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Erschienen in: Indian Journal of Hematology and Blood Transfusion 4/2020

01.10.2020 | Original Article

Double Hit and Double Expresser Diffuse Large B Cell Lymphoma Subtypes: Discrete Subtypes and Major Predictors of Overall Survival

verfasst von: Anurag Mehta, Ajita Verma, Garima Gupta, Rupal Tripathi, Anurag Sharma

Erschienen in: Indian Journal of Hematology and Blood Transfusion | Ausgabe 4/2020

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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.
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Metadaten
Titel
Double Hit and Double Expresser Diffuse Large B Cell Lymphoma Subtypes: Discrete Subtypes and Major Predictors of Overall Survival
verfasst von
Anurag Mehta
Ajita Verma
Garima Gupta
Rupal Tripathi
Anurag Sharma
Publikationsdatum
01.10.2020
Verlag
Springer India
Erschienen in
Indian Journal of Hematology and Blood Transfusion / Ausgabe 4/2020
Print ISSN: 0971-4502
Elektronische ISSN: 0974-0449
DOI
https://doi.org/10.1007/s12288-019-01248-w

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