How is relapsed or refractory primary mediastinal B-cell lymphoma (PMBCL) treated?

Updated: Sep 14, 2019
  • Author: Sonali M Smith, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Answer

While PMBCL is often cured with standard first-line therapies, approximately 200 patients per year in the United States are diagnosed with relapsed/refractory PMBCL, which has a poor prognosis with a two-year survival of 15%. [37] Due to its rarity, no standard of care has been identified and relapsed/refractory disease is generally treated following protocols for other DLBCL subtypes. [20]

Patients with relapsed disease often have systemic involvement and many also have extranodal disease. These patients are recommended to undergo salvage systemic chemotherapy followed by stem cell collection and subsequent transplantation. Rituximab, ifosfamide, carboplatin, and etoposide (RICE) is a common salvage approach, although other regimens are acceptable. Patients who demonstrate chemosensitive disease (improvement radiographically and on PET) are taken to transplantation. Patients who have refractory disease should be offered clinical trials, although some can be considered for allogeneic bone marrow transplantation.

PMBCL frequently involves PD-1 ligand overexpression, potentially making PMBCL susceptible to PD-1 blockade. NCCN has included pembrolizumab, a humanized anti–PD-1 monoclonal antibody blocking interaction of PD-1 with its ligands, PD-L1 and PD-L2, among the recommended treatments for relapsed/refractory PMBCL. [20]  


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