Abstract
Primary mediastinal B cell lymphoma is a rare entity and often should be promptly treated as a hematological emergency: The initial treatment decision is crucial for the management of this disease. An observational retrospective study was conducted with the aim to improve information on treatment and outcomes of primary mediastinal B cell lymphoma in real practice. After 12 cycles of MACOP-B regimen (methotrexate, doxorubicin, cyclophosphamide, vincristine, bleomycin, and prednisone) with or without rituximab, 120 patients out of 151 (79.5%) achieved a complete response and 12 (7.9%) a partial response leading to a global response of 87.4%. The 21-year overall survival is 82.6%; progression-free and disease-free survivals are 69.3% and 86.4%, respectively. Regarding the role of radiotherapy (RT), patients with a negative PET scan after MACOP-B did not undergo RT: One out of these 48 (2.1%) showed a relapse at 11 months. All relapsed/refractory patients who achieved a response with checkpoint inhibitors are still in continuous complete response with a median follow-up of 14 months. Data that we have gathered over a 30-year experience in the treatment of primary mediastinal B cell lymphoma patients clearly indicate that a third-generation chemotherapy regimen such as MACOP-B is feasible and easily deliverable on an outpatient basis. Regarding the unmet medical need of relapsed/refractory patients, new encouraging results occurred with the advent of the checkpoint inhibitors.
Highlights
Primary mediastinal B cell lymphoma (PMBCL) is a rare subtype of non-Hodgkin lymphoma with unique clinical and molecular characteristics [1]
Patients whose disease is refractory to standard treatments or who relapse after an initial response are difficult to manage and generally respond poorly to conventional regimens: For this reason, new drugs with innovative mechanisms of action are urgently needed in this context
We report our 30-year monocentric experience in the first-line treatment of PMBCL patients with 151 consecutive cases to analyze response and outcomes
Summary
Primary mediastinal B cell lymphoma (PMBCL) is a rare subtype of non-Hodgkin lymphoma with unique clinical and molecular characteristics [1]. The 1994 Revised European American Lymphoma Classification firstly recognizes PMLBCL as a subtype of diffuse large B cell lymphoma (DLBCL), it has been regarded as a specific clinical and biological entity since the 2001 World Health Organization classification [2, 3]. The disease classically occurs in adolescent and young adult women and presents with bulky mediastinal adenopathy. PMBCL is distinct from DLBCL and shares many biologic similarities with classical Hodgkin lymphoma, including dysregulation of JAK-STAT and NF-κB signaling and overexpression of PD1 ligands [4,5,6,7]. Patients whose disease is refractory to standard treatments or who relapse after an initial response are difficult to manage and generally respond poorly to conventional regimens: For this reason, new drugs with innovative mechanisms of action are urgently needed in this context
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