Double Hit and Double Expresser Diffuse Large B Cell Lymphoma Subtypes: Discrete Subtypes and Major Predictors of Overal
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ORIGINAL ARTICLE
Double Hit and Double Expresser Diffuse Large B Cell Lymphoma Subtypes: Discrete Subtypes and Major Predictors of Overall Survival Anurag Mehta1,3 • Ajita Verma1 • Garima Gupta2 • Rupal Tripathi2 Anurag Sharma2
•
Received: 25 September 2019 / Accepted: 28 December 2019 Ó Indian Society of Hematology and Blood Transfusion 2020
Abstract Double hit lymphomas (DHL) and double expresser lymphomas (DEL) are subsets of diffuse large B cell lymphomas (DLBCL) which are being increasingly recognised as cause of treatment failure. This emphasizes the need for their separation from other DLBCL cases in order to prognosticate and administer more aggressive treatment to this set of patients. The present study was conducted with the aim to identify the DHL/DEL patients and study their distinctive clinicopathological profile and overall survival. This retrospective analysis involved 172 cases of DLBCL sub-classified on the basis of cell of origin. Immunohistochemical (IHC) analysis for MYC, BCL2, BCL6, MUM1 and CD10 was performed. Rearrangement studies were performed using break apart Fluorescent in situ hybridization. Overall survival (OS) was also evaluated. Distinctive clinical and pathological features of DHL and DEL were identified. Rearrangement study by FISH revealed seven cases of DHL (MYC ? BCL2 &/or BCL6 rearrangement). Also, 20 patients (11.6%) showed a concurrent expression of BCL2 and MYC oncoproteins
(DEL) on IHC. Most (6/7) DHL patients were double expressors also. The DHL patients demonstrated a significant association with female gender, high serum LDH levels ([ 750 U/L) and GCB phenotype. DEL patients contrarily predominated amongst males, had intermediate LDH levels (251–500 U/L) and non GCB phenotype. The OS of the patients was 63.8% at 4 years. The OS of the DLBCL, DEL and DHL patients was 71.9%, 46.9%, and 0%, respectively at 4 years (p value 0.010). In case of DEL subtype, factors such as age \ 60 years (66.7%), male sex (60.8%), nodal localization (52.5%), early disease stage (84.6%), low IPI score (60%), absence of B symptoms (50%), LDH \ 250 U/L (80%) and GCB phenotype (53.3%) were associated with better OS. Further, the OS of DHL cases was 0% at 4 years. Double hit and double expresser lymphomas have poor prognostic outcomes and should be separated from DLBCL. All DELs should be tested for DHLs and especially those with immunoblastic morphology. DHL and DEL subtypes delineate the subtypes with inferior OS and reinstate the need for aggressive interventions.
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s12288-019-01248-w) contains supplementary material, which is available to authorized users.
Keywords Double hit lymphoma Double expresser lymphoma Diffuse large B cell lymphoma C-MYC BCL2 BCL6
& Anurag Mehta [email protected] 1
Department of Laboratory and Transfusion Services, Rajiv Gandhi Cancer Institute and Research Centre, Rohini, Delhi, India
2
Department of Research, Rajiv Gandhi Cancer Institute and Research C
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