title: Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype.
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.
authors.affiliation: Department of Leukemia and.
authors.forename: Nitin
authors.initials: N
authors.lastname: Jain
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX; and.
authors.forename: Audrey V
authors.initials: AV
authors.lastname: Lamb
authors.affiliation: Chao Family Comprehensive Cancer Center, University of California Irvine Medical Center, Orange, CA.
authors.forename: Susan
authors.initials: S
authors.lastname: O'Brien
authors.affiliation: Department of Leukemia and.
authors.forename: Farhad
authors.initials: F
authors.lastname: Ravandi
authors.affiliation: Department of Leukemia and.
authors.forename: Marina
authors.initials: M
authors.lastname: Konopleva
authors.affiliation: Department of Leukemia and.
authors.forename: Elias
authors.initials: E
authors.lastname: Jabbour
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX; and.
authors.forename: Zhuang
authors.initials: Z
authors.lastname: Zuo
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX; and.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Jorgensen
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX; and.
authors.forename: Pei
authors.initials: P
authors.lastname: Lin
authors.affiliation: Department of Leukemia and.
authors.forename: Sherry
authors.initials: S
authors.lastname: Pierce
authors.affiliation: Department of Leukemia and.
authors.forename: Deborah
authors.initials: D
authors.lastname: Thomas
authors.affiliation: Department of Leukemia and.
authors.forename: Michael
authors.initials: M
authors.lastname: Rytting
authors.affiliation: Department of Leukemia and.
authors.forename: Gautam
authors.initials: G
authors.lastname: Borthakur
authors.affiliation: Department of Leukemia and.
authors.forename: Tapan
authors.initials: T
authors.lastname: Kadia
authors.affiliation: Department of Leukemia and.
authors.forename: Jorge
authors.initials: J
authors.lastname: Cortes
authors.affiliation: Department of Leukemia and.
authors.forename: Hagop M
authors.initials: HM
authors.lastname: Kantarjian
authors.affiliation: Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX; and.
authors.forename: Joseph D
authors.initials: JD
authors.lastname: Khoury
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018949:Antigens, CD1 / Q000235:genetics; D017252:CD3 Complex / Q000235:genetics; D002454:Cell Differentiation; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D054504:Precursor Cells, T-Lymphoid / Q000276:immunology; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy*; D011379:Prognosis; D016016:Proportional Hazards Models; D012008:Recurrence; D012074:Remission Induction; D012307:Risk Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D018949:Antigens, CD1; C419393:CD1a antigen; D017252:CD3 Complex