Abstract:
: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.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Jakobsen LH,Ellin F,Smeland KB,Wästerlid T,Christensen JH,Jørgensen JM,Josefsson PL,Øvlisen AK,Holte H,Blaker YN,Grauslund JH,Bjørn J,Molin D,Lagerlöf I,Smedby KE,Colvin K,Thanarajasingam G,Maurer MJ,Habermann TM,Sodoi
10.1111/bjh.16425subject
Has Abstractpub_date
2020-05-01 00:00:00pages
661-671issue
4eissn
0007-1048issn
1365-2141journal_volume
189pub_type
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