substantially by a stem cell transplantation in CR1 (> 50%). T-LBLs receive the identical treatment, however without maintenance therapy. Mediastinal tumor, a common feature for T-ALL and T-LBL, can be life threatening, however in most cases respond well to chemotherapy. Mediastinal irradiation is necessary only in case of residual tumors in T-ALL, but recommended in all patients with T-LBL. New molecular markers, MRD-stratified treatment and new drugs, particularly T-lineage specific, will further improve the chance of cure of T-ALL and T-LBL.
    No preview · Article · Feb 2009 · Arzneimitteltherapie
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