Expression of CD56 defines a distinct subgroup in childhood T-ALL with inferior outcome: Results of the ALL-BFM 2000 trial
This study reports the prognostic impact of the expression of the natural killer cell marker CD56 in a large series of risk-adapted paediatric patients with T cell acute lymphoblastic leukaemia (T-ALL; n = 493) treated within the ALL-Berlin-Frankfurt-Münster (BFM) 2000 protocol. The immunophenotype...
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| Hauptverfasser: | , , |
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| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
7 June 2018
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| In: |
British journal of haematology
Year: 2018, Jahrgang: 183, Heft: 1, Pages: 96-103 |
| ISSN: | 1365-2141 |
| DOI: | 10.1111/bjh.15503 |
| Online-Zugang: | Verlag, Volltext: https://doi.org/10.1111/bjh.15503 Verlag, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1111/bjh.15503 
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| Verfasserangaben: | Stephan Fuhrmann, Richard Schabath, Anja Möricke, Martin Zimmermann, Joachim B. Kunz, Andreas E. Kulozik, Wolf-Dieter Ludwig, Martin Schrappe, Leonid Karawajew and Richard Ratei |
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| 520 | |a This study reports the prognostic impact of the expression of the natural killer cell marker CD56 in a large series of risk-adapted paediatric patients with T cell acute lymphoblastic leukaemia (T-ALL; n = 493) treated within the ALL-Berlin-Frankfurt-Münster (BFM) 2000 protocol. The immunophenotype was analysed centrally at diagnosis using flow cytometry and correlated with clinical parameters and outcome. CD56 expression was detected in 7·1% and early T-cell precursor (ETP) phenotype in 6·7% of all T-ALL patients. The percentage of ETP in the CD56+ T-ALL cohort was 4-fold higher than in the whole cohort. CD56+ T-ALL frequently expressed the progenitor marker CD34 and myeloid antigens CD13 and CD33. The 5-year event-free survival (EFS) rates for the European Group for the Immunological classification of Leukaemias/World Health Organization subgroups and the ETP phenotype were not statistically different. By contrast, patients with CD56 expression had a significantly reduced EFS (60 ± 8%) and overall survival (60 ± 8%) at 5 years, with a hazard ratio of 2·46 (P = 0·002) and 2·99 (P < 0·001), respectively. Moreover, CD56 expression in combination with the minimal residual disease (MRD)-based high risk assignment defined a population with a ‘very-high’ risk probability of relapse in the ALL-BFM 2000 trial. The CD56 marker has the potential to augment MRD-based risk stratification and may serve as a molecular target for antibody-based treatment strategies in childhood T-ALL. | ||
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