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https://www.readbyqxmd.com/read/27904117/successful-cord-blood-stem-cell-transplantation-for-an-adult-case-of-chronic-active-epstein-barr-virus-infection
#1
Masuho Saburi, Masao Ogata, Takako Satou, Natsumi Yoshida, Kentaro Nagamatsu, Yuko Nashimoto, Yui Moroga, Kuniko Takano, Kazuhiro Kohno, Kuniaki Shirao
A 41-year-old man was referred to our hospital for treatment of anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma. Chronic active Epstein-Barr virus (CAEBV) was diagnosed based on the findings of elevated EBV antibody titers and positive EBV-DNA in the peripheral blood, and cord blood stem cell transplantation (CBT) was performed. The EBV-DNA levels in the blood fell below the limit of detection. His lymphoma relapsed on Day 165 with the appearance of eruptions, which disappeared after the withdrawal of tacrolimus...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27795539/epstein-barr-virus-infection-and-related-hematological-diseases
#2
Akihisa Sawada
Once the Epstein-Barr virus (EBV) has infected a person, it then latently infects B cells. This latent infection lasts a lifetime. However, EBV can infect T or NK cells (T/NK cells) in rare cases. Therefore, EBV causes various hematological diseases. Among these diseases, CAEBV is regarded as the most problematic because, although it is not particularly uncommon, the diagnostic tests for this disease are not covered by health insurance, a serious illness in the "non-active" periods is lacking, and the appropriate motivation for early initiation of treatment can easily be lost...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27495050/early-diagnosis-and-follow-up-of-chronic-active-epstein-barr-virus-associated-cardiovascular-complications-with-cardiovascular-magnetic-resonance-imaging-a-case-report
#3
Shu Jiang, Xiao Li, Jian Cao, Di Wu, Lingyan Kong, Lu Lin, Zhengyu Jin, Jing An, Yining Wang
INTRODUCTION: Chronic active Epstein-Barr virus (EBV) infection (CAEBV) is characterized as chronic or recurrent mononucleosis-like symptoms and elevated EBV deoxyribonucleic acid (EBV-DNA) copies. Cardiovascular complications have high morbidity and mortality. The treatment regimen for CAEBV has not been established yet, resulting in poor prognoses. Herein, we present a case of cardiovascular magnetic resonance imaging (CMRI) evaluation with a series of sequences for CAEBV-associated cardiovascular involvement, which has never been reported...
August 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27263789/chronic-active-epstein-barr-virus-infection-with-marked-pericardial-effusion-successfully-treated-with-allogeneic-peripheral-blood-stem-cell-transplantation
#4
Shinichiro Matsui, Yusuke Takeda, Yusuke Isshiki, Atsuko Yamazaki, Sanshiro Nakao, Koji Takaishi, Yuhei Nagao, Nagisa Hasegawa, Emi Togasaki, Ryoh Shimizu, Chika Kawajiri, Shio Sakai, Naoya Mimura, Masahiro Takeuchi, Chikako Ohwada, Emiko Sakaida, Tohru Iseki, Ken-Ichi Imadome, Chiaki Nakaseko
A 23-year-old woman presented with a persistent fever and shortness of breath. Computed tomography showed marked pericardial effusion, hepatosplenomegaly, and cervical and mediastinal lymph node swelling. Epstein-Barr virus (EBV) antibody titers were abnormally elevated, and the copy number of EBV-DNA was increased in peripheral blood. Based on these observations, she was diagnosed with chronic active EBV infection (CAEBV). The EBV-infected cells in her peripheral blood were CD4(+)T lymphocytes. Fever and pericardial effusion improved following treatment with a combination of prednisolone, etoposide, and cyclosporine; however, peripheral blood EBV-DNA levels remained high...
May 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27256608/-an-analysis-of-immunophenotyping-of-peripheral-lymphocytes-in-adult-patients-with-infectious-mononucleosis-and-chronic-active-epstein-barr-virus-infection
#5
J Xie, H L Wang, Z F Qiu, T S Li
OBJECTIVE: To determine the immunophenotypic features of peripheral lymphocytes in adult patients with Epstein-Barr virus(EBV)-associated infectious mononucleosis(IM) and chronic active EBV infection (CAEBV). METHODS: Eighteen IM patients, 12 CAEBV patients and 18 healthy donors were included. Lymphocyte subsets including CD3(-)CD19(+) B cells, CD3(-)CD16/56(+) NK cells, CD4(+) and CD8(+) T cells in peripheral blood were measured by flow cytometry. The expression of activation markers (HLA-DR and CD38) on CD8(+) T cells and CD28 expression on T cells were also determined...
June 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27157296/clinicopathological-categorization-of-epstein-barr-virus-positive-t-nk-cell-lymphoproliferative-disease-an-analysis-of-42-cases-with-an-emphasis-on-prognostic-implications
#6
Jin Ho Paik, Ji-Young Choe, Hyojin Kim, Jeong-Ok Lee, Hyoung Jin Kang, Hee Young Shin, Dong Soon Lee, Dae Seog Heo, Chul-Woo Kim, Kwang-Hyun Cho, Tae Min Kim, Yoon Kyung Jeon
Epstein-Barr virus-positive T/NK-cell lymphoproliferative diseases (EBV-T/NK-LPDs) include several overlapping EBV-related conditions with variably aggressive courses. For prognostic categorization, we retrospectively analyzed 42 EBV-T/NK-LPD cases. Male (79% [33/42]), young (≤40 years; 83% [35/42]) patients and T-cell lineage (81% [34/42]; CD8/CD4 = 1.8) were predominant. Clinicopathologically, three systemic and one cutaneous category were developed: hemophagocytic lymphohistiocytosis (HLH; 26% [11/42]), chronic active EBV infection (CAEBV; 31% [13/42]), systemic unclassifiable disease (24% [10/42]), and hydroa vacciniforme/hydroa vacciniforme-like lymphoma (HV/HVL; 19% [8/42])...
May 9, 2016: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/26952849/chronic-active-ebv-infection-with-features-of-granulomatosis-with-polyangiitis
#7
Masaki Shimomura, Hideaki Morishita, Takaaki Meguro, Shiro Seto, Mitsuaki Kimura, Minoru Hamazaki, Ayako Hashimoto, Yuka Sugiyama, Hiroshi Kimura
Herein, we report the case of a 13-year-old boy with multiple recurrent ulcers on his legs. He developed severe sinusitis at 10 years of age and had significant weight loss (6 kg) in the 2 months prior to admission. Histology of tissue biopsied from the ulcer indicated small vessel vasculitis and granulomatous inflammation. Given that these findings met the diagnostic criteria for granulomatosis with polyangiitis (GPA), he was treated with immunosuppressive agents. Further pathology, however, indicated Epstein-Barr virus (EBV)-encoded RNA (EBER) in most lymphocytes in the same sample...
July 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/26844502/epstein-barr-virus-positive-t-nk-cell-lymphoproliferative-disorders-manifested-as-gastrointestinal-perforations-and-skin-lesions-a-case-report
#8
Hai-Juan Xiao, Ji Li, Hong-Mei Song, Zheng-Hong Li, Mei Dong, Xiao-Ge Zhou
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders (LPDs) of childhood is a highly aggressive EBV-positive T/natural killer (NK)-cell LPD, which emerges in the background of chronic active EBV infection (CAEBV) or shortly after primary acute EBV infection. The clinical presentations of CAEBV are varied; patients with atypical manifestations are easily misdiagnosed. We described a 14-year-old boy suffering from digestive disorders and intermittent fever for 1 year and 9 months, whose conditions worsened and skin lesions occurred 2 months before hospitalization...
February 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/26809631/-a-case-of-severe-chronic-active-epstein-barr-virus-infection-with-aplastic-anemia-and-hepatitis
#9
Ja In Lee, Sung Won Lee, Nam Ik Han, Sang Mi Ro, Yong-Sun Noh, Jeong Won Jang, Si Hyun Bae, Jong Young Choi, Seung Kew Yoon
Epstein-Barr virus (EBV) causes various acute and chronic diseases. Chronic active EBV infection (CAEBV) is characterized by infectious mononucleosis-like symptoms that persist for more than 6 months with high viral loads in peripheral blood and/or an unusual pattern of anti-EBV antibodies. Severe CAEBV is associated with poor prognosis with severe symptoms, an extremely high EBV-related antibody titer, and hematologic complications that often include hemophagocytic lymphohistiocytosis. However, CAEBV which led to the development of aplastic anemia (AA) has not been reported yet...
January 25, 2016: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/26712582/diagnostic-values-for-the-viral-load-in-peripheral-blood-mononuclear-cells-of-patients-with-chronic-active-epstein-barr-virus-disease
#10
Yoshinori Ito, Michio Suzuki, Jun-ichi Kawada, Hiroshi Kimura
Chronic active Epstein-Barr virus disease (CAEBV) is a distinct EBV-associated lymphoproliferative disease with a poor prognosis. Although the viral load in blood samples has been widely used for diagnosing CAEBV, well-defined viral load thresholds to guide clinicians are currently lacking. The aim of the present study was to determine standardized diagnostic values for EBV load in blood samples of CAEBV patients using the World Health Organization international standard for reporting. Levels of EBV DNA in 103 peripheral blood mononuclear cells (PBMCs) and 95 plasma/serum samples from 107 cases with CAEBV were quantified and expressed in international units...
April 2016: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/26148205/chronic-active-epstein-barr-virus-infection-with-cutaneous-and-sinus-lymphoproliferation-in-a-white-female-patient-with-25%C3%A2-years-follow-up-an-original-case-report
#11
C Picard, S Gouarin, F Comoz, M Barreau, L Verneuil, X Troussard, A Dompmartin
Chronic active Epstein-Barr virus infection (CAEBV) is characterized by chronic infectious mononucleosis-like symptoms associated with very high viral load, as assessed by quantitative polymerase chain reaction. We present an unusual case in a French woman who was followed up over 25 years with cutaneous and sinus lymphoproliferation. This white woman presented with a long history of recurrent cutaneous necrotic papules of the skin, which started during childhood and healed spontaneously with depressed scars...
November 2015: British Journal of Dermatology
https://www.readbyqxmd.com/read/25947952/late-onset-severe-chronic-active-ebv-in-a-patient-for-five-years-with-mutations-in-stxbp2-munc18-2-and-prf1-perforin-1
#12
Jeffrey I Cohen, Julie E Niemela, Jennifer L Stoddard, Stefania Pittaluga, Helen Heslop, Elaine S Jaffe, Kennichi Dowdell
Severe chronic active Epstein-Barr virus (CAEBV) disease is defined as a severe progressive illness lasting 6 months or longer with infiltration of tissues with EBV-positive lymphocytes, markedly elevated levels of EBV DNA in the blood, and no known immunodeficiency such as HIV. These patients usually have fever, splenomegaly, lymphadenopathy, and may have markedly elevated EBV antibody titers to viral capsid antigen. Although the cause of most cases of severe CAEBV is unknown, one well-documented case was associated with compound heterozygous mutations in PRF1 (perforin 1)...
July 2015: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/25938379/recent-concise-viewpoints-of-chronic-active-epstein-barr-virus-infection
#13
REVIEW
Motohiko Okano
Chronic active Epstein-Barr virus infection (CAEBV) is characterized mainly by prolonged or intermittent fever, lymphadenopathy and hepatosplenomegaly without definite underlying diseases at the diagnosis. Patients with CAEBV also may have various life-threatening conditions including hematological, neurological, pulmonary, cardiac, digestive tract, ocular and/or dermal disorders. Additionally, during the course of illness, they often develop hematological malignancies such as T cell, NK cell or B cell lymphoproliferative disorder (LPD) and/or lymphoma...
2015: Current Pediatric Reviews
https://www.readbyqxmd.com/read/25809637/pulmonary-arterial-hypertension-associated-with-chronic-active-epstein-barr-virus-infection
#14
Yutaka Fukuda, Nobuo Momoi, Mitsuko Akaihata, Katsutoshi Nagasawa, Masaki Mitomo, Yoshimichi Aoyagi, Kisei Endoh, Mitsuaki Hosoya
Chronic active Epstein-Barr virus (EBV) infection (CAEBV), characterized by persistent infectious mononucleosis-like symptoms, can lead to cardiovascular complications including coronary artery aneurysm or myocarditis. Here, we present the case of an 11-year-old boy with pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia associated with CAEBV. The patient did not have any major symptoms attributed to CAEBV, such as fever, lymphadenopathy or splenomegaly when the PAH developed. Mild liver dysfunction was found at the first examination, and it persisted...
August 2015: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/25553464/dehydroxymethylepoxyquinomicin-selectively-ablates-t-caebv-cells
#15
Hui Zhang, Wen-Tao Yang, Zhao Wang, Chun-Mei Yao, Xiao-Fang Wang, Zhi-Qing Tian, Ying-Ying Jin, Lin-Lin Wang, Tong-Xin Chen
Chronic active Epstein-Barr virus infection (CAEBV) represents a new subtype of lymphoproliferative disorders characterized by high morbidity and mortality rates and often leads to malignant transformation of infected cells. Efficient therapeutic strategies are presently unavailable; therefore, the development of therapies to prevent CAEBV-mediated transformation and disease progression is crucial. Here, we used microarray analysis and luciferase reporter assays to reveal the potential role of activated nuclear factor kappa B (NF-kB) in T cell type of-CAEBV infection...
2015: Frontiers in Bioscience (Landmark Edition)
https://www.readbyqxmd.com/read/25342015/-recurrence-of-chronic-active-epstein-barr-virus-infection-presenting-with-myelopathy-after-umbilical-cord-blood-transplantation
#16
Shohei Watanabe, Masaya Okada, Tazuko Tokugawa, Akihiro Sawada, Hiroyasu Ogawa, Hiroo Yoshikawa
A 38-year-old man was admitted to our hospital with neck pain, dysesthesia of both hands, and weakness of the left upper limb. He had been diagnosed with a chronic active Epstein-Barr virus infection (CAEBV) at the age of 34 and had undergone umbilical cord blood transplantation at the age of 37. MRI of the spinal cord revealed an intramedullary hyperintense lesion on T₂-weighted images with gadolinium enhancement. Because his laboratory tests revealed proliferation of CD19(+) lymphocytes in the peripheral blood, and EBV DNA was detected in both peripheral blood and CSF, he was diagnosed as having post-transplant EBV associated lymphoproliferative disease...
2014: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/25197370/clinicopathological-characteristics-of-four-cases-of-ebv-positive-t-cell-lymphoproliferative-disorders-of-childhood-in-china
#17
Wenting Huang, Ning Lv, Jianming Ying, Tian Qiu, Xiaoli Feng
A new category, "EBV positive T-cell lymphoproliferative disorders (LPD) of childhood", was proposed in the 2008 World Health Organization's (WHO) classifications of lymphoma. This series of lymphoproliferative disorders is rare. There are two major types of this series of disorders: systemic EBV positive T-cell LPD of childhood and hydroa vacciniforme-like lymphoma (HVLL). In this study, we describe the distinct features of four cases of EBV positive T-cell LPD of childhood in China. Two were systemic EBV positive T-cell LPD of childhood, one was HVLL and one was chronic active EBV (CAEBV)...
2014: International Journal of Clinical and Experimental Pathology
https://www.readbyqxmd.com/read/25109697/epstein-barr-virus-associated-t-cell-lymphoproliferative-disorder-affecting-skin-and-lung-in-an-elderly-patient
#18
Tomomi Hoshino, Kazuki Tatsuno, Takatoshi Shimauchi, Satoko Okada, Taisuke Ito, Takaaki Ono, Koichi Ohshima, Yoshiki Tokura
A 70-year-old man presented with papular skin lesions and was diagnosed with Epstein-Barr virus (EBV)-associated T-cell lymphoproliferative disorder (T-LPD). The patient showed infiltration of a large number of EBV-encoded RNA-positive T cells in the skin and lung, presence of EBV load in the peripheral blood, and expansion of clonal EBV-infected γδ T cells and CD8(+) T cells in the blood and skin, as assessed by EBV-terminal repeat Southern blot, T-cell receptor polymerase chain reaction and flow cytometric analyses...
September 2014: Journal of Dermatology
https://www.readbyqxmd.com/read/24988166/-epstein-barr-virus-infection-with-severe-consequences-ebv-haemophagocytic-lymphohistiocytosis-and-hodgkin-lymphoma-with-down-syndrome
#19
Pieter C J Kuitert, Floor C H Abbink, Chantal J M Broers, Paul van der Valk, A Marceline van Furth, Martijn van der Kuip
BACKGROUND: Over 90% of the population is infected with the Epstein-Barr virus (EBV). Following primary infection, the virus remains latent in B-lymphocytes. In isolated cases, especially in immunocompromised patients, the Epstein-Barr virus can result in a chronic active infection (CAEBV). CASE DESCRIPTION: We describe an 11-year-old boy with Down syndrome who was admitted because of fever of unknown origin during several periods. Serological findings (high VCA-IgG and absent EBNA-IgG) were suggestive of CAEBV, which was confirmed by the high circulating EBV viral load...
2014: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/24966953/spectrum-of-epstein-barr-virus-associated-t-cell-lymphoproliferative-disorder-in-adolescents-and-young-adults-in-taiwan
#20
Ren-Ching Wang, Sheng-Tsung Chang, Yen-Chuan Hsieh, Wan-Ting Huang, Jeng-Dong Hsu, Chih-En Tseng, Ming-Chung Wang, Wei-Shou Hwang, John Wang, Shih-Sung Chuang
Epstein-Barr Virus (EBV) is a herpesvirus usually infecting B-cells but may occasionally infect T- or natural killer (NK)-cells. EBV-associated T- or NK-cell lymphoproliferations represent a continuous spectrum of diseases ranging from asymptomatic infection, infectious mononucleosis (IM), to clonal and malignant lymphoproliferations including systemic EBV-positive T/NK-cell lymphoproliferative disease (EBV-T/NK-LPD) of childhood and hydroa-vacciniforme-like lymphoma of the skin. The clonal diseases are more prevalent in East Asia and exhibit overlapping clinical and pathological features with chronic active EBV infection...
2014: International Journal of Clinical and Experimental Pathology
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