Transformation to Aggressive Lymphoma in Nodular Lymphocyte-Predominant Hodgkin's Lymphoma

Abstract

PURPOSE Prior observations suggest a higher risk of transformation of nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL) to aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL), than in classical Hodgkin's lymphoma. We evaluated the frequency of transformation in all patients diagnosed with NLPHL at the British Columbia Cancer Agency with long-term follow-up. PATIENTS AND METHODS The Lymphoid Cancer Database of the British Columbia Cancer Agency was searched to identify all patients diagnosed with NLPHL between 1965 and 2006. After pathologic review, 95 patients with NLPHL were confirmed. Results Patients with NLPHL had the following characteristics at diagnosis: median age of 37 years, 73% male, and 68% stage I or II disease. With a median follow-up time for living patients of 6.5 years (range, 2.5 to 33 years), 13 patients (14%) experienced transformation to aggressive lymphoma (median time to transformation, 8.1 years; range, 0.35 to 20.3 years). The actuarial risk of transformation to aggressive lymphoma was 7% and 30% at 10 and 20 years, respectively. Transformation was more likely in patients with initial splenic involvement (P = .006) at the time of diagnosis of NLPHL. The 10-year progression-free and overall survival rates in patients with transformed lymphoma were 52% and 62%, respectively. CONCLUSION The risk of transformation in patients with NLPHL to DLBCL is substantial and underappreciated. Because transformation can occur years after the primary diagnosis of NLPHL, long-term follow-up of these individuals is necessary to accurately estimate the risk of development of secondary DLBCL.

New developments in the pathology of malignant lymphoma: a review of the literature published from October 2009 to January 2010

“…Therefore, in this patient group, local treatment, even with a limited field of radiation, is the best choice. These data already indicate that the risk on large cell transformation is low, which was confirmed by Al-Mansour et al [29] who followed 95 NLPHL (all stages) and found 7% transformation at 10 years follow-up. Remarkably, this was 30% at 20 years.…”

Nodular lymphocyte predominant Hodgkin lymphoma: a Lymphoma Study Association retrospective study

“…26 The short follow-up of our patients does not allow for any conclusions on risk factors, especially the risk of histological transformation, as previously reported. [1][2][3][4] In conclusion, our study shows that the treatment of NLPHL at diagnosis can improve PFS, compared to watchful waiting. The policy of watchful waiting at diagnosis should be revisited, and discussed for carefully selected patients in order to maintain an excellent PFS and reduce the risk of late effects.…”

“…NLPHL usually follows an indolent course that can last for more than 10 years, late relapses may occur, and it can transform into aggressive non-Hodgkin B-cell lymphoma, with a reported rate of 12-23% in recent series. [1][2][3][4] Due to the rarity of the disease, clinical trials evaluating a riskadapted approach such as in cHL could not be conducted in NLPHL. Despite the issue of guidelines by the European Society for Medical Oncology 5 and the National Comprehensive Cancer Network, 6 questions such as the value of watchful waiting, the benefit of early treatment with a curative intent, and the optimal modalities of immunochemotherapy, are still being debated.…”

Nodular lymphocyte predominant Hodgkin lymphoma behaves as a distinct clinical entity with good outcome: evidence from 14-year follow-up in the West of Scotland Cancer Network

“…This risk of transformation, often long after the initial diagnosis, has been confirmed in previous studies. [5][6][7] Previous retrospective studies have examined the presentation of NLPHL [8][9][10] , however, due to its rarity and low incidence of events, there have been no published prospective randomised controlled trials in this area, outwith the context of NLPHL cases included in larger cHL trials. As a result, there is widespread variation worldwide in treatment of this condition.…”