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Liu Y , Barta SK
Diffuse Large B cell Lymphoma: 2019 update on diagnosis, risk-stratification and treatment
Am J Hematol. 2019 May;94(5) :604-616
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DISEASE OVERVIEW: Diffuse Large B cell lymphoma is the most common type of aggressive Non-Hodgkin lymphoma originating from the germinal center, and represents a heterogeneous group of diseases with variable outcomes that are differentially characterized by clinical features, cell of origin, molecular features, and most recently, frequently recurring mutations. DIAGNOSIS: DLBCL is ideally diagnosed off an excisional biopsy of a suspicious lymph node, which shows sheets of large cells that disrupt the underlying structural integrity of the follicle center and stain positive for pan-B cell antigens, such as CD20 and CD79a. Cell of origin is determined by immuno-histochemical stains, while molecular features such as double or triple hit disease is determined by FISH analysis. Commercial tests for frequently recurring mutations are currently not routinely used to inform treatment. RISK STRATIFICATION: Clinical prognostic systems for DLBCL, including the R-IPI, age-adjusted and NCCN-IPI use clinical factors to risk stratify patients, although this does not affect treatment approach. Furthermore, DLBCL patients with non-GCB-like DLBCL (activated B-cell like and unclassifiable) have a poorer response to up-front chemo-immunotherapy compared to patients with GCB-like DLBCL. Those with C-MYC altered disease alone and in combination with translocations in BCL2 and/or BCL6 (particularly when the MYC translocation partner is immunoglobulin) respond poorly to up-front chemoimmunotherapy and salvage autologous stem cell transplant at relapse. RISK-ADAPTED THERAPY: This review will focus on differential treatment of DLBCL up-front and at the time of relapse by cell of origin and molecular features. This article is protected by copyright. All rights reserved.
1096-8652 Liu, Yang ORCID: https://orcid.org/0000-0001-6606-9201 Barta, Stefan Klaus Journal Article United States Am J Hematol. 2019 May;94(5):604-616. doi: 10.1002/ajh.25460