Addition of nivolumab (immune checkpoint inhibitor) to standard chemotherapy (DA-EPOCH-R) may improve outcome in children with primary mediastinal large B-cell lymphoma

A total of 6 blocks of Nivolumab+DA-EPOCH-R are planned for all patients regardless of stage and other risk factors. Nivolumab 40 mg (Day 2), Rituximab 375 mg/m2 (Day 1) and Prednisolone 60 mg/m2 (Days 1-5) are administered at constant unchangeable doses. Other drugs are administered at starting doses (Etoposide 50 mg/m2 (Days 1-4), Doxorubicin 10 mg/m2 (Days 1-4), Vincristine 0,4 mg/m2 (Days 1-4) and Cyclophosphamide 750 mg/m2 (Day 5). These starting doses are adjusted further according to standard principles of DA-EPOCH-R.

Inclusion Criteria:

• Age from 0 years to 17 years 11 months

  • Established on the basis of histological and immunohistochemical studies, the diagnosis of PMLBL (the presence of programmed death-ligand on tumor cells is not necessary)
  • Lack of specific therapy for lymphoma
  • Signed informed consent

Exclusion Criteria:

• Age >18

  • History of specific therapy for lymphomas.
  • Drug intolerance