Expression of CD56 defines a distinct subgroup in childhood T-ALL with inferior outcome

• Verfasst von: Fuhrmann, Stephan [VerfasserIn]
• Verfasst von: Kunz, Joachim [VerfasserIn]
• Verfasst von: Kulozik, Andreas [VerfasserIn]
• Titel: Expression of CD56 defines a distinct subgroup in childhood T-ALL with inferior outcome
• Titelzusatz: Results of the ALL-BFM 2000 trial
• Verf.angabe: Stephan Fuhrmann, Richard Schabath, Anja Möricke, Martin Zimmermann, Joachim B. Kunz, Andreas E. Kulozik, Wolf-Dieter Ludwig, Martin Schrappe, Leonid Karawajew and Richard Ratei
• E-Jahr: 2018
• Jahr: 7 June 2018
• Umfang: 8 S.
• Fussnoten: Gesehen am 01.08.2019
• Titel Quelle: Enthalten in: British journal of haematology
• Ort Quelle: Oxford [u.a.] : Wiley-Blackwell, 1955
• Jahr Quelle: 2018
• Band/Heft Quelle: 183(2018), 1, Seite 96-103
• ISSN Quelle: 1365-2141
• DOI: doi:10.1111/bjh.15503
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: ALL
• Sach-SW: childhood leukaemia
• Sach-SW: immunophenotyping
• K10plus-PPN: 1670428168

Abstract

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.