Epstein-Barr Virus–Positive CD20- and CD45-Negative Diffuse Large B-Cell Lymphoma: A Diagnostic Challenge

Starr, Amy G.; Kwon, Dong Hyang; Kallakury, Bhaskar

doi:10.1177/1066896918784669

Cited by 10 publications

(7 citation statements)

References 14 publications

(15 reference statements)

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“…Immunohistochemistry revealed that the atypical cells were markedly positive for the B-cell-specific transcription factors Oct-2 and BOB.1 (Fig. 2b), which confirmed the B-cell derivation of the tumor cells [6]. An extensive analysis of FISH showed the tumor to be negative for C-Myc / IGH translocation as well as translocations involving CCND1 , BCL2 , and BCL-6 .…”

Section: Case Presentationmentioning

confidence: 71%

Successful treatment of refractory retroperitoneal Epstein-Barr virus-positive diffuse large B-cell lymphoma with secondary hemophagocytic syndrome by sequential combination regimen of PD-1 blockade and chimeric antigen receptor T cells: a case report

Zhang

et al. 2021

Anti-Cancer Drugs

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Epstein-Barr virus (EBV) is convincingly contributed to the development of several types of lymphomas such as NK/T cell lymphoma, Burkitt lymphoma, plasmablastic lymphoma, and diffuse large B cell lymphoma (DLBCL). Herein, we reported an atypical case of EBV-positive DLBCL in an immunocompetent young male patient who presented with epistaxis due to hypergammaglobulinemia. 2-Deoxy-2-[fluorine-8] fluoro-d-glucose PET/computed tomography showed multiple highly metabolic retroperitoneal tissue masses with the involvement of bilateral adrenal gland. Ultrasonography-guided biopsy revealed a significant number of lymphocytes and plasma-like cells that are immunopositive for plasmacell markers and partly positive for pan-B cell markers. The Ki-67 proliferation index was 20%. The extensive distribution of EBV-encoded small RNAs was confirmed by in-situ hybridization. Due to atypical/overlapping pathological characteristics, it was initially misdiagnosed as extramedullary plasmacytoma and treated with two cycles of bortezomib, lenalidomide, and dexamethasone. Disease progression occurred and pathology consultation for the retroperitoneal biopsies modified the diagnosis to EBV-positive DLBCL with plasma cell differentiation. The treatment was adjusted to etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, rituximab, and lenalidomide (R2-EPOCH), but no response was observed after three cycles of treatment and he developed hemophagocytic syndrome during treatment. A monotherapy of anti-programmed cell death-1 (PD-1) treatment with tiririzumab was administered, successfully controlling hemophagocytic syndrome and EBV infection. The response assessment was partial for EBV-positive DLBCL, subsequent anti-CD19 chimeric antigen receptor-T (CAR-T) cell therapy resulted in complete remission including lumps, immunoglobulins, and negative EBV-DNA 1.5 months later. The present case study proved the possibility of PD-1 blockade in controlling EBV infection and associated hemophagocytic syndrome and offered an example of the combination of CAR-T therapy and PD-1 blockade for refractory EBV-positive DLBCL in clinic. Anti-Cancer Drugs 33: e769-e775

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Section: Case Presentationmentioning

confidence: 71%

Successful treatment of refractory retroperitoneal Epstein-Barr virus-positive diffuse large B-cell lymphoma with secondary hemophagocytic syndrome by sequential combination regimen of PD-1 blockade and chimeric antigen receptor T cells: a case report

Zhang

et al. 2021

Anti-Cancer Drugs

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“… 9 Also, Starr et al described a case of CD20/CD45 negative EBV positive DLBCL but their case was positive for PAX-5 and CD79a while ours was negative for PAX-5. 25 Furthermore, it is still not clear why B-cell markers are downregulated in EBV positive B-cell lymphomas and association of B-cell marker suppression with EBV infection. 20 However, in our patient the serology was EBV and HIV negative as it was EBER in situ hybridisation ( Figure 1 ).…”

Section: Discussionmentioning

confidence: 99%

Primary Nodal Unclassifiable CD20 Negative Diffuse Large B-cell Lymphoma With Dual IgK and TCR Gene Rearrangement: A Diagnostic Challenge

Hadžisejdić

Klarica

Babarović

et al. 2023

Clin Med�Insights�Pathol

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Non-Hodgkin lymphomas are most frequently classified based on the lineage marker expression. However, lymphomas with aberrant marker expression as well as monoclonal IgH/IgΚ and TCR gene rearrangements may co-exist which can be misleading and confusing. Primary CD20 negative diffuse large B-cell lymphomas (DLBCL) represent a rare entity, and they account for 1% to 3% of cases. However, some CD20 negative DLBCLs could not be classified into known variants, creating both diagnostic and therapeutic dilemma’s. Primary CD20 negative DLBCL are more likely to have a non-germinal centre subtype, a higher proliferation index, more frequent extra-nodal involvement, a poorer response, and poorer prognosis to conventional treatment compared to CD20 positive DLBCL. A 66- year-old postmenopausal lady, presented with palpable, bilateral neck lymphadenopathy and difficulty swallowing. She also had left leg lymphoedema, poor appetited, fatigue and weight loss. Her symptoms lasted approximately 1 month. After histological, immunohistochemical and clonality analysis of the lymph node the patient was diagnosed with primary nodal CD20 and PAX-5 negative DLBCL with dual immunoglobulin light-chain kappa ( IgK) and T-cell receptor ( TCR) gene rearrangement. This unusual and unique case presented a diagnostic challenge because it was CD20 and PAX-5 negative, had dual IgK and TCR gene rearrangement and, it could not be classified within the known and well established CD20 negative DLBCL variants. Describing such cases emphasises the fact that lymphomas unclassifiable within known variants of CD20 negative DLBCL do exist and that range and heterogeneity of CD20 negative DLBCL continues to evolve, and pathologist should be aware of these uncommon, atypical mature B-cell neoplasms.

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“…The DLBCL is characterized by medium- to large-sized neoplastic cells that express a wide range of B-cell markers including CD19, CD20, CD22, and CD79a. [24] Since CD20-negative DLBCL was reported, [25] several mechanisms for the CD20 phenotypic change after rituximab administration have been proposed, including selection of a CD20-negative clone as a consequence of rituximab exposure or downregulation of the CD20 antigen by genetic and epigenetic changes. [26] As an extremely rare disease, CD20-negative DLBCLs include primary effusion lymphoma (PEL), plasmablastic lymphoma (PBL), anaplastic lymphoma kinase (ALK)-positive DLBCL, and large B-cell lymphoma arising from human herpesvirus 8 (HHV-8)-associated multicentric Castleman disease (MCD).…”

Section: Discussionmentioning

confidence: 99%

CD20-negative primary middle ear diffuse large B-cell lymphoma coexpressing MYC and BCL-2 secondary to acute lymphoblastic leukemia

Ding

Huang²,

Shi³

et al. 2019

Medicine

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Rationale: Second diffuse large B-cell lymphoma (DLBCL) after treatment of acute lymphoblastic leukemia (ALL) is uncommon. To our knowledge, primary middle ear DLBCL which presents CD20-negative and coexpression of MYC and BCL-2 has not been reported yet. Patient concerns: A 20-year-old Chinese man complained fever and weakness for 2 months. Subsequently bone marrow morphology and flow cytometry immunophenotype suggested ALL. Administrated with 9 cycles of multiagent combined chemotherapy, he felt right ear progressive hearing loss, otalgia, aural fullness. Otoendoscopic examination revealed a pitchy mass obstructing the right external auditory canal. Then the mass resection was performed for biopsy and immunohistochemistry examination. Diagnosis: The mass was diagnosed as DLBCL which was negative for CD20 and double expression of MYC and BCL-2. Interventions: Chemotherapy. Outcomes: The patient eventually gave up and died of severe infection. Lessons: Although intensive chemotherapy has markedly improved the survival of ALL, more and more secondary cancers have been reported. In addition, primary middle ear lymphoma is much rare; hence, it is easy to be misdiagnosed. Furthermore, DLBCL with negative CD20 and double expression of MYC and BCL-2 is aggressive, which is characterized by chemotherapy resistance and inferior survival rates. We discuss this case aiming at raising awareness of tumors secondary to ALL and exploring the appropriate treatment options for the rare DLBCL.

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Epstein-Barr Virus–Positive CD20- and CD45-Negative Diffuse Large B-Cell Lymphoma: A Diagnostic Challenge

Cited by 10 publications

References 14 publications

Successful treatment of refractory retroperitoneal Epstein-Barr virus-positive diffuse large B-cell lymphoma with secondary hemophagocytic syndrome by sequential combination regimen of PD-1 blockade and chimeric antigen receptor T cells: a case report

Successful treatment of refractory retroperitoneal Epstein-Barr virus-positive diffuse large B-cell lymphoma with secondary hemophagocytic syndrome by sequential combination regimen of PD-1 blockade and chimeric antigen receptor T cells: a case report

Primary Nodal Unclassifiable CD20 Negative Diffuse Large B-cell Lymphoma With Dual IgK and TCR Gene Rearrangement: A Diagnostic Challenge

CD20-negative primary middle ear diffuse large B-cell lymphoma coexpressing MYC and BCL-2 secondary to acute lymphoblastic leukemia

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