Encouraging activity for R-CHOP in advanced stage nodular lymphocyte–predominant Hodgkin lymphoma

Abstract

Nodular lymphocyte Hodgkin lymphoma (NLPHL) is a rare disease for which the optimal therapy is unknown. We hypothesized that rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) could decrease rates of relapse and transformation. We retrospectively reviewed patients with NLPHL diagnosed between 1995 and 2015 confirmed by central pathologic review. Fifty-nine had sufficient treatment and follow-up data for analysis. We described progression-free survival (PFS), overall survival (OS), and histologic transformation according to treatment strategy and explored prognostic factors for PFS and OS. The median age at diagnosis was 41 years; 75% were male, and 61% had a typical growth pattern. Twenty-seven patients were treated with R-CHOP with an overall response rate of 100% (complete responses 89%). The median follow-up was 6.7 years, and the estimated 5- and 10-year PFS rates for patients treated with R-CHOP were 88.5% (95% confidence interval [CI], 68.4% to 96.1%) and 59.3 (95% CI, 25.3% to 89.1%), respectively. Excluding patients with histologic transformation at diagnosis, the 5-year cumulative incidence of histologic transformation was 2% (95% CI, 87% to 100%). No patient treated with R-CHOP experienced transformation. A high-risk score from the German Hodgkin Study Group was adversely prognostic for OS ( = .036), whereas male sex and splenic involvement were adversely prognostic for PFS ( = .006 and .002, respectively) but not OS. Our data support a potential role for R-CHOP in patients with NLPHL. Larger prospective trials are needed to define the optimal chemotherapy regimen.

Outcomes in intermediate‐risk pediatric lymphocyte‐predominant Hodgkin lymphoma: A report from the Children's Oncology Group

“…In a retrospective study of newly diagnosed adult patients with LPHL ( n = 6), R‐ABVD was efficacious with an estimated 6‐year EFS and OS of 75% and 100% respectively . Similarly, a retrospective analysis of 27 adult patients with advanced‐stage LPHL demonstrated that R‐CHOP was an effective treatment approach with an overall response rate of 100% and a CR rate of 89% …”

“…The National Comprehensive Care Network (NCCN) guidelines (https://www.nccn.org/professionals/physician_gls/pdf/hodgkins.pdf) suggest CVP, ABVD, or CHOP with or without rituximab as the most common regimens for advanced‐stage LPHL in adults. In adult studies of LPHL, rituximab has been used alone and in combination with other chemotherapy with promising results . Rituximab has been used as a single agent to treat 39 adult patients all of whom achieved a CR or PR after four weekly doses, with durable responses seen in patients treated with maintenance rituximab every 6 months for 2 years.…”

Modern principles in the management of nodular lymphocyte‐predominant Hodgkin lymphoma

“…Even for patients without a definitive pathological diagnosis of large cell transformation, the presence of high‐risk variant patterns (C–F), B symptoms, bulky disease and infradiaphragmatic disease (especially splenic involvement) are all indications for more aggressive frontline therapy. For these patients, we typically recommend chemotherapy with R‐CHOP (Fanale et al , ). In contrast, for advanced stage patients who are asymptomatic with low tumour burden and without unfavourable clinical or pathological features, an initial strategy of observation is reasonable and does not compromise OS (Borchmann et al , ).…”

“…A recent report from MDACC hypothesized that rituximab plus cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) could decrease rates of relapse and transformation (Fanale et al, 2017). This study included 59 patients with NLPHL diagnosed from 1995 to 2015 and confirmed by central pathological review.…”

Unfavorable presentation but comparable outcome: Presentation and outcome of children with nodular lymphocyte predominant Hodgkin lymphoma from India

“…Relapses in NLPHL have been found amenable to salvage chemotherapy, hence the trend toward decreasing the intensity of frontline therapy to minimize the risk of treatment toxicity and late effects . Rituximab has shown potential benefit in frontline as well as salvage treatment in adult NLPHL, however it is yet to be studied in pediatric NLPHL . Recent guidelines recommend complete resection alone in Stage IA NLPHL, with low dose chemotherapy/low dose radiation only in unresectable early stage disease, or in fully grown adolescent with localized disease .…”