Efficacy of ibrutinib as salvage treatment in a secondary central nervous system lymphoma (SCNSL) progressed after chemorefractory Primary Mediastinal B Cell Lymphoma (PMBCL)

In the last few years, promising data have been published about frontline treatment in Primary Mediastinal BCell Lymphoma (PMBCL), both in adults and children; adapting different rituximab-based regimens, relapse free survival (RFS) rates range from 78% to 93% and overall survival (OS) from 88% to 97% in distinct cohorts [1–3]. However, few data are available for relapsed/refractory (R/R) patients, globally showing a poor prognosis. Patients with chemo-sensitive relapse usually undergo to a salvage treatment followed by autologous stem cell transplant (ASCT) but only 25%–40% of these patients receive ASCT and 60–70% of them show durable remission [4]. Patients with chemo-resistant disease have historically a dismal outcome demonstrating lack of therapeutic strategies in this subset. Central nervous system (CNS) relapse is a rare event in PMBCL accounting for approximately 5% of Diffuse Large B cell Lymphomas (DLBCL) in adult series [5]. Data regarding treatment and prognosis in these patients are still lacking. Therefore, treatments of PMBCL CNS involvement often refer to those employed in the more frequent diffuse large B-cell lymphoma (DLBCL).