Dual Threat of Epstein-Barr Virus: an Autopsy Case Report of HIV-Positive Plasmablastic Lymphoma Complicating EBV-Associated Hemophagocytic Lymphohistiocytosis

Koizumi, Yusuke; Imadome, Ken‐Ichi; Ota, Yasunori; Minamiguchi, Hitoshi; Kodama, Yoshinori; Watanabe, Dai; Mikamo, Hiroshige; Uehira, Tomoko; Okada, Seiji; Shirasaka, Takuma

doi:10.1007/s10875-018-0500-4

Cited by 13 publications

(15 citation statements)

References 20 publications

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“…A previous study has suggested that EBV positivity is of importance when the differential diagnosis exists between MM and plasmablastic lymphoma (PBL), which is an aggressive malignant B-cell neoplasm. PBL patients (60–75%) have EBV infections and usually die within 1 year [22]. However, the clinical course of MM is indolent.…”

Section: Discussionmentioning

confidence: 99%

Epstein–Barr virus infection is associated with clinical characteristics and poor prognosis of multiple myeloma

et al. 2019

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The aim of the present study was to evaluate the relationship of Epstein–Barr virus (EBV) infection and multiple myeloma (MM) and its impact on clinical characteristics and prognosis. Fresh peripheral blood mononuclear cells (PBMCs) from 139 MM patients who had been diagnosed and treated from January 2010 to May 2018 and 50 PBMC samples from healthy donors were obtained. PCR was carried out for detection of EBV-DNA. The results indicated a significantly higher EBV-DNA concentration among 139 MM patients compared with healthy controls (P<0.05). Correlation analysis showed that the expression of EBV-DNA was positively correlated with the serum free light chain ratio (sFLCR) and progressive disease (PD)/relapse (P<0.05). Especially, in EBV-DNA high-expression MM patients, EBV-DNA concentration for patients with sFLCR ≥100 was higher than that of patients with sFLCR <100. EBV-DNA concentration was higher in patients with disease PD/relapse than those without disease PD/relapse. In univariate analysis, the progress free survival (PFS) was inferior in MM patients with high expression of EBV-DNA, high lactate dehydrogenase (LDH), and high-risk according to mSMART and International Myeloma Working Group (IMWG), stage III according to R-ISS staging, extramedullary lesions, and genetic changes (P<0.05). However, in multivariate analysis, LDH, poor karyotype, R-ISS staging, and mSMART were independent prognostic factors for PFS. Taken together, our studies suggest that an association exists between EBV infection and clinical characteristics of MM patients, and EBV infection appears to have a slight impact on the prognosis of MM. However, the results require further validation in other independent prospective MM cohorts.

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Section: Discussionmentioning

confidence: 99%

Epstein–Barr virus infection is associated with clinical characteristics and poor prognosis of multiple myeloma

et al. 2019

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“…PBMCs were isolated by density centrifugation. Samples of 1.0 × 10 6 /mL PBMCs were then serially bead‐sorted by positive selection (BD Biosciences, USA) into CD19 + , CD4 + , CD8 + , and CD56 + cell fractions in order 8,9 . DNA was extracted from each fraction.…”

Section: Methodsmentioning

confidence: 99%

“…Samples of 1.0 × 10 6 /mL PBMCs were then serially bead-sorted by positive selection (BD Biosciences, USA) into CD19 + , CD4 + , CD8 + , and CD56 + cell fractions in order. 8,9 DNA was extracted from each fraction. EBV loads are measured in copies/μg DNA to identify the EBV-positive fraction.…”

Section: Identification Of Ebv-infected Cellsmentioning

confidence: 99%

Fatal Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis with virus‐infected T cells after pediatric multivisceral transplantation: A proof‐of‐concept case report

Yamada

Sakamoto

et al. 2020

Pediatric Transplantation

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“…Analysis of EBV gene expression by real time-PCR was carried out as previously described (15). Briefly, peripheral blood mononuclear cells (PBMCs) were isolated by centrifugation on a Lymphosepar I (Immuno-Biological Laboratories Co., Ltd., Gunma, Japan).…”

Section: Methodsmentioning

confidence: 99%

Systemic Epstein–Barr Virus-Positive T/NK Lymphoproliferative Diseases With SH2D1A/XIAP Hypomorphic Gene Variants

et al. 2019

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X-linked lymphoproliferative disease (XLP) is one of the X-linked primary immunodeficiency diseases (PIDs) with defective immune response to Epstein–Barr virus (EBV) infection. Chronic active EBV infection (CAEBV) and EBV-hemophagocytic lymphohistiocytosis (HLH) are recognized as systemic EBV-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases (LPDs) arising from the clonal proliferations of EBV-infected T cells and NK cells. A high incidence of CAEBV in East Asia implies the unknown genetic predisposition. In patients with XLP, EBV-infected cells are generally B cells. No mutation of SH2D1A / XIAP genes has ever been identified in patients with systemic EBV-positive T-cell and NK-cell LPD. We report herewith a male case of NK-cell type CAEBV with SH2D1A hypomorphic mutation (c.7G > T, p.Ala3Ser), two male cases of CAEBV/EBV-HLH with XIAP hypomorphic variant (c.1045_1047delGAG, p.Glu349del), and another female case of CD4 + CAEBV with the same XIAP variant. The female underwent bone marrow transplantation from an HLA-matched sister with the XIAP variant and obtained a complete donor chimerism and a cure of laryngeal LPD lesion, but then suffered from donor-derived CD4 + T cell EBV-LPD. These observations demonstrated that SH2D1A and XIAP genes are critical for the complete regulation of EBV-positive T/NK cell LPD. X-linked lymphoproliferative disease (XLP) is one of the X-linked primary immunodeficiency diseases (PIDs) reported to have a defective immune response to Epstein–Barr virus (EBV) infection. Mutations in SH2D1A and XIAP genes cause XLP. Systemic EBV-positive T-cell and natural killer (NK)-cell lymphoproliferative diseases (LPDs) consist of three major types: EBV-positive hemophagocytic lymphohistiocytosis (HLH), chronic active EBV infection (CAEBV), and EBV-positive T-cell/NK-cell lymphoma. CAEBV is recognized as a poor prognostic disease of EBV-associated T-cell and NK-cell LPD arising from the clonal proliferation of EBV-infected T cells (CD4 + , CD8 + , and TCRγδ + ) and/or NK cells. The majority of cases with CAEBV were reported from East Asia and South America. In Caucasian patients with CAEBV disease, the target of infection is exclusively B cells. These imply a genetic predisposition to EBV-positive T/NK cell LPD according to ethnicity. In reported cases with XLP, EBV-infected cells are B cells. On the other hand, no mutation of SH2D1A / XIAP genes have been determined in patients with T/NK-cell-type (Asian type) CAEBV. We here describe, for the first time, four case series of CAEBV/EBV-HLH patients who carried the hypomorphic variants of XLP-related genes. These cases included a male ...

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Dual Threat of Epstein-Barr Virus: an Autopsy Case Report of HIV-Positive Plasmablastic Lymphoma Complicating EBV-Associated Hemophagocytic Lymphohistiocytosis

Cited by 13 publications

References 20 publications

Epstein–Barr virus infection is associated with clinical characteristics and poor prognosis of multiple myeloma

Epstein–Barr virus infection is associated with clinical characteristics and poor prognosis of multiple myeloma

Fatal Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis with virus‐infected T cells after pediatric multivisceral transplantation: A proof‐of‐concept case report

Systemic Epstein–Barr Virus-Positive T/NK Lymphoproliferative Diseases With SH2D1A/XIAP Hypomorphic Gene Variants

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