Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2016 Apr 14;127(15):1863-9.
doi: 10.1182/blood-2015-08-661702. Epub 2016 Jan 8.

Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype

Nitin Jain  1 , Audrey V Lamb  2 , Susan O'Brien  3 , Farhad Ravandi  1 , Marina Konopleva  1 , Elias Jabbour  1 , Zhuang Zuo  2 , Jeffrey Jorgensen  2 , Pei Lin  2 , Sherry Pierce  1 , Deborah Thomas  1 , Michael Rytting  1 , Gautam Borthakur  1 , Tapan Kadia  1 , Jorge Cortes  1 , Hagop M Kantarjian  1 , Joseph D Khoury  2
Affiliations

Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype

Nitin Jain et al. Blood. .

Abstract

Early T-cell precursor (ETP) acute lymphoblastic leukemia/lymphoma (ALL/LBL) is a recently recognized high-risk T lymphoblastic leukemia/lymphoma (T-ALL/LBL) subgroup. The optimal therapeutic approaches to adult patients with ETP-ALL/LBL are poorly characterized. In this study, we compared the outcomes of adults with ETP-ALL/LBL who received treatment on frontline regimens with those of patients with other T-ALL/LBL immunophenotypic subtypes. Patients with newly diagnosed T-ALL/LBL who received frontline chemotherapy between the years 2000 and 2014 at The University of Texas MD Anderson Cancer Center were identified and immunophenotypically categorized into early, thymic, and mature per the World Health Organization (WHO) classification using CD1a and surface CD3 status. Patients with ETP-ALL/LBL were identified on the basis of the following immunophenotypes: CD1a(-), CD8(-), CD5(-)(dim), and positivity for 1 or more stem cell or myeloid antigens. A total of 111 patients with T-ALL/LBL (68% T-ALL; 32% T-LBL) with adequate immunophenotype data were identified. The median age was 30 years (range, 13-79). There was no difference in the outcomes of patients based on the WHO subtypes. Nineteen patients (17%) had ETP-ALL/LBL. The complete remission rate /complete remission with incomplete platelet recovery rate in patients with ETP-ALL/LBL was significantly lower than that of non-ETP-ALL/LBL patients (73% vs 91%;P= .03). The median overall survival for patients with ETP-ALL/LBL was 20 months vs not reached for the non-ETP-ALL/LBL patients (P= .008). ETP-ALL/LBL represents a high-risk disease subtype of adult ALL. Novel treatment strategies are needed to improve treatment outcomes in this T-ALL/LBL subset.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Survival for the entire study population. (A) Event-free survival and (B) overall survival for the entire study population of patients with T-ALL/LBL (n = 111).
Figure 2
Figure 2
Survival for the entire study population categorized by immunophenotype per WHO classification. (A) Event-free survival and (B) overall survival of patients with T-ALL/LBL (n = 111) categorized as early, thymic, and mature per WHO classification. NR, not reached.
Figure 3
Figure 3
Survival for the entire study population categorized as ETP vs non-ETP. (A) Event-free survival and (B) overall survival of patients with ETP ALL (n = 19) compared with non-ETP ALL (n = 92). NR, not reached.
Figure 4
Figure 4
Survival for the entire study population categorized by ETP and WHO classification. Overall survival of patients with the WHO early classification subcategorized as ETP vs non-ETP, WHO thymic, and WHO mature (n = 111). NR, not reached.
Figure 5
Figure 5
Survival for the patients presenting as ALL categorized as ETP vs non-ETP. Overall survival of ETP subtype (presenting as ALL, n = 15) vs non-ETP subtype (presenting as ALL, n = 61).
See this image and copyright information in PMC

References

    1. Bene MC, Castoldi G, Knapp W, et al. European Group for the Immunological Characterization of Leukemias (EGIL) Proposals for the immunological classification of acute leukemias. Leukemia. 1995;9(10):1783–1786. - PubMed
    1. Ludwig WD, Raghavachar A, Thiel E. Immunophenotypic classification of acute lymphoblastic leukaemia. Baillieres Clin Haematol. 1994;7(2):235–262. - PubMed
    1. Ludwig WD, Reiter A, Löffler H, et al. Immunophenotypic features of childhood and adult acute lymphoblastic leukemia (ALL): experience of the German Multicentre Trials ALL-BFM and GMALL. Leuk Lymphoma. 1994;13(Suppl 1):71–76. - PubMed
    1. Borowitz MJ, Chan JKC. T Lymphoblastic Leukemia/Lymphoma. In: Swerdlow SH, Campo E, Harris NL, et al., editors. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, France: IARC; 2008. pp. 176–178.
    1. Coustan-Smith E, Mullighan CG, Onciu M, et al. Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia. Lancet Oncol. 2009;10(2):147–156. - PMC - PubMed

MeSH terms