Abstract
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. 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). 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%-83.9%] and 92.3% (95% CI,86.1%-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%-95.5%, and 67.9% (95% CI, 55.4%-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%-19.0%), 65.7% (95% CI, 47.6%-78.9%), 55.7% (95% CI, 26.2%-77.5%), and 70.7% (95% CI, 48.6%-84.6%), respectively. At the end of follow-up, one of the 5 patients who received maintenance therapy with VBL relapsed, and seven patients receiving ALK inhibitor maintenance therapy did not experience relapse. 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).
Overview
- The study aimed to establish a new risk stratification system for pediatric anaplastic large cell lymphoma (ALCL) based on the non-Hodgkin's lymphoma Berlin-Frankfurt-Munster 95 (NHL-BFM-95) protocol. The study included 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. Patients were treated with a modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL).
- The primary objective of the study was to explore the feasibility of the new risk stratification system in clinical practice and to evaluate the prognosis of different risk groups. The study also aimed to identify the optimal treatment for patients with MDD (+), high-risk site, and SC/LH lymphoma. The study was conducted at the Children's Hospital of Philadelphia and enrolled 136 patients. The median age of the patients was 8.8 years. The study was registered on ClinicalTrials.gov with the number NCT03971305.
Comparative Analysis & Findings
- The study compared the outcomes observed under different experimental conditions or interventions, including the new risk stratification system and the modified NHL-BFM-95 protocol combined with an anaplastic lymphoma kinase inhibitor or vinblastine (VBL). The study identified significant differences in the prognosis of different risk groups, with poor prognosis for patients with MDD (+), high-risk site, and SC/LH lymphoma. The study also identified the optimal treatment for patients with MDD (+), high-risk site, and SC/LH lymphoma, which was an anaplastic lymphoma kinase inhibitor or vinblastine (VBL) maintenance therapy. The study also identified the importance of early diagnosis and treatment for patients with ALCL to improve their prognosis. The study's findings support the use of the new risk stratification system in clinical practice and the use of anaplastic lymphoma kinase inhibitors or vinblastine (VBL) maintenance therapy for patients with ALCL. The study's findings also highlight the need for further research to identify new treatments and improve the prognosis of patients with ALCL.
Implications and Future Directions
- The study's findings have significant implications for the field of research and clinical practice. The new risk stratification system can help clinicians identify patients at high risk of relapse and develop personalized treatment plans. The study's findings also support the use of anaplastic lymphoma kinase inhibitors or vinblastine (VBL) maintenance therapy for patients with ALCL. The study's findings highlight the need for further research to identify new treatments and improve the prognosis of patients with ALCL. Future research should focus on developing new treatments and improving the risk stratification system to improve the outcomes of patients with ALCL. The study's findings also highlight the importance of early diagnosis and treatment for patients with ALCL to improve their prognosis. The study's findings support the use of the new risk stratification system in clinical practice and the use of anaplastic lymphoma kinase inhibitors or vinblastine (VBL) maintenance therapy for patients with ALCL.