Article
Pediatrics
Aleksandra Szczawinska-Poplonyk, Elzbieta Grzesk, Eyal Schwartzmann, Anna Materna-Kiryluk, Jadwiga Maldyk
Summary: ALPS is a disorder characterized by disruptions in lymphocyte apoptosis pathway and immune system homeostasis. This study highlights the challenges in diagnosing ALPS in the presence of CAEBV infection. Further research is needed to explore the role of CAEBV as a triggering and disease-modifying factor in ALPS.
FRONTIERS IN PEDIATRICS
(2021)
Article
Medicine, General & Internal
Masayo Yamamoto, Motohiro Shindo, Takuya Funayama, Chihiro Sumi, Takeshi Saito, Yasumichi Toki, Mayumi Hatayama, Ken-Ichi Imadome, Yusuke Mizukami, Toshikatsu Okumura
Summary: This article reports a case of late-onset EBV-related HLH after CBT for CAEBV. Late-onset EBV-related PTLD may be life-threatening.
Article
Infectious Diseases
Yue Song, Jingshi Wang, Yini Wang, Lin Wu, Yahong You, Deli Song, Leilei Chen, Gaungqiang Meng, Xiaodan He, Tingting Cui, Xiaodi Wang, Qingxia Yin, Zhao Wang
Summary: In this study, the efficacy and safety of PD-1 blockade combined with lenalidomide in CAEBV patients were investigated. The results showed that this treatment approach could significantly reduce EBV-DNA copies, increase the proportion of CD8+T cells, and activate different cytokines. The survival probability at 1 year was estimated to be 91.3%.
CLINICAL MICROBIOLOGY AND INFECTION
(2023)
Article
Infectious Diseases
David B. Villafuerte, Olga Lavrynenko, Rayan Qazi, Marco F. Passeri, Fernando L. Sanchez
Summary: A 60-year-old Hispanic female was admitted to the hospital with recurrent fevers, altered mental status, lymphadenopathy, hepatosplenomegaly, and pancytopenia. Extensive workup revealed chronic active Epstein-Barr virus infection. Treatment initially showed positive results, but the patient became co-infected with COVID-19, leading to a rapid decline in health and death.
INTERNATIONAL JOURNAL OF INFECTIOUS DISEASES
(2022)
Article
Immunology
Yaxian Ma, Peiling Zhang, Yuhan Bao, Hui Luo, Jiachen Wang, Liang Huang, Miao Zheng
Summary: Chronic active Epstein-Barr virus (CAEBV) disease is a high-mortality disease characterized by persistent infectious mononucleosis-like symptoms. This study analyzed the outcomes of PD-1 inhibitors treatment for CAEBV patients and found that the treatment had a high response rate and tolerable toxicity.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Kyoichi Obata, Tatsuo Okui, Sawako Ono, Koki Umemori, Shoji Ryumon, Kisho Ono, Mayumi Yao, Norie Yoshioka, Soichiro Ibaragi, Akira Sasaki
Summary: MTX-LPD or EBV-MCU are diseases related to the use of MTX, mainly associated with EBV infection. A retrospective study found that the oral mucosa is the most common site of onset, and all cases achieved remission. The most common risk factor for developing EBV-MCU is the use of immunosuppressive drugs.
Article
Medicine, General & Internal
Austin Feindt, Montserrat Lara-Velazquez, Ahmad Alkhasawneh, Dinesh Rao, Raafat Makary, Keith Dombrowski, Daryoush Tavanaiepour, Gazanfar Rahmathulla
Summary: This case report presents a 72-year-old female patient who had undergone renal transplant 11 years ago and developed progressively worsening headaches and confusion. Imaging revealed brain edema in the frontal and temporal lobes. Surgical biopsy confirmed a diagnosis of Polymorphic PTLD. Early diagnosis is crucial for patient survival with PTLD.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Infectious Diseases
Julian Lindsay, Jad Othman, Madeleine R. Heldman, Monica A. Slavin
Summary: Management of EBV PTLD involves complex measures such as risk stratification, preemptive therapy, and balancing treatment options. Experimental therapies like donor-derived multipathogen and EBV specific CTLs show promise in prevention and treatment of EBV PTLD.
CURRENT OPINION IN INFECTIOUS DISEASES
(2021)
Article
Hematology
Cristina Venturini, Charlotte J. Houldcroft, Arina Lazareva, Fanny Wegner, Sofia Morfopoulou, Persis J. Amrolia, Zainab Golwala, Anupama Rao, Stephen D. Marks, Jacob Simmonds, Tetsushi Yoshikawa, Paul J. Farrell, Jeffrey Cohen, Austen J. Worth, Judith Breuer
Summary: CAEBV is a rare disease characterized by persistent EBV infection, with defective EBV genomes found in East Asian patients. In this study, EBV deletions were observed in blood samples from CAEBV patients but not in saliva samples, and these deletions were lost over time after successful treatment. This suggests that CAEBV is associated with the evolution and persistence of EBV+ haematological clones that are lost on successful treatment.
BRITISH JOURNAL OF HAEMATOLOGY
(2021)
Article
Immunology
Guillaume Vial, Laure Barthod, Sophie Schneider, Patrick Mercie, Pierre Duffau, Agathe Vermorel, Emmanuel Ribeiro
Summary: This article presents a case of a 33-year-old patient with multisystem CAEBV, with notable ophthalmological and renal damage caused by the infection.
OPEN FORUM INFECTIOUS DISEASES
(2022)
Article
Genetics & Heredity
Ruyue Chen, Qiang Lin, Yun Zhu, Yunyan Shen, Qinying Xu, Hanyun Tang, Ningxun Cui, Lu Jiang, Xiaomei Dai, Weiqing Chen, Xiaozhong Li
Summary: The article reports successful cases of using PD-1 inhibitor Sintilimab to treat CAEBV and EBV-HLH. Among the three patients, two had CAEBV and one had EBV-HLH. After treatment, the clinical symptoms of the patients improved significantly, and the EBV-DNA load decreased significantly. The study supports the efficacy of PD-1 targeted therapy in certain patients with CAEBV and EBV-HLH and suggests that Sintilimab may provide a cure without HSCT.
ORPHANET JOURNAL OF RARE DISEASES
(2023)
Article
Immunology
Ang Wei, Wenxin Ou, Yunze Zhao, Honghao Ma, Liping Zhang, Hongyun Lian, Xiaoxi Zhao, Qing Zhang, Dong Wang, Zhigang Li, Tianyou Wang, Rui Zhang
Summary: Different subtypes in chronic active Epstein-Barr virus infection have specific clinical features. Patients with CD8+ T-cell type have a poor prognosis. The study found that patients with CD8+ T-cell type were more likely to have a rash, while patients with CD4+ T-cell type were more likely to develop hepatomegaly.
JOURNAL OF INFECTIOUS DISEASES
(2023)
Article
Clinical Neurology
Daisuke Sato, Hirokazu Takami, Shunsaku Takayanagi, Kazuki Taoka, Mariko Tanaka, Reiko Matsuura, Shota Tanaka, Nobuhito Saito
Summary: This is the first report documenting the development of other iatrogenic immunodeficiency-associated lymphoproliferative disorder (OIIA-LPD) during temozolomide therapy. Timely diagnosis of the disease and discontinuation of temozolomide were considered to be the preferred management. Close monitoring for relapse should be continued.
Review
Immunology
Shanfen Shi, Liangda Li, Cuiping Pan, Yandi Yang, Gun Chen, Yongping He
Summary: This article describes a rare case of chronic active Epstein-Barr virus infection-associated generalized myositis with extranodal NK/T-cell lymphoma. It is different from polymyositis and is prone to developing poor prognosis lymphoma.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Microbiology
Ayako Arai
Summary: Chronic active Epstein-Barr virus infection (CAEBV) is a disease characterized by the clonal proliferation of EBV-infected T or NK cells, leading to systemic inflammation and multiple organ failures. The only effective treatment currently available is allogeneic stem cell transplantation, and research on the disease is advancing.