Pugliese Novella, Picardi Marco, Della Pepa Roberta, Giordano Claudia, Muriano Francesco, Leone Aldo, Delle Cave Giuseppe, D'Ambrosio Alessandro, Marafioti Violetta, Rascato Maria Gabriella, Russo Daniela, Mascolo Massimo, Pane Fabrizio
Department of Clinical Medicine and Surgery, Hematology Section, University of Naples "Federico II", Via Sergio Pansini, 5, 80131 Naples, Italy.
Pathology Unit, Department of Advanced Biomedical Sciences, University of Naples "Federico II", Via Sergio Pansini, 5, 80131 Naples, Italy.
Cancers (Basel). 2021 Apr 7;13(8):1760. doi: 10.3390/cancers13081760.
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare variant of HL that accounts for 5% of all HL cases. The expression of CD20 on neoplastic lymphocytes provides a suitable target for novel treatments based on Rituximab. Due to its rarity, consolidated and widely accepted treatment guidelines are still lacking for this disease.
Between 1 December 2007 and 28 February 2018, sixteen consecutive newly diagnosed adult patients with NLPHL received Rituximab (induction ± maintenance)-based therapy, according to the baseline risk of German Hodgkin Study Group prognostic score system. The treatment efficacy and safety of the Rituximab-group were compared to those of a historical cohort of 12 patients with NLPHL who received Doxorubicin, Bleomycin, Vinblastine, Dacarbazine (ABVD) chemotherapy followed by radiotherapy (RT), if needed, according to a similar baseline risk. The primary outcome was progression-free survival (PFS) and secondary outcomes were overall survival (OS) and side-effects (according to the Common Terminology Criteria for Adverse Events, v4.03).
After a 7-year follow-up (range, 1-11 years), PFS was 100% for patients treated with the Rituximab-containing regimen versus 66% for patients of the historical cohort ( = 0.036). Four patients in the latter group showed insufficient response to therapy. The PFS for early favorable and early unfavorable NLPHLs was similar between treatment groups, while a better PFS was recorded for advanced-stages treated with the Rituximab-containing regimen. The OS was similar for the two treatment groups. Short- and long-term side-effects were more frequently observed in the historical cohort. Grade ≥3 neutropenia was more frequent in the historical cohort compared with the Rituximab-group (58.3% vs. 18.7%, respectively; = 0.03). Long-term non-hematological toxicities were observed more frequently in the historical cohort.
Our results confirm the value of Rituximab in NLPHL therapy and show that Rituximab (single-agent) induction and maintenance in a limited-stage, or Rituximab with ABVD only in the presence of risk factors, give excellent results while sparing cytotoxic agent- and/or RT-related damage. Furthermore, Rituximab inclusion in advanced-stage therapeutic strategy seems to improve PFS compared to conventional chemo-radiotherapy.
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体，占所有霍奇金淋巴瘤病例的5%。肿瘤淋巴细胞上CD20的表达为基于利妥昔单抗的新型治疗提供了合适的靶点。由于其罕见性，该疾病仍缺乏统一且广泛接受的治疗指南。
在2007年12月1日至2018年2月28日期间，16例连续新诊断的成年NLPHL患者根据德国霍奇金研究组预后评分系统的基线风险接受了基于利妥昔单抗（诱导±维持）的治疗。将利妥昔单抗组的治疗疗效和安全性与一个历史队列进行比较，该队列中有12例NLPHL患者，他们根据相似的基线风险接受了多柔比星、博来霉素、长春碱、达卡巴嗪（ABVD）化疗，必要时接受放疗（RT）。主要结局是无进展生存期（PFS），次要结局是总生存期（OS）和副作用（根据不良事件通用术语标准，第4.03版）。
经过7年的随访（范围为1 - 11年），接受含利妥昔单抗方案治疗的患者PFS为100%，而历史队列患者的PFS为66%（P = 0.036）。后一组中有4例患者对治疗反应不足。治疗组之间早期有利和早期不利的NLPHL的PFS相似，而含利妥昔单抗方案治疗的晚期患者PFS更好。两个治疗组的OS相似。历史队列中更频繁观察到短期和长期副作用。历史队列中≥3级中性粒细胞减少症的发生率高于利妥昔单抗组（分别为58.3%和18.7%；P = 0.03）。历史队列中更频繁观察到长期非血液学毒性。
我们的结果证实了利妥昔单抗在NLPHL治疗中的价值，并表明在有限期使用利妥昔单抗（单药）诱导和维持治疗，或仅在存在风险因素时将利妥昔单抗与ABVD联合使用，可取得优异效果，同时避免细胞毒性药物和/或放疗相关的损害。此外，与传统放化疗相比，在晚期治疗策略中加入利妥昔单抗似乎可改善PFS。
