Cancer Res Treat.  2024 Oct;56(4):1252-1261. 10.4143/crt.2024.104.

Stratified Treatment in Pediatric Anaplastic Large Cell Lymphoma: Result of a Prospective Open-Label Multiple-Institution Study

• Chen T
• Zeng C
• Wang J
• Sun F
• Huang J
• Zhu J
• Lu S
• Liao N
• Zhang X
• Chen Z
• Yuan X
• Yang Z
• Guo H
• Yang L
• Wen C
• Zhang W
• Li Y
• Luo X
• Wu Z
• Yang L
• Liu R
• Zheng M
• He X
• Sun X
• Zhen Z

• KMID: 2560258
• DOI: http://doi.org/10.4143/crt.2024.104

Abstract

Purpose
The risk stratification of pediatric anaplastic large cell lymphoma (ALCL) has not been standardized. In this study, new risk factors were included to establish a new risk stratification system for ALCL, and its feasibility in clinical practice was explored.
Materials and Methods
On the basis of the non-Hodgkin’s lymphoma Berlin–Frankfurt–Munster 95 (NHL-BFM-95) protocol, patients with minimal disseminated disease (MDD), high-risk tumor site (multiple bone, skin, liver, and lung involvement), and small cell/lymphohistiocytic (SC/LH) pathological subtype were enrolled in risk stratification. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL).
Results
A total of 136 patients were enrolled in this study. The median age was 8.8 years. The 3-year event-free survival (EFS) and overall survival of the entire cohort were 77.7% (95% confidence interval [CI], 69.0% to 83.9%) and 92.3% (95% CI, 86.1% to 95.8%), respectively. The 3-year EFS rates of low-risk group (R1), intermediate-risk group (R2), and high-risk group (R3) patients were 100%, 89.5% (95% CI, 76.5% to 95.5%), and 67.9% (95% CI, 55.4% to 77.6%), respectively. The prognosis of patients with MDD (+), stage IV cancer, SC/LH lymphoma, and high-risk sites was poor, and the 3-year EFS rates were 45.3% (95% CI, 68.6% to 19.0%), 65.7% (95% CI, 47.6% to 78.9%), 55.7% (95% CI, 26.2% to 77.5%), and 70.7% (95% CI, 48.6% to 84.6%), respectively. At the end of follow-up, one of the five patients who received maintenance therapy with VBL relapsed, and seven patients receiving anaplastic lymphoma kinase inhibitor maintenance therapy did not experience relapse.
Conclusion
This study has confirmed the poor prognostic of MDD (+), high-risk site and SC/LH, but patients with SC/LH lymphoma and MDD (+) at diagnosis still need to receive better treatment (ClinicalTrials.gov number, NCT03971305).

Keyword

Pediatric; Anaplastic large cell lymphoma; Stage; Stratified treatment; Minimal disseminated disease; Small cell/lymphohistiocytic

Figure

• 

  Fig. 1. Survival rates of 136 patients with anaplastic large cell lymphoma. (A) Kaplan-Meier estimate of event-free survival for the whole cohort. (B) Kaplan-Meier estimate of overall survival for the whole cohort. (C) Kaplan-Meier estimate of event-free survival stratified by stage. (D) Kaplan-Meier estimate of overall survival stratified by stage. (E) Kaplan-Meier estimate of event-free survival stratified by risk stratification. (F) Kaplan-Meier estimate of overall survival stratified by risk stratification.

• 

  Fig. 2. Survival rates of 136 patients with anaplastic large cell lymphoma stratified by new risk factors. (A) Kaplan-Meier estimate of event-free survival stratified by pathological type. (B) Kaplan-Meier estimate of overall survival according to pathological type. (C) Kaplan-Meier estimate of event-free survival stratified by high-risk site of multiple bone/lung/liver/skin involvement. (D) Kaplan-Meier estimate of overall survival stratified by high-risk site. (E) Kaplan-Meier estimate of event-free survival stratified by minimal disseminated disease (MDD). (F) Kaplan-Meier estimate of overall survival stratified by MDD. LH, lymphohistiocytic; SC, small cell.

Reference

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