Diffuse large B-cell lymphoma research in Malaysia: A review.

Abstract

Lymphomas are prevalent worldwide and a common malignancy reported in Malaysia. Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of B-cell lymphomas accounting for 54% to 65% of all B-cell lymphomas and 39% to 57% of all malignant lymphomas. However, DLBCL comprises a heterogeneous group of diseases with different clinical presentations, biology and response to treatment. Recent advances in understanding the genetic landscape and molecular features of DLBCL have identified high-risk subsets with poor outcomes to chemo-immunotherapy that are actively being studied in various clinical trials. C-MYC is a proto-oncogene located in chromosome 8q24. 10 to 15 % of patients with newly diagnosed DLBCL have an underlying rearrangement of the MYC oncogene, resulting in dysregulated cellular survival and proliferation. Approximately half of these cases also carry a rearrangement of the anti-apoptotic proto-oncogene BCL2 and/or its transcription repressor BCL6. Over 20 case reports of DLBCL cases with notable features in Malaysia have found in the literature, in addition to a few extensive case series and included in this review. R-CHOP remains the mainstay of therapy and can help achieve control of long-term disease in nearly 90% of patients presenting with limited-stage and in up to 60% of those presenting with advanced stages. This review captures all 52 studies that reported DLBCL in Malaysia and summarises the essential aspects, including prevalence, subtype, prognostic markers clinical features in presentation and limited outcomes of cases when available.