2020/02/04 by Lasse Hjort Jakobsen, Lasse H. Jakobsen, Fredrik Ellin +30 · 24 citations
Medicine · #Burkitt's lymphoma #CNS Lymphoma Diagnosis and Treatment #Internal medicine #Lymphoma #Lymphoma Diagnosis and Treatment #Medicine #Normalization (sociology) #Oncology #Viral-associated cancers and disorders
paper · pdf · doi:10.1111/bjh.16425
published in British Journal of Haematology 189(4), 661-671 (Wiley)
openalex publication_date 2020/02/04 · openalex created_date 2025/10/10 · openalex updated_date 2026/08/01
Non-endemic Burkitt lymphoma (BL) is a rare germinal centre B-cell-derived malignancy with the genetic hallmark of MYC gene translocation and with rapid tumour growth as a distinct clinical feature. To investigate treatment outcomes, loss of lifetime and relapse risk in adult BL patients treated with intensive immunochemotherapy, retrospective clinic-based and population-based lymphoma registries from six countries were used to identify 264 real-world patients. The median age was 47 years and the majority had advanced-stage disease and elevated LDH. Treatment protocols were R-CODOX-M/IVAC (47%), R-hyper-CVAD (16%), DA-EPOCH-R (11%), R-BFM/GMALL (25%) and other (2%) leading to an overall response rate of 89%. The two-year overall survival and event-free survival were 84% and 80% respectively. For patients in complete remission/unconfirmed, the two-year relapse risk was 6% but diminished to 0·6% for patients reaching 12 months of post-remission event-free survival (pEFS12). The loss of lifetime for pEFS12 patients was 0·4 (95% CI: -0·7 to 2) months. In conclusion, real-world outcomes of adult BL are excellent following intensive immunochemotherapy. For pEFS12 patients, the relapse risk was low and life expectancy similar to that of a general population, which is important information for developing meaningful follow-up strategies with increased focus on survivorship and less focus on routine disease surveillance.