Background. R-CHOP can cure approximately 75% of patients with primary mediastinal large B-cell lymphoma (PMLBCL), but prognostic factors have not been sufficiently evaluated yet. Rda-EPOCH is potentially more effective but also more toxic than R-CHOP. Reliable prognostic classification is needed to guide treatment decisions.Materials and Methods. We analyzed the impact of clinical prognostic factors on the outcome of 332 PMLBCL patients ≤65 years treated with R-CHOP AE radiotherapy in a multicenter setting in Greece and Cyprus. Results. With a median follow-up of 69 months, 5-year freedom from progression (FFP) was 78% and 5-year
Malignant T-cell lymphoproliferative diseases are relatively rare. T cells are activated through the T-cell receptor with the aid of costimulating molecules that can be either excitatory or inhibitory. Such pathways have been also implicated in mechanisms of malignant T-cell lymphoproliferative diseases' persistence and relapse by circumventing immune responses. To date, three major immunoinhibitory molecules have been recognized, namely programmed cell death-1 (PD-1), B and T lymphocyte attenuator (BTLA) and cytotoxic T lymphocyte antigen 4 (CTLA-4). Although CTLA-4 is considered the 'gatekeeper' of immune tolerance, PD-1 negatively regulates immune responses broadly, whereas BTLA activation has been shown to inhibit CD8+ cancer-specific T cells. Both PD-1 and BTLA downregulate proximal T-cell receptor signalling cascade and are involved in immune evasion of leukaemias and lymphomas, even after allogeneic stem cell transplantation. These immunoregulatory molecules can have seemingly a synergistic effect on weakening the immune response of patients with haematological malignancies, and their manipulation represents a very active field of preclinical as well as clinical interest.
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