scholarly journals EBV-Negative Monomorphic B-Cell Posttransplant Lymphoproliferative Disorder with Marked Morphologic Pleomorphism and Pathogenic Mutations in ASXL1, BCOR, CDKN2A, NF1, and TP53

2017 ◽  
Vol 2017 ◽  
pp. 1-8 ◽  
Author(s):  
Agata M. Bogusz

Posttransplant lymphoproliferative disorders (PTLDs) are a diverse group of lymphoid or plasmacytic proliferations frequently driven by Epstein-Barr virus (EBV). EBV-negative PTLDs appear to represent a distinct entity. This report describes an unusual case of a 33-year-old woman that developed a monomorphic EBV-negative PTLD consistent with diffuse large B-cell lymphoma (DLBCL) 13 years after heart-lung transplant. Histological examination revealed marked pleomorphism of the malignant cells including nodular areas reminiscent of classical Hodgkin lymphoma (cHL) with abundant large, bizarre Hodgkin-like cells. By immunostaining, the malignant cells were immunoreactive for CD45, CD20, CD79a, PAX5, BCL6, MUM1, and p53 and negative for CD15, CD30, latent membrane protein 1 (LMP1), and EBV-encoded RNA (EBER). Flow cytometry demonstrated lambda light chain restricted CD5 and CD10 negative B-cells. Fluorescence in situ hybridization studies (FISH) were negative for cMYC, BCL2, and BCL6 rearrangements but showed deletion of TP53 and monosomy of chromosome 17. Next-generation sequencing studies (NGS) revealed numerous genetic alterations including 6 pathogenic mutations in ASXL1, BCOR, CDKN2A, NF1, and TP53(x2) genes and 30 variants of unknown significance (VOUS) in ABL1, ASXL1, ATM, BCOR, BCORL1, BRNIP3, CDH2, CDKN2A, DNMT3A, ETV6, EZH2, FBXW7, KIT, NF1, RUNX1, SETPB1, SF1, SMC1A, STAG2, TET2, TP53, and U2AF2.

2010 ◽  
Vol 34 (3) ◽  
pp. 377-384 ◽  
Author(s):  
Dylan V. Miller ◽  
Dennis J. Firchau ◽  
Rebecca F. McClure ◽  
Paul J. Kurtin ◽  
Andrew L. Feldman

2016 ◽  
Vol 28 (6) ◽  
pp. 789 ◽  
Author(s):  
Mi-Hye Lee ◽  
Ik-Jun Moon ◽  
Woo-Jin Lee ◽  
Chong-Hyun Won ◽  
Sung-Eun Chang ◽  
...  

Blood ◽  
2007 ◽  
Vol 110 (3) ◽  
pp. 972-978 ◽  
Author(s):  
Sarah Park ◽  
Jeeyun Lee ◽  
Young Hyeh Ko ◽  
Arum Han ◽  
Hyun Jung Jun ◽  
...  

AbstractTo define prognostic impact of Epstein-Barr virus (EBV) infection in diffuse large B-cell lymphoma (DLBCL), we investigated EBV status in patients with DLBCL. In all, 380 slides from paraffin-embedded tissue were available for analysis by EBV-encoded RNA-1 (EBER) in situ hybridization, and 34 cases (9.0%) were identified as EBER-positive. EBER positivity was significantly associated with age greater than 60 years (P = .005), more advanced stage (P < .001), more than one extranodal involvement (P = .009), higher International Prognostic Index (IPI) risk group (P = .015), presence of B symptom (P = .004), and poorer outcome to initial treatment (P = .006). The EBER+ patients with DLBCL demonstrated substantially poorer overall survival (EBER+ vs EBER− 35.8 months [95% confidence interval (CI), 0-114.1 months] vs not reached, P = .026) and progression-free survival (EBER+ vs EBER− 12.8 months [95% CI, 0-31.8 months] vs 35.8 months [95% CI, 0-114.1 months], respectively (P = .018). In nongerminal center B-cell–like subtype, EBER in situ hybridization positivity retained its statistical significance at the multivariate level (P = .045). Nongerminal center B-cell–like patients with DLBCL with EBER positivity showed substantially poorer overall survival with 2.9-fold (95% CI, 1.1-8.1) risk for death. Taken together, DLBCL patients with EBER in situ hybridization+ pursued more rapidly deteriorating clinical course with poorer treatment response, survival, and progression-free survival.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Seyedeh Maryam Hosseini ◽  
Mojtaba Abrishami ◽  
Elham Barashki ◽  
Ghodsieh Zamani

Purpose. Herein, we report a case of primary intraocular lymphoma (PIOL) with the first presentation of bilateral Epstein-Barr virus- (EBV-) associated panuveitis. Case Presentation. A 69-year-old male was referred with a three-day history of blurred vision and pain and redness in his left eye following cataract surgery. Examination revealed panuveitis, vitritis, and necrotizing retinitis with retinal hemorrhage. A month later, the right eye was also involved. Polymerase chain reaction-based analysis of the vitreous sample was positive for EBV, and cytological evaluation was compatible with the diagnosis of B-cell lymphoma. A significant improvement was observed following serial intravitreal rituximab and methotrexate injections. The central nervous system and lungs were involved after 6 months, and the patient expired despite systemic chemotherapy. Conclusion. There may be an association between EBV panuveitis and primary intraocular B-cell lymphoma.


Sign in / Sign up

Export Citation Format

Share Document