Advanced-stage nodular lymphocyte predominant Hodgkin lymphoma compared with classical Hodgkin lymphoma: a matched pair outcome analysis

Abstract

Key Points• TTP is inferior in patients with advanced-stage NLPHL compared with CHL.• Spleen involvement is associated with an increased risk of secondary aggressive lymphoma in patients treated with ABVD-like chemotherapy.Due to disease rarity, there is limited information regarding the optimal therapy and outcome for patients with advanced-stage nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). Forty-two patients with NLPHL by the Revised European-American Lymphoma/World Health Organization classification with advanced-stage disease were identified and paired 1:2 with a matched control with classical Hodgkin lymphoma (CHL) matched by age, gender, stage, decade of diagnosis, and treatment received. The median follow-up was 11.3 years (range, 1.9 to 35.5 years) for NLPHL patients and 10.7 years (range, 1.6 to 26.3 years) for CHL patients. The majority received doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD)-like chemotherapy. Although the 10-year overall survival (OS) (P 5 .579) and HL freedom from treatment failure (HL-FFTF) were similar between NLPHL and CHL patients (75% vs 73%; P 5 .610), the time to progression (TTP), which also includes the development of secondary aggressive lymphoma, was inferior in NLPHL (10-year, 63% vs 73%; P 5 .040). Splenic involvement was associated with an inferior 10-year TTP in patients treated with ABVD (48% vs 71%; P 5 .049) and an increased cumulative incidence of secondary aggressive lymphoma (P 5 .014) providing a rationale for further evaluation of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with rituximab in NLPHL. (Blood. 2014;123(23):3567-3573)

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

“…Chemotherapy alone has not been commonly used in adults with early stage NLPHL, 18 and is the main option for patients with advanced stage. 7,8,19 In this study, patients with stages III-IV who were initially treated received mainly chemotherapy or immuno-chemotherapy (71.1%), a management which is consistent with the international guidelines. 5,6 An unsolved question is the choice of the chemotherapy regimen, in particular between a treatment similar to cHL with ABVD, or a treatment with CHOP, as in indolent NHL.…”

How I treat nodular lymphocyte predominant Hodgkin lymphoma

“…The BCCA reported a matched control outcome analysis of patients with NLPHL (n 5 42) or cHL (n 5 82) treated with ABVD or ABVD-like chemotherapy. 49 At 15 years, there was a trend toward superior PFS for cHL compared with LPHL (72% vs 44%; P 5 .096). The largest series of patients with advanced disease is from the GHSG, comparing outcomes of 83 patients with NLPHL with outcomes of 3083 patients with cHL included in prospective trials.…”

The 2016 revision of the World Health Organization classification of lymphoid neoplasms

“…Recent data indicate that progression to a process with features of THRLBCL is associated with a more aggressive clinical course, and requires different management, such that the term NLPHL in this setting may not be sufficient. 147,148 However, cases with only focal diffuse areas are not considered transformation. It is also of interest that aside from their immunomorphologic appearance, GEP and array comparative genomic hybridization studies have shown similarities between NLPHL and THRLBCL, suggesting a relationship to each other, in spite of other major differences.…”