Abstract: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… Show more
“…Dunleavy et al then showed the world remarkable results of a phase II trial with 67 patients submitted to a non-RT-based DA-EPOCH-R, giving this regimen a high scientific acceptance [ 13 ]. However, some later real-life evidences may suggest that, in some specific circumstances, DA-EPOCH-R might not be that better than R-CHOP, and also that the mediastinal RT might still have its role in the management of PMBCL [ 11 , 25 – 28 ]. Despite all its statistical limitations and bias, our findings find a path with those studies, summarized in Table 4 .…”
Primary mediastinal B-cell lymphoma (PMBCL) is an uncommon disease, consisting of 2–4% of non-Hodgkin lymphomas. Radiotherapy-free DA-EPOCH-R and R-CHOP plus radiotherapy (RT) have been the upfront standard regimens worldwide. However, performing DA-EPOCH-R in resource-constrained settings can be burdensome, especially in low/middle-income countries, where data on PMBCL are still largely unknown. We retrospectively analyzed 93 patients with PMBCL diagnosed between 2008 and 2018 with the intention of comparing the characteristics of the patients and the results obtained with each protocol and to verify if the use of less intensive chemotherapy is still possible to be used. The median age was 28 years, 59.1% were female, 42.3% were in advanced stages, and 92.1% were with bulky disease. DA-EPOCH-R (41.9%), R-CHOP (35.5%), and R-CHOEP (22.6%) were the regimens used, and no difference was observed in the characteristics of the patients. After four cycles of chemotherapy, complete response (CR), partial response (PR), and progressive disease (PD) rates were 40%, 55.7%, and 4.5%, respectively. At the end of treatment, metabolic CR and PD rates were 56.8% and 11.1%. RT was performed in 42.1% of DA-EPOCH-R, 75% of R-CHOP, and 83% of R-CHOEP, and switched PR to CR in 73.7%. Estimated 5-year PFS and OS were 77.2% and 77.4%, respectively. Only LDH levels remained independently associated with PFS, and type of treatment was not associated with OS, PFS, or relapse rate. Therefore, we conclude that in a resource-constrained setting, R-CHOP or R-CHOEP could be still safely adopted in upfront treatment for PMBCL.
“…Dunleavy et al then showed the world remarkable results of a phase II trial with 67 patients submitted to a non-RT-based DA-EPOCH-R, giving this regimen a high scientific acceptance [ 13 ]. However, some later real-life evidences may suggest that, in some specific circumstances, DA-EPOCH-R might not be that better than R-CHOP, and also that the mediastinal RT might still have its role in the management of PMBCL [ 11 , 25 – 28 ]. Despite all its statistical limitations and bias, our findings find a path with those studies, summarized in Table 4 .…”
Primary mediastinal B-cell lymphoma (PMBCL) is an uncommon disease, consisting of 2–4% of non-Hodgkin lymphomas. Radiotherapy-free DA-EPOCH-R and R-CHOP plus radiotherapy (RT) have been the upfront standard regimens worldwide. However, performing DA-EPOCH-R in resource-constrained settings can be burdensome, especially in low/middle-income countries, where data on PMBCL are still largely unknown. We retrospectively analyzed 93 patients with PMBCL diagnosed between 2008 and 2018 with the intention of comparing the characteristics of the patients and the results obtained with each protocol and to verify if the use of less intensive chemotherapy is still possible to be used. The median age was 28 years, 59.1% were female, 42.3% were in advanced stages, and 92.1% were with bulky disease. DA-EPOCH-R (41.9%), R-CHOP (35.5%), and R-CHOEP (22.6%) were the regimens used, and no difference was observed in the characteristics of the patients. After four cycles of chemotherapy, complete response (CR), partial response (PR), and progressive disease (PD) rates were 40%, 55.7%, and 4.5%, respectively. At the end of treatment, metabolic CR and PD rates were 56.8% and 11.1%. RT was performed in 42.1% of DA-EPOCH-R, 75% of R-CHOP, and 83% of R-CHOEP, and switched PR to CR in 73.7%. Estimated 5-year PFS and OS were 77.2% and 77.4%, respectively. Only LDH levels remained independently associated with PFS, and type of treatment was not associated with OS, PFS, or relapse rate. Therefore, we conclude that in a resource-constrained setting, R-CHOP or R-CHOEP could be still safely adopted in upfront treatment for PMBCL.
“…The risk of t-MDS/AML after R-DA-EPOCH should be accurately quantified and correlated to the reached dose level, especially in the young population of patients with PMLBCL. The accurate prediction of prognosis is required in order to tailor treatment to the individualized risk of failure [27][28][29] and potentially restrict more toxic chemotherapy to higher-risk patients. Meanwhile, PET/CT might facilitate the omission of radiotherapy in lower-risk patients [4][5][6]29] in strictly negative patients.…”
Background: R-DA-EPOCH is an effective regimen for PMLBCL, which permits the omission of consolidative radiotherapy in the majority of patients. Patient: We describe a 27-year-old female patient, who achieved a complete remission after treatment with six cycles of R-DA-EPOCH (up to the final level). At 6 months after the end of treatment, PET/CT revealed an unexpected, diffusely increased 18FDG uptake by the bone marrow. Simultaneously, pancytopenia with monocytosis was observed. Result: The patient was diagnosed with therapy-related myelodysplastic syndrome, which rapidly evolved into acute myeloid leukemia (t-MDS/AML) with MLL rearrangements. She achieved a complete remission after induction therapy, received an allogenic transplant and remains disease-free 2 years later. Conclusions: The extremely early onset of t-MDS/AML, together with the unexpected PET/CT findings make this case unique and highlights the need for the accurate estimation of the possible dose-dependent risk of t-MDS/AML after R-DA-EPOCH in the real-life setting in patients with PMLBCL.
“…Based on the above data, the clinical stage was IVB, the International Prognostic Index (IPI) was 2 and the age-adjusted IPI was also 2 falling into the low/intermediate and high/intermediate risk group respectively. However, this case is considered as “high-risk” based on extranodal involvement and highly elevated LDH (>2xULN) or bulky disease according to the scoring system, which was recently published by our group ( Vassilakopoulos et al, 2021 ). Fig.…”
Section: Case Presentationmentioning
confidence: 99%
“…PMLBCL is an uncommon thymic neoplasm of B-cell origin accounting for approximately 2.5% of non-Hodgkin lymphomas (NHL) ( Swerdlow et al, 2017 ). Unlike most cases of diffuse large B-cell lymphoma (DLBCL), PMLBCL typically affects young and middle-aged adults with a female predominance ( Vassilakopoulos et al, 2012 , Savage et al, 2006 , Vassilakopoulos et al, 2021 ). The majority of patients present with bulky mediastinal lymphadenopathy often causing dyspnea, dry cough and occasionally the superior vena cava syndrome.…”
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