Clin Pediatr Hematol Oncol.  2018 Oct;25(2):116-127. 10.15264/cpho.2018.25.2.116.

Outcome and Prognostic Factors in Pediatric Precursor T-Cell Acute Lymphoblastic Leukemia: A Single-Center Experience

• Rhee ES
• Kim H
• Kang SH
• Yoo JW
• Koh KN
• Im HJ
• Seo JJ

• KMID: 2435403
• DOI: http://doi.org/10.15264/cpho.2018.25.2.116

Abstract

BACKGROUND
Precursor T-cell acute lymphoblastic leukemia (T-ALL) has worse prognosis than B-cell ALL. We aimed to evaluate prognostic variables in pediatric T-ALL.
METHODS
Medical records of 36 T-ALL patients (27 males and 9 females; median age at diagnosis, 10.6 years) diagnosed and treated at Asan Medical Center from 2001 to 2017 were reviewed. Six patients (16.7%) had early T-cell precursor ALL (ETP-ALL). Most patients received the Children's Cancer Group-1882 (CCG1882) or Korean multicenter high risk ALL (ALL0601) protocols and prophylactic cranial irradiation. Clinical features at presentation, response to therapy, and treatment outcomes were analyzed.
RESULTS
The six patients with ETP-ALL and 17 of 30 with non-ETP-ALL received CCG1882 or ALL0601 chemotherapy. Three patients, including two with ETP-ALL, did not achieve complete remission after induction. Rapid early response during induction was achieved by 26 patients. Five year overall survival (OS) and event free survival (EFS) rates were 71.4% and 70.2%, respectively. ETP-ALL and slow early response during induction were significant adverse prognostic factors, while hyperleukocytosis at diagnosis was not. CCG1882/ALL0601 chemotherapy resulted in superior survival (OS: 78.9%, EFS: 73.3%) compared with CCG1901 chemotherapy (OS: 64.3%, EFS: 64.3%), and patients undergoing prophylactic cranial irradiation had superior EFS to non-radiated patients.
CONCLUSION
A high risk ALL protocol with intensified post-remission therapy, including prophylactic cranial irradiation, conferred T-ALL survival outcomes comparable with those of Western studies. Further treatment intensification should be considered for patients with ETP-ALL and slow induction responders. Additionally, CNS-directed treatment intensification, without prophylactic cranial irradiation, is needed.

Keyword

Acute lymphoblastic leukemia; Pediatric; Precursor T-cell lymphoblastic leukemia-lymphoma; Survival

MeSH Terms

B-Lymphocytes
Chungcheongnam-do
Cranial Irradiation
Diagnosis
Disease-Free Survival
Drug Therapy
Female
Humans
Male
Medical Records
Precursor Cell Lymphoblastic Leukemia-Lymphoma*
Precursor Cells, T-Lymphoid
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
Prognosis
T-Lymphocytes*