Journal Article

Prognostic value and outcome for acute lymphocytic leukemia in children with MLL rearrangement: a case-control study

Huang, Ke, Xu, Lu-hong, Liao, Xiong-yu, Weng, Wen-jun, Li, Yang, Fang, Jian-pei, Zhou, Dun-hua, Xu, Hong-gui, Qiu, Kun-yin

2022

Overview

Purpose To evaluate the prognostic factors and outcome for acute lymphoblastic leukemia (ALL) in children with MLL rearrangement (MLL-r). Methods A total of 124 pediatric patients who were diagnosed with ALL were classified into two groups based on the MLL-r status by using a retrospective case-control study method from June 2008 to June 2020. Results The prevalence of MLL-r positive in the whole cohort was 4.9%. The complete remission (CR) rate on Day 33 in the MLL-r positive group was not statistically different from the negative group (96.8% vs 97.8%, P  = 0.736). Multivariate analysis showed that T-cell, white blood cell counts (WBC) ≥ 50 × 10 9 /L, MLL-AF4, and D15 minimal residual disease (MRD) positive were independent risk factors affecting the prognosis of MLL-r positive children. Stem cell transplantation (SCT) was a favorable independent prognostic factor affecting event-free survival (EFS) in MLL-r positive patients ( P  = 0.027), and there was a trend toward an independent prognostic effect on overall survival (OS) ( P  = 0.065). The 10-year predicted EFS for patients with MLL-AF4, MLL-PTD, MLL-ENL, other MLL partner genes, and MLL-r negative cases were 46.67 ± 28.61%, 85.71 ± 22.37%, 75 ± 32.41%, 75 ± 32.41%, and 77.33 ± 10.81%, respectively ( P  = 0.048). The 10-year predicted OS were 46.67 ± 28.61%, 85.71 ± 22.37%, 75 ± 32.41%, 75 ± 32.41%, and 85.2 ± 9.77%, respectively ( P  = 0.049). The 124 patients with ALL were followed up and eventually 5 (4%) cases relapsed, with a median relapse time of 3.9 years. Conclusion Patients with MLL-r positive ALL have moderate remission rates, but are prone to relapse with low overall survival. The outcome of MLL-r positive ALL was closely related to the partner genes, and clinical attention should be paid to screening for MLL partner genes and combining them with other prognostic factors for accurate risk stratification.

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
• Subject: Acute lymphoblastic leukemia / Acute lymphocytic leukemia / Age / Biomedical and Life Sciences / Biomedicine / Bone marrow / Cancer / Cancer Research / Care and treatment / Chemotherapy / Children / Development and progression / Evaluation / Genes / Health Promotion and Disease Prevention / Leukemia / Leukemia in children / Lymphatic leukemia / Lymphoblastic leukemia in children / Lymphocytes T / Lymphocytic leukemia in children / Medical prognosis / Medicine/Public Health / Minimal residual disease / MLL / Multivariate analysis / Nervous system / Oncology / Oncology, Experimental / Outcome / Patients / Pediatrics / Prognosis / Remission / Remission (Medicine) / Risk factors / Stem cell transplantation / Surgical Oncology