Data Sheet 1_Exploring the complexity, treatment challenges, and outcomes in pediatric nodular lymphocyte predominant Hodgkin lymphoma: a perspective from a low–middle-income country.docx

BackgroundIn children, nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is an uncommon subtype of Hodgkin lymphoma. Given the lack of data on the best chemotherapy regimen, there is a knowledge gap in best management.
MethodsThis retrospective study included all pediatric patients with NLPHL diagnosed and treated at the Children’s Cancer Hospital, Egypt (2007–2018). We analyzed the clinical characteristics, and treatment outcome according to disease stage (early and advanced), treatment strategy (doxorubicin, bleomycin, vinblastine, and dacarbazine [ABVD] or rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone [R-CHOP] and explored the prognostic factors for progression-free survival.
ResultsThe median age at diagnosis was 12 years; 40 (68%) patients had early-stage disease, and 19 (32%) had advanced-stage disease. The median follow-up was 70 months; the 5-year EFS and OS were 75% and 97%, respectively. The 5-year EFS was 78% for early-stage disease and 60% for advanced-stage disease; the 5-year OS was 100% for early-stage disease and 88% for advanced-stage disease. The patients who underwent R-CHOP had a better 3-year EFS (100%) compared with those who underwent ABVD (65%) regimen (P = 0.01). Seventeen (25%) patients relapsed: 9/40 (22%) had early-stage disease, and 8/19 (42%) had advanced-stage disease. Splenic involvement, mediastinal disease, extranodal disease, and slow early response were independent predictors of relapse risk.
ConclusionPediatric patients with early-stage NLPHL have an excellent prognosis, with a 5-year OS of 100%. However, those with advanced stages had a high relapse rate. R-CHOP was associated with a better response and relapse-free rate than ABVD.

背景 在儿童群体中，结节性淋巴细胞为主型霍奇金淋巴瘤（nodular lymphocyte-predominant Hodgkin lymphoma，NLPHL）是霍奇金淋巴瘤的一种罕见亚型。由于目前缺乏针对最优化疗方案的相关数据，其最佳临床管理策略仍存在认知空白。
方法 本回顾性研究纳入了2007年至2018年间，埃及儿童癌症医院确诊并接受治疗的所有NLPHL儿科患者。研究人员根据疾病分期（早期与晚期）、治疗方案（阿霉素、博来霉素、长春花碱与达卡巴嗪[ABVD]，或利妥昔单抗、环磷酰胺、阿霉素、长春新碱与泼尼松[R-CHOP]）分析了患者的临床特征与治疗结局，并探索了无进展生存期的预后影响因素。
结果 患者确诊时的中位年龄为12岁；其中40例（68%）为早期疾病，19例（32%）为晚期疾病。中位随访时间为70个月，5年无事件生存期（Event-Free Survival，EFS）与总生存期（Overall Survival，OS）分别为75%与97%。早期疾病患者的5年EFS为78%，晚期患者为60%；早期患者的5年OS为100%，晚期患者为88%。接受R-CHOP方案治疗的患者，其3年EFS（100%）优于接受ABVD方案治疗的患者（65%）（P = 0.01）。共有17例（25%）患者复发：其中早期疾病患者9/40（22%），晚期患者8/19（42%）。脾脏受累、纵隔病变、结外病变以及早期治疗反应缓慢均为复发风险的独立预测因素。
结论 早期NLPHL儿科患者预后极佳，5年OS可达100%。但晚期患者的复发率较高。与ABVD方案相比，R-CHOP方案可带来更优的治疗反应与无复发生存率。