Long‐term follow‐up of patients with nodular lymphocyte predominant Hodgkin lymphoma: A report from the Spanish Lymphoma Oncology Group

Abstract

Nodular lymphocytic predominance Hodgkin lymphoma (NLPHL) is a very uncommon subtype of Hodgkin lymphoma (HL), representing approximately 5% of all HL cases, with an incidence of 0.3/100,000 cases per year and with unique characteristics which distinguish it from classic Hodgkin lymphoma. Given its low frequency, there is a lack of prospective randomized studies to inform practice, the accumulated experience of academic groups being the main source of relevant information for the management of these patients. Eighty‐five patients recruited by the Spanish Lymphoma Group (GOTEL) from 12 different hospitals were retrospectively analyzed to describe their sociodemographic and clinical characteristics. The median follow‐up was 16 years, with a 10‐year overall survive of 92.9% and 81.2% at 20 years. Five patients developed a second malignancy. No transformation to a more aggressive lymphoma was detected. A total of 31% tumor relapses was found: 77% in a single location; most of them at a supra‐diaphragmatic level. Patients received different first‐line treatments, and progression was observed in 3/4 (75%) of the patients who did not receive any type of treatment, 6/23 (26%) who received both chemotherapy (CH) and radiotherapy (RT), 12/43 (27%) who received RT and 7/15 (47%) that received only CH treatment. The mean time to relapse was 3 years and 47% presented relapses beyond 5 years (higher probability in stage IV p < 0.001). This is one of the longest follow‐up series of NLPHL published, confirming its excellent prognosis, and that treatments may be adapted to reduce toxicity. Causes of death in these patients are varied, and the minority due to a primary malignancy relapses.

Microenvironmental immune cell alterations across the spectrum of nodular lymphocyte predominant Hodgkin lymphoma and T-cell/histiocyte-rich large B-cell lymphoma

“…Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a rare lymphoma with incidence of up to 0.3 cases per 100,000 person years ( 1 , 2 ). The sparse neoplastic “lymphocyte predominant” (LP) cells of NLPHL retain a germinal centre B-cell programme including expression of markers such as CD20, CD79a and BCL6; ongoing somatic hypermutation; and engagement with PD1-expressing T-follicular helper cells (Tfh) through immunological synapses ( 3 – 6 ).…”

“…( 13 ). Typical cases show LP cells residing in B-cell rich nodular/serpiginous formations (patterns A/B) and present with localised disease following an indolent course ( 1 , 2 , 14 ). Less typically, LP cells reside in a T-cell rich TME which is either extra-nodular (pattern C), intra-nodular (pattern D), diffuse (THRLBCL-like pattern E), or within “moth eaten” diffuse B-cell areas (pattern F).…”