Abstract
Simple SummaryThe current literature on NLPHL therapy is scarce due to the disease rarity. Our study aims to focus on the management and treatment strategies of NLPHL based on risk stratification. This paper contributes to the current literature, based mainly on retrospective studies and on small cohort studies, confirming the benefit of Rituximab for patients with NLPHL, in particular for those with advanced disease. We hope that our results can be confirmed by larger cohort studies from the research community.Background: 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. Methods: 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). Results: 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 (p = 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; p = 0.03). Long-term non-hematological toxicities were observed more frequently in the historical cohort. Conclusion: 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.
Highlights
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare HL subtype since it represents approximately 5% of all cases [1]
Risk-adapted strategies for historical cohort and Rituximab-containing group are summarized in Table 2 and described in the Materials and Methods section
Taken together, the already available data and the results of our study indicate that front-line Rituximab induction treatment, when followed by two-year Rituximab maintenance, seems to have a low toxicity and a high efficacy in treating patients with early stage NLPHL without risk factors, replacing previous chemotherapy and/or
Summary
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare HL subtype since it represents approximately 5% of all cases [1]. Considering possible recurrences requiring further therapies in NLPHL, a less toxic treatment schedule would be desirable in order to reduce treatment-related mortality and morbidity From this consideration, the different phenotypes of expression on the cell of origin (“popcorn” cells or lymphocyte-predominant (LP) cells in NLPHL is a feature of CD20 and CD79a expression, unlike Hodgkin-Reed-Sternberg cHL cells), provides the rationale for the inclusion of the anti-CD20 antibody Rituximab in the treatment schemes of this disease, as a low toxic component able to replace CT and/or RT in order to reduce observed toxicity. 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. Results: 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 (p = 0.036) Four patients in the latter group showed insufficient response to therapy. Rituximab inclusion in advanced-stage therapeutic strategy seems to improve PFS compared to conventional chemo-radiotherapy
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