Diffuse large B-cell lymphoma with MYC gene rearrangements: Current perspective on treatment of diffuse large B-cell lymphoma with MYC gene rearrangements; case series and review of the literature. (March 2016)

• Record Type: Journal Article
• Authors: de Jonge, A.V.; Roosma, T.J.A.; Houtenbos, I.; Vasmel, W.L.E.; van de Hem, K.; de Boer, J.P.; van Maanen, T.; Lindauer-van der Werf, G.; Beeker, A.; Timmers, G.J.; Schaar, C.G.; Soesan, M.; Poddighe, P.J.; de Jong, D.; Chamuleau, M.E.D.
• Is Part Of: European journal of cancer. Volume 55(2016)
• Page Start: 140
• Page End: 146
• Publication Date: 2016-03
• Subjects: DLBCL -- BCL-U -- Double hit lymphoma -- Triple hit lymphoma -- Single hit lymphoma -- MYC -- BCL2 -- R-CHOP -- DA-EPOCH-R; Cancer -- Periodicals; Neoplasms -- Periodicals; Cancer -- Périodiques; Cancer; Tumors; Electronic journals; Periodicals; Electronic journals; 616.994
• DOI: 10.1016/j.ejca.2015.12.001
• Languages: English
• ISSNs: 0959-8049

Abstract

Abstract: In the past decade, patients with newly diagnosed diffuse large B-cell lymphoma (DLBCL) were treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) therapy. Standard treatment is now changing as a result of deeper understanding of underlying biologic differences of such lymphomas. One of the most powerful predictors of an adverse outcome on R-CHOP therapy is the presence of a MYC gene rearrangement ( MYC + lymphoma). Determination of MYC gene rearrangement by FISH (fluorescent in situ hybridisation) has recently become a standard diagnostic procedure. In this paper, an overview of current literature on MYC function and MYC + lymphoma patient outcome is presented. Furthermore, we present 26 patients from our tertiary referral centre who were diagnosed with MYC + lymphoma between 2009 and 2014. In our patient series, we confirm the dismal prognosis of MYC + lymphoma patients. Intensification of classical chemotherapy does not lead to better overall survival, justifying new treatment modalities. First line therapy should be more specifically targeted against MYC and the genes and proteins that are deregulated by MYC. To this end, the first clinical trial in which MYC + patients will be offered targeted treatment has recently been launched. Highlights: Diffuse large B-cell lymphoma patients with MYC gene rearrangements have a dismal outcome on rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) therapy. Dismal outcome on standard therapy with R-CHOP justifies new treatment modalities Intensification of classical chemotherapy is not leading to better overall survival New therapies will be more specifically targeted against MYC and MYC related proteins