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).

Objective:This study aimed to investigate the effect of tumor lysis syndrome (TLS) on the prognosis of children and adolescents with intermediate- or high-risk high-grade mature B-cell nonHodgkin lymphoma (HG B-NHL) .Methods:This study collected the clinical data and prognosis of 283 patients aged <18 years with newly diagnosed intermediate- or high-risk HG B-NHL treated at the Sun Yat-sen University Cancer Center from January 2010 to December 2022. The clinical characteristics, laboratory indicators during TLS, and prognosis of the patients were analyzed. The optimal cutoff values of laboratory indicators during TLS were identified using R studio according to event-free survival (EFS) .Results:Of the 283 patients enrolled, the median age was 7 (range: 1-18) years and the male-to-female ratio was 3.6∶1, 76 (26.9%) developed TLS, and 207 (73.1%) did not. Patients with TLS demonstrated higher proportions of the pathological subtype Burkitt lymphoma, high-risk stratification, age <12 years, and LDH of ≥1 000 IU/L compared with patients without TLS (all P<0.05). The 5-year EFS and overall survival (OS) rates of the entire group were (84.5±2.2) % and (88.2±2.0) %, respectively. The 5-year OS rate of patients with TLS was significantly lower than that of those without TLS [ (80.8±4.6) % vs (91.0±2.0) %, P=0.01]. Among patients with TLS, those with serum uric acid of ≤612.7 μmol/L ( n=36) exhibited lower 5-year EFS [ (67.8±8.1) % vs (87.5±5.2) %, P=0.04] and OS rates [ (69.9±8.1) % vs (90.0±4.7) %, P=0.04] compared with those with uric acid of >612.7 μmol/L ( n=40). Similarly, patients with serum phosphate of ≤1.89 mmol/L ( n=58) demonstrated lower 5-year EFS [ (71.6±6.0) % vs 100%, P=0.02] and OS rates [ (74.8±5.8) % vs 100%, P=0.03] compared with those with phosphate of >1.89 mmol/L ( n=18) . Conclusions:TLS is associated with poor prognosis in patients with HG B-NHL. Patients with lower serum uric acid and phosphate levels during TLS demonstrated worse prognoses, indicating their potential value in predicting prognosis and guiding stratified treatment.

Objective:This study aimed to investigate the effect of tumor lysis syndrome (TLS) on the prognosis of children and adolescents with intermediate- or high-risk high-grade mature B-cell nonHodgkin lymphoma (HG B-NHL) .Methods:This study collected the clinical data and prognosis of 283 patients aged <18 years with newly diagnosed intermediate- or high-risk HG B-NHL treated at the Sun Yat-sen University Cancer Center from January 2010 to December 2022. The clinical characteristics, laboratory indicators during TLS, and prognosis of the patients were analyzed. The optimal cutoff values of laboratory indicators during TLS were identified using R studio according to event-free survival (EFS) .Results:Of the 283 patients enrolled, the median age was 7 (range: 1-18) years and the male-to-female ratio was 3.6∶1, 76 (26.9%) developed TLS, and 207 (73.1%) did not. Patients with TLS demonstrated higher proportions of the pathological subtype Burkitt lymphoma, high-risk stratification, age <12 years, and LDH of ≥1 000 IU/L compared with patients without TLS (all P<0.05). The 5-year EFS and overall survival (OS) rates of the entire group were (84.5±2.2) % and (88.2±2.0) %, respectively. The 5-year OS rate of patients with TLS was significantly lower than that of those without TLS [ (80.8±4.6) % vs (91.0±2.0) %, P=0.01]. Among patients with TLS, those with serum uric acid of ≤612.7 μmol/L ( n=36) exhibited lower 5-year EFS [ (67.8±8.1) % vs (87.5±5.2) %, P=0.04] and OS rates [ (69.9±8.1) % vs (90.0±4.7) %, P=0.04] compared with those with uric acid of >612.7 μmol/L ( n=40). Similarly, patients with serum phosphate of ≤1.89 mmol/L ( n=58) demonstrated lower 5-year EFS [ (71.6±6.0) % vs 100%, P=0.02] and OS rates [ (74.8±5.8) % vs 100%, P=0.03] compared with those with phosphate of >1.89 mmol/L ( n=18) . Conclusions:TLS is associated with poor prognosis in patients with HG B-NHL. Patients with lower serum uric acid and phosphate levels during TLS demonstrated worse prognoses, indicating their potential value in predicting prognosis and guiding stratified treatment.