Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
Department of Pediatric Hematology/Oncology, King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
BMC Cancer. 2021 Apr 1;21(1):351. doi: 10.1186/s12885-021-08074-2.
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is an uncommon variant of Hodgkin lymphoma. There is limited data on treatment, management of refractory and relapsed disease, and long-term outcome. Many registries or country-wide data reports are unable to provide detailed primary and subsequent management. We are reporting our observation on patient's characteristics, management, and outcome.
This single-institution retrospective cohort analysis includes NLPHL patients seen from 1998 to July 2019. We used Fisher's exact test, chi-square, and Kaplan-Meier (KM) method for various analyses.
Two hundred patients were identified, (6.34% of all the HL). Male:female was 3:1. The median age at diagnosis was 22 years (4-79 years). Stage I-II in 145 (72.5%) cases. One hundred patients (50%) received chemotherapy, 68 (34%) chemotherapy + radiation therapy (RT); 87% of all chemotherapy was ABVD (adriamycin, bleomycin, vinblastine, dacarbazine). Thirteen patients (6.5%) received RT alone and 16 (8%) had surgery alone. Complete response in 82%, partial response in 5.5% and progressive disease in 10.5%. The median follow is 60 months (5-246). Median 5 and 10 years overall survival (OS) is 94.8 and 92.4% (stages I-II, 97.7 and 97.7%, stage III-IV, 94.8 and 92.4%). Median event-free survival (EFS) is 62.3 and 54% respectively (stage I-II, 72 and 64%, stage III-IV, 36.4 and 18.2%). Stage I-II vs III-IV OS (p = < 0.001) and EFS (p = < 0.001) were significant. For stage I-II, 5 year EFS of chemotherapy + RT (83.3%) was superior to chemotherapy alone (60%, p = 0.008). Five year EFS for early favorable (80%), early unfavorable (60%), and advanced (36.4%) was significant (p = < 0.001). Eleven patients (5.5%) had high-grade transformation. Twenty-nine patients underwent HDC auto-SCT, all are alive (28 in remission). 25% of patients had pathologically proved nodal hyperplasia at some point in time.
OS of NLPHL is excellent and independent of treatment type. EFS is better for chemotherapy + RT than chemotherapy alone. Stem cell transplant in refractory / multiple relapses resulted in excellent disease control. There is a need to identify optimal treatment strategies accordingly to the risk stratification.
结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）是霍奇金淋巴瘤的一种罕见变异型。关于其治疗、难治性和复发性疾病的管理以及长期预后的数据有限。许多登记处或全国性数据报告无法提供详细的初始和后续管理信息。我们在此报告我们对患者特征、治疗和预后的观察结果。
本单中心回顾性队列分析纳入了 1998 年至 2019 年 7 月期间就诊的 NLPHL 患者。我们使用 Fisher 精确检验、卡方检验和 Kaplan-Meier（KM）方法进行了各种分析。
共确定了 200 例患者（所有 HL 的 6.34%）。男女比例为 3:1。中位诊断年龄为 22 岁（4-79 岁）。Ⅰ-Ⅱ期 145 例（72.5%）。100 例患者（50%）接受化疗，68 例（34%）接受化疗+放疗（RT）；所有化疗中 87%为 ABVD（阿霉素、博来霉素、长春新碱、达卡巴嗪）。13 例（6.5%）仅接受 RT，16 例（8%）仅接受手术。82%患者达到完全缓解，5.5%患者部分缓解，10.5%患者疾病进展。中位随访时间为 60 个月（5-246 个月）。5 年和 10 年总生存率（OS）分别为 94.8%和 92.4%（Ⅰ-Ⅱ期为 97.7%和 97.7%，Ⅲ-Ⅳ期为 94.8%和 92.4%）。中位无事件生存率（EFS）分别为 62.3%和 54%（Ⅰ-Ⅱ期为 72%和 64%，Ⅲ-Ⅳ期为 36.4%和 18.2%）。Ⅰ-Ⅱ期与Ⅲ-Ⅳ期 OS（p<0.001）和 EFS（p<0.001）均有显著差异。对于Ⅰ-Ⅱ期患者，化疗+RT 的 5 年 EFS（83.3%）优于单纯化疗（60%，p=0.008）。早期预后良好（80%）、不良（60%）和晚期（36.4%）患者的 5 年 EFS 差异有统计学意义（p<0.001）。11 例（5.5%）患者发生高级别转化。29 例患者接受了高剂量化疗自体造血干细胞移植（HDC auto-SCT），均存活（28 例缓解）。25%的患者在某个时间点出现病理性淋巴结增生。
NLPHL 的 OS 非常好，与治疗类型无关。化疗+RT 的 EFS 优于单纯化疗。在难治性/多次复发时进行干细胞移植可获得良好的疾病控制。需要根据风险分层确定最佳治疗策略。
