![]() ![]() Notably, patients were not exposed to radiotherapy 11. DA-EPOCH-R regimen recently promoted by the group from National Cancer Institute, USA, was implemented in a number of North American centers based on results of phase II study of 51 PMBL patients with 5-year event free survival and 5-year OS of 93% and 97%, respectively 11. A continuous infusion variant of CHOP chemotherapy enriched in etoposide, i.e. Recent studies suggested that RT can be safely abandoned if end-of treatment positron emission tomography (EOT-PET) is negative 7, 11. Generally, R-CHOP with or without RT is the most widely used regimen for PMBL 4, 5, 6, 7, 8, 9, 10 with a 5-year PFS and OS of 77–95% and 84–98%, respectively. Our knowledge is based primary on retrospective reports with a limited number of patients. ![]() Due to rarity of the disease and absence of randomized studies there is general uncertainty on the optimal treatment approach. Recent studies based on targeted sequencing and mutational profile suggest common molecular features of PMBL and non-mediastinal DLBCL tumours as well as relation to classical Hodgkin lymphoma 2, 3. Primary mediastinal large (thymic) B cell lymphoma (PMBL) is an aggressive disease with unique pathological, molecular and clinical features 1. The attenuated dose of doxorubicin and intermediate dose of methotrexate may contribute to low incidence of late cardiotoxicity and effective CNS prophylaxis. Intensive alternating immunochemotherapy protocol GMALL/B-ALL/NHL2002 is curative for more than 90% of PMBL patients and late toxicity in young patients is moderated. Second malignancies and late cardiotoxicity occurred in 2.4% and 2.4% of patients, respectively. Acute toxicity included pancytopenia grade 3/4, neutropenic fever, and treatment related mortality rate of 0.8%. ![]() Eight (6%) patients had recurrent/refractory disease, however, no central nervous system (CNS) relapse was observed. ![]() Positron emission tomography-computed tomography (PET-CT) results at the end of chemotherapy were predictive for outcome: OS and PFS at 5 year were 96% and 94% in PET-CT negative patients, respectively, and 70% and 70% in PET-CT-positive patients (p = 0.004 for OS, p = 0.01 for PFS). With a median (range) follow up of 9 (1–17) years, 5-year overall survival (OS) and 5-year progression free survival (PFS) were 94% and 92%, respectively. A median (range) age of patients was 30 (18–59) years, and 60% were female. Majority of patients (77%) received consolidative radiotherapy. Here we present results of treatment of 124 patients with PMBL over a period between 20 with the use of a protocol designed for aggressive B-cell lymphoma GMALL/B-ALL/NHL2002 including 6 cycles of alternating immunochemotherapy with intermediate-dose methotrexate in each cycle, and reduced total doxorubicin dose (100 mg/m 2 for whole treatment). Treatment regimens frequently used include RCHOP ± radiotherapy, DAEPOCH-R, or occasionally more intensive protocols. Primary mediastinal B-cell lymphoma (PMBL) is currently curable in 85–95% of patients. ![]()
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