Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL) is an indolent lymphoma with excellent overall survival (OS) and distinct clinicopathological characteristics reclassified as separate to Classical Hodgkin Lymphoma (CHL). Debate is ongoing as to optimum management. Guidelines differ in approach, particularly around watch and wait (W+W); ranging from selected use in all stages (B J Haem 2015), to advanced disease (NCCN 2015) to not at all (ESMO 2014). Few studies have assessed W+W, with discordant results. All were retrospective and include cases diagnosed before reclassification.
We conducted a retrospective analysis to investigate outcomes of adults with NLPHL diagnosed and managed at St Bartholomew’s hospital from 1994 (reclassification) to 2014. This represents the largest single centre study reporting data obtained since reclassification. Clinical characteristics, treatment, OS, progression free survival (PFS) and Disease Free Survival (DFS) were collected. Forty-four patients were included. 82% had limited (stage IA or IIA) disease, 73% were male. Median age was 38. Patients of Black (25%) and Asian (39%, mainly Bangladeshi) origins were overrepresented compared to CHL cases (7% Black, 13% Asian) or any haematological malignancy (8% Black, 10% Asian) managed during this period. Initial management consisted of W+W +/- excision (32%), Radiotherapy +/- excision (29%) and Chemotherapy (mostly ABVD; in 2 cases R-ABVD) or Combined Modality (39%). W+W was used in 36% of patients with limited and 25% with advanced disease. Median follow-up was 63 months. 10-year OS and PFS estimates were 100% and 60% respectively. Two transformation events were observed, none in the W+W group. One patient died from an unrelated cause. 80% of W+W patients remained untreated at median follow up of 29 (5-198) months. No significant differences were seen in PFS, DFS or OS between W+W and immediate treatment.
Our data is consistent with an increased incidence seen in African-Americans. Raised incidence in Asian groups has not previously been reported. The differing racial demographic profile to CHL supports NLPHL’s separate classification. The relatively good PFS and excellent OS support consideration of W+W as a management option, given that most patients remained treatment free after long follow up. Our data support the need for a clinical trial to assess W+W versus immediate treatment. Given the rarity of NLPHL this would require an international collaborative study.