Outcome of relapsed and refractory nodular lymphocyte‐predominant Hodgkin lymphoma: a North American analysis

Abstract

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare entity, with limited data on the outcome in the relapsed/refractory setting. We evaluated the outcome of all patients diagnosed between 04/1979 and 01/2019 with relapsed or progressive NLPHL after initial active therapy at two institutions, refractory disease being defined as lack of response to treatment and/or relapse within three months of treatment. NLPHL patients with histological evidence of transformation at time of first relapse or progression were excluded. In total, 69 patients with recurrent NLPHL were included in the study. After a median follow-up after initial diagnosis of 14 years (range, 0Á5-46 years), median progression-free survival after front-line treatment (PFS-1) was four years. Second-line therapy included chemotherapy in 28 (41%) patients, biological therapy (rituximab, lenalidomide or brentuximab vedotin) in 14 (20%), high-dose chemotherapy followed by autologous stem cell transplant in 14 (20%) and radiation therapy (RT) alone in 10 (15%). The five-year PFS after second-line therapy (PFS-2) was 68% [95% confidence interval (CI), 54-79%] but the fiveyear overall survival (OS) after second-line therapy (OS-2) remained excellent, at 94% (95% CI, 85-99%). Due to excellent outcome in case of recurrence, studies aimed at characterizing its biology to guide therapy deescalation are needed.

Management of children and adults with all stages of nodular lymphocyte predominant Hodgkin lymphoma — All StAGEs: A consensus‐based position paper from the Hodgkin lymphoma subgroup of the UK National Cancer Research Institute

“…Whilst the clinical outcome for most patients with NLPHL is very good, a small number will relapse; some with multiple, often late, relapses. Most patients with recurrent or poorly responsive disease respond to additional immunochemotherapy and/or radiotherapy 15,35,63,64 . Clinical relapse must be confirmed histologically to exclude diffuse large B‐cell lymphoma (DLBCL) transformation before initiating second‐line treatment.…”

“…Most patients with recurrent or poorly responsive disease respond to additional immunochemotherapy and/or radiotherapy. 15,35,63,64 Clinical relapse must be confirmed histologically to exclude diffuse large B-cell lymphoma (DLBCL) transformation before initiating second-line treatment. Treatment approaches at relapse have included single-agent monoclonal anti-CD20 rituximab or ofatumumab, 65 conventional chemotherapy such as ABVD, BEACOPP, DHAP (dexamethasone, cytarabine and cisplatin) with or without radiotherapy and high-dose chemotherapy followed by autologous stem cell rescue.…”

Phase II study of fixed‐duration single‐agent ibrutinib in relapsed nodular lymphocyte‐predominant Hodgkin lymphoma: A report from the German Hodgkin Study Group

“…This finding underscores that NLPHL mostly has an indolent course and a very good prognosis even in case of multiple relapses. 3,4,9,10 Taken together, the present study demonstrated that a significant proportion of individuals with relapsed NLPHL did not achieve long-term remission after treatment with fixed-duration single-agent ibrutinib. Thus, most patients with NLPHL recurrence are no candidates for treatment with this approach.…”

“…7,8 The male predominance among patients treated within the present study is consistent with earlier analyses. 3,4,7,8 Study treatment with ibrutinib was generally well tolerated.…”

Role of Rituximab Addition to First-line Chemotherapy Regimens in Nodular Lymphocyte-predominant Hodgkin Lymphoma: A Study by Fondazione Italiana Linfomi

“…This limit is described in other papers focused on this theme due to a lack of consensus on the role of autologous SCT or other regimens. 32 , 33 …”