Clinical characteristics and outcomes of patients with Hodgkin lymphoma with central nervous system involvement: An international multicenter collaboration

Abstract

Central nervous system (CNS) involvement is rare in patients with Hodgkin lymphoma (HL). Thus, the clinical features and outcomes are not well described. Cases of histologically confirmed CNS HL diagnosed between 1995 and 2015 were retrospectively identified in institutional (n 5 7), national (n 5 2), and cooperative group (n 5 1) databases. We screened 30,781 patients with HL in our combined databases and identified 21 patients meeting eligibility criteria, an estimated frequency of 0.07%. CNS involvement was present at initial diagnosis in 10 patients (48%) and a feature of relapsed/refractory disease in 11 (52%). Among these 11 patients, the median time from initial diagnosis of HL to development of CNS involvement was 1.9 years (range 0.4-6.6) and the median number of prior lines of therapy was 2 (range 1-7). Altogether, treatments included radiation, multiagent systemic chemotherapy, combined modality therapy, and subtotal resection. The overall response rate was 65%. After a median follow-up of 3.6 years (range 0.8-13.2) from diagnosis of CNS HL, the median PFS and OS were 7.6 and 29 months, respectively. CNS involvement as a feature of relapsed/refractory disease was adversely prognostic for both PFS and OS; however, four patients remain alive and free of relapse at 7-78 months follow-up. CNS involvement in HL is exceedingly rare and has a distinct clinical presentation with predilection for parenchymal lesions with dural extension. Around one-quarter of patients, mostly with CNS involvement at initial HL diagnosis, experience prolonged disease-free survival.

Meningeal Relapse of Nodular Lymphocyte Predominant Hodgkin Lymphoma Transformed to T-Cell/Histiocyte-Rich Large B-Cell Lymphoma: A Case Report

“…CNS involvement in HL is extremely rare at diagnosis and in patients with relapsed or refractory disease. In a recent study, Cheah et al screened the clinical data of more than 30,000 HL patients and identified only 21 cases with CNS involvement, corresponding to an incidence <0.1% (3). Two retrospective series have reported a total of 37 HL patients with CNS involvement (2,3).…”

“…In a recent study, Cheah et al screened the clinical data of more than 30,000 HL patients and identified only 21 cases with CNS involvement, corresponding to an incidence <0.1% (3). Two retrospective series have reported a total of 37 HL patients with CNS involvement (2,3). With the exception of one case of NLPHL who had CNS involvement at the time of the diagnosis, the other 36 cases were classical HL with roughly half of them presenting CNS involvement at the time of primary diagnosis, and the other half presenting CNS relapse.…”

Durable Response to Brentuximab Vedotin-Based Chemotherapy in Refractory Hodgkin Lymphoma with Central Nervous System (CNS) Involvement

“…The incidence of CNS involvement in Hodgkin lymphoma (HL) is 0.07% [1]. It can occur at the time of diagnosis or in the case of relapsed/refractory disease.…”

“…Risk factors for the dissemination of HL into CNS are unknown due to the limited number of patients and heterogeneity of data. Various treatments were used in these patients, including surgical resection, corticosteroids, radiation, multiagent systemic chemotherapy, and autologous stem cell transplantation (ASCT) [1]. We report the case of a patient with refractory Hodgkin lymphoma with CNS involvement who achieved durable response with brentuximab vedotin-based chemotherapy.…”

“…The overall response rate was 65%. A median follow-up of 3.6 years (range 0.8-13.2) since diagnosis of CNS HL was observed, and the median progression-free survival (PFS) and overall survival (OS) were 7.6 and 29 months, respectively [1]. Relapsed/refractory HL in CNS had an inferior median PFS (4 vs. 14 months, P=0.002) and OS (13 vs. 105 months, P=0.004) as compared to initial CNS involvement.…”