Early T-cell precursor leukemia: a subtype of high risk childhood acute lymphoblastic leukemia.
Acute lymphoblastic leukemia includes T-cell acute lymphoblastic leukemia (T-ALL) and B-cell acute lymphoblastic leukemia (B-ALL). In children, T-ALL usually has a worse prognosis than B-ALL, although childhood T-ALL prognoses have improved remarkably. The varying outcomes among T-ALL cases suggest that an unrecognized biological heterogeneity may contribute to chemo-resistance. Deep exploration of T-lymphocyte development in recent years has found a subgroup of patients with a phenotype that resembles early T-cell precursor, which confers a much poorer prognosis than any other form of T-ALL. This novel subtype of T-ALL was called early T-cell precursor acute lymphoblastic leukemia (ETP-ALL). Flow cytometry data from T-ALL patients enrolled in Shanghai Children's Medical Center between July 2002 and October 2010 were assessed according to Dr. Campana's protocol. Among total 89 T-ALL cases, 74 cases had enough immunophenotype data available to differentiate between ETP (CD1a(-), CD8(-), CD5(dim), at least one marker of stem cell or myeloid lineage) and non-ETP. From these 74 subjects, 12 ETP-ALL cases (16.2%) were identified. The event-free survival (EFS) rate at 66.8 months was 11.1% ± 10.1% for ETP-ALL and 57.6% ± 5.6% for non-ETP-ALL (P = 0.003). The overall survival rates were 13.3% ± 11.0% for ETP-ALL and 64.7% ± 6.3% for non-ETP-ALL (P = 0.002). Our findings demonstrate that early T-cell precursor leukemia is a very high-risk subtype of acute lymphoblastic leukemia with poor prognosis.
Department of Hematology/Oncology, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China.
Meilin
M
Ma
Xiang
X
Wang
Jingyan
J
Tang
Huiliang
H
Xue
Jing
J
Chen
Ci
C
Pan
Hua
H
Jiang
Shuhong
S
Shen
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage* / Q000627:therapeutic use; D002648:Child; D002681:China; D019008:Drug Resistance, Neoplasm / Q000235:genetics* / Q000276:immunology; D006801:Humans; D053208:Kaplan-Meier Estimate; D008499:Medical Records / Q000706:statistics & numerical data; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000235:genetics* / Q000276:immunology; D011379:Prognosis; D012008:Recurrence; D012074:Remission Induction / Q000379:methods; D017211:Treatment Failure