Dayharsh et al reported an increased risk of epsteinbarr virus ebvpositive lymphoma in adults with ibd treated with azathioprine or 6mercaptopurine. Epsteinbarr virus latent membrane protein1 oncogene. The pathology of posttransplant lymphoproliferative disorders occurring in the setting of cyclosporine aprednisone immunosuppression. As a group, ebvtnklpd are more commonly encountered in asians and native americans from central and south america compared to western populations. The incidence of epsteinbarr virus ebvassociated lymphoproliferative disorders has increased with greater use of immunomodulatory therapies. Epsteinbarr virusassociated lymphomas sciencedirect.
Adjunct aprof amanda oakley, dermatologist, hamilton, new zealand. Epsteinbarr virus associated b cell lymphoproliferative disorders following bone marrow transplantation by ralph s. Epsteinbarr virusassociated lymphoproliferative disorder. Primary ebv infection occurs most often in childhood and is generally asymptomatic. Primary infection with epsteinbarr virus ebv is usually asymptomatic and, in a normal host, ebv remains latent in b cells after primary infection for the remainder of life. Epsteinbarr virusassociated lymphoproliferative disorders. Clinicopathologic manifestations of epsteinbarr virus. With regard to lymphocytes, latent ebv infection is linked to development of heterogen. Epsteinbarr virusrelated late membrane protein 1, and epsteinbarr virusencoded rna, and were negative for igg4. Epsteinbarr virus associated b cell lymphoproliferative disorders following bone marrow transplantation. Barr virus ebv positive b cell lymphoproliferative disease lpd while. Epsteinbarr virusassociated tnatural killercell lymphoproliferative disorders sanghui park,1 young h.
In some of these diseasessuch as ebvpositive diffuse large bcell lymphoma dlbcl, not otherwise specifiedvirus detection is required for the diagnosis, while in others its detection is not necessary for diagnosis. This patient was described previously by oono et al. How i treat ebv lymphoproliferation blood american society of. Ebv was first identified as an oncogenic virus in a burkitt lymphoma cell line, though subsequently has been found to drive a variety of malignancies, including diffuse large b. Most persons have a chronic asymptomatic infection with ebv. Figure 3 from pathogenic link between hydroa vacciniforme. A major concern is that ebv can cause a diverse range of neoplasms and autoimmune diseases. Infusion of donor leukocytes to treat epsteinbarr virusassociated lymphoproliferative disorders after allogenic bone marrow transplantation new engl. Epsteinbarr virusassociated lymphoproliferative disorder i. Epsteinbarr virus ebvassociated lymphoproliferative disorder ebvlpd following bone marrow transplantation can be fatal. Epsteinbarr virus, a ubiquitous human herpesvirus, can induce both lytic and latent infections that result in a variety of human diseases, including lymphoproliferative disorders.
Epsteinbarr virus ebv is a member of the human herpesvirus family that was initially isolated from a cultured. Epsteinbarr virusassociated syndromes in immunosuppressed liver transplant recipients. Pdf the management of epsteinbarr virus associated post. Epsteinbarr virusassociated lymphoproliferative disorders author. Pdf epsteinbarr virusassociated bcell lymphoproliferative. The patient died of epsteinbarr virusassociated malignant lymphoma with hemophagocytic syndrome at the age of 18 years.
Epsteinbarr virus latent membrane protein1 oncogene deletions. There are several forms of epsteinbarr virus ebv infection. Lymphomatoid granulomatosis lyg or lg is a very rare lymphoproliferative disorder first characterized in 1972. To elucidate clinicopathologic manifesta tions of cutaneous lymphoproliferative disorders asso ciated with epsteinbarr virus ebv infection. Robertson published september 15, 2001 citation information. Some categories of primary immunodeficiency diseases pids including xlinked lymphoproliferative syndrome xlp, are characterized by susceptibility and vulnerability to ebv infection. Ebv was first identified as an oncogenic virus in a burkitt lymphoma cell line, though subsequently has been found to drive a variety of malignancies, including diffuse large bcell lymphoma dlbcl and othe. Despite a growing understanding of the pathogenesis and spectrum of epsteinbarr virus ebv and ebv. Epsteinbarr viruspositive tnkcell lymphoproliferative. Pdf epsteinbarr virus ebv load and interleukin10 in ebv.
A manifestation of epsteinbarr virusassociated t lymphoproliferative disorder mineui hong young hyeh ko1 department of pathology, hallym university kangnam sacred heart hospital, ease are unknown. In some of these diseasessuch as ebvpositive diffuse large bcell lymphoma dlbcl, not otherwise specifiedvirus detection is required for the diagnosis. Opportunities to target the life cycle of epsteinbarr. Epstein barr virus ebv is associated with a variety of lymphoproliferative disorders lpds and other malignancies, including nasopharyngeal carcinoma, hodgkin disease, and burkitt lymphoma. Herein, we report a rare case of the coincidence of leio. Kingma dw, weiss wb, jaffe es, kumar s, frekko k, raffeld m 1996 epsteinbarr virus latent membrane protein1 oncogene deletions. Diagnosis and treatment of epsteinbarr virusassociated. Ebv was first identified as an oncogenic virus in a.
A case of epsteinbarr virusassociated leiomyosarcoma. Epsteinbarr virusassociated lymphoproliferative disease. Epsteinbarr virus ebv exhibits tropism for both lymphocytes and epithelial cells and can induce both replicative productivelytic and latent persistent infections that result in a variety of human diseases. The frequency depends on the degree of immunosuppression and the specific organ transplanted, but can be as high as 39% in heart or lung transplant patients 2. Review and update on 2016 who classification june 2017 journal of pathology and translational medicine 514. Comprehensive molecular diagnosis of epsteinbarr virus. Epstein barr virus associated diseases in allogeneic. Based on the diagnosis of both epsteinbarr virusassociated bcell lymphoproliferative disorder and igg4related disease, the patient received eight cycles of rituximab combined with cyclophosphamide and prednisolone.
Pdf epsteinbarr virus ebv is a ubiquitous herpesvirus, affecting up to 90% of the population. Despite a growing understanding of the pathogenesis and spectrum of epsteinbarr virus ebv and ebvassociated posttransplant lymphoproliferative disease ptld in organ transplant recipients, the optimal management of this complication. The ebv genome contained in this episomal form is thought to replicate utiliz ing host dna polymerases, since, in this form, the virus is resistant to antiviral agents which inhibit viral dna polymerases 176. Activity of the ebna1 promoter associated with lytic. The 2016 revised world health organization classification recognizes the following ebvpositive lymphoproliferative disorders lpd. Bcell lymphomas predominate among the ebvrelated lymphoproliferative disorders. Ebvassociated t and nkcell lymphoproliferative diseases ebvtnk lpds are characterized by the transformation and proliferation of ebvinfected t or nk cells. Frontiers epsteinbarr virusassociated t and nkcell. Epstein barr virus associated bcell lymphomas and iatrogenic. Pmc free article randhawa ps, markin rs, starzl te, demetris aj. Multiple methods, pedigree analysis, clinical evaluation, and epsteinbarr virus ebvspecific serology, ebv dna hybridization of tissues to probe for viral genome, staining of touch imprints for ebv nuclearassociated antigen, establishment of spontaneous infected bcell lines from peripheral blood or tissues, examination of peripheral blood smears, and hematopathology studies, were used to. Endobronchial epsteinbarr virus associated posttransplant. To maintain the integrity of the viral genome and to get out of the control of the host immune system, in the phase of the latent infection ebv shows the expression of several genes, including genes for six nuclear antigens, three latent membrane proteins, two short.
Over 90% of humans are infected with the epsteinbarr virus ebv and the infection persists for life. In addition, patients undergoing hematopoietic stem cell transplantation or solid organ transplantation can experience post. Epsteinbarr virus ebv lymphoproliferative disease lpd is the result of the outgrowth of ebvinfected b cells that would. The epsteinbarr virus, which infects 90% of the world population, is also a common cause of these disorders, being responsible for a wide range of nonmalignant, premalignant, and malignant epsteinbarr virusassociated lymphoproliferative diseases. Epsteinbarr virus ebv is a ubiquitous herpesvirus, affecting up to 90% of the population.
Lymphomatoid means lymphomalike and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis within it lg most commonly affects middle aged people, but has occasionally been observed in. British journal of haematology, 2003, 122, 927933 epsteinbarr virus ebv load and interleukin10 in ebvpositive and ebvnegative posttransplant lymphoproliferative disorders giuliana muti, 1 catherine klersy, 2 fausto baldanti, 3 simonetta granata, 4 pierluigi oreste, 5 laura pezzetti, 1 marta gatti, 3 livio gargantini, 1 marianna caramella, 1 valentina mancini, 1 giuseppe gerna 3 and. Are natural killer cells the key to treating epsteinbarr. Some categories of primary immunodeficiency diseases pids including xlinked lymphoproliferative syndrome xlp, are characterized by susceptibility and vulnerability to ebv. Adoptive immunotherapy for epsteinbarr virusassociated. Jci are natural killer cells the key to treating epstein. Epsteinbarr virusassociated lymphoproliferative disorders hum pathol, 38 2007, pp. Epsteinbarr virus associated cns lymphoproliferative. Pdf epstein barr virus associated bcell lymphomas and. Latent state maintained by expression of viral transcripts and proteins. It is also associated with various nonmalignant, premalignant, and. The major risk factors for the development of ebvlpd are ex vivo tcell depletion or in vivo tcell depletion with either antithymocyte globulin atg or monoclonal antitcell antibodies.
Epsteinbarr virusassociated lymphoproliferative disease in non. Epsteinbarrvirusassociated lymphoproliferative disease. Cd30positive tcell lymphoproliferative disease of the oral mucosa in children. Risk of lymphoproliferative disorders after bone marrow transplantation. Lymphoproliferative disorders in autoimmune diseases in japan. Epsteinbarr virusassociated lymphoproliferative disorders have been frequently reported as a complication of solid organ and allogeneic bone marrow transplantation. Epsteinbarrvirusassociated lymphoproliferative disease of the lung. Pdf documentation of epsteinbarr virus infection in. Are natural killer cells the key to treating epsteinbarr virusassociated lymphoproliferative disorders. Webber2, lymphoproliferative disorders in pediatric solidorgan transplant david rowe5 and jorge reyes3,4 recipients. Ebvassociated reactive lymphoid proliferations are a set of disorders in which b cells or nkt cells proliferate as an apparent reaction to ebv infection.
Clinicopathologic implications of epsteinbarr virus related. A new therapy in epsteinbarr virusassociated lymphoproliferative. Epsteinbarr virusassociated bcell lymphoproliferative disorders. Favorable outcome of epsteinbarr virusassociated bcell. Epsteinbarr virusassociated tnatural killercell lymphoproliferative disorders. Apr 01, 2015 the epsteinbarr virus ebv is the first identified human virus with a proven association with the pathogenesis of cancer. Ebv is a doublestranded dna virus it is best known as the cause of infectious mononucleosis mono or glandular fever.
Endobronchial epsteinbarr virus associated posttransplant lymphoproliferative disorder in hematopoietic stem cell transplantation s. A lymphoproliferative disorder associated with epsteinbarr virus. Pdf epsteinbarr virusassociated lymphoproliferative. The epsteinbarr virus ebv has been shown to infect t lymphocytes and is associated with two recently recognized human t lymphoproliferative disorders. Jun 01, 2017 epsteinbarr virusassociated lymphoproliferative disorders hum pathol, 38 2007, pp. After primary infection, ebv maintains a lifelong latent infection. Dr selene ting, medical registrar, middlemore hospital, auckland, new zealand. Spontaneous regression of lymphoproliferative disorders in patients treated with methotrexate for rheumatoid arthritis and other rheumatic. Cutaneous lymphoproliferative disorders associated with. Frontiers transcriptomic abnormalities in epstein barr. Epsteinbarr virus ebv is associated with several lifethreatening diseases, such as lymphoproliferative disease lpd, particularly in immunocompromised hosts. Between march 1999 and january 2001, a total of 23 transplants with atg of. Epsteinbarr virus infection might also induce leiomyosarcoma, which can be diagnosed based on the detection of epsteinbarr virusencoded small rna eber in tumor tissue.
Bcell lymphomas predominate among the ebv related lymphoproliferative disorders. Ko2 1department of pathology, ewha womans university school of medicine, and 2department of pathology, samsung medical center, sungkyunkwan university school of medicine, seoul, korea abstract. Durandy a, le deist f, emile jf, debatin k, fischer a. In some of these diseasessuch as ebv positive diffuse large bcell lymphoma dlbcl, not otherwise specified virus detection is required for the diagnosis. These include asymptomatic infections, the primary infection, infectious mononucleosis, and the progression of asymptomatic or primary infections to. Analysis of clinicopathological features and epsteinbarr virus infection int j cancer 108. Shapiro, kenneth mcclain, glauco frizzera, kazimiera j. Epsteinbarr virusassociated malignant lymphoproliferative. Bone body has been shown to be effective in the treatment of marrow transplant 1995.
Sep 01, 2007 presence of epsteinbarr virus latency type iii at the single cell level in posttransplantation lymphoproliferative disorders and aids related lymphomas j clin pathol, 50 1997, pp. Ebv positive lymphoproliferative disorders are heterogeneous, with variable clinical features and prognoses depending on the context in which. Hematopathology epsteinbarr virus and bcell proliferation in tcell nonhodgkin lymphoma. In adolescence, it is associated with a selflimiting infectious mononucleosis.
This category includes, but is not limited to, burkitt lymphoma, classic hodgkin lymphoma, and lymphomas arising in immunocompromised individuals. Advances in understanding the pathogenesis of epsteinbarr virusassociated lymphoproliferative disorders. Infusions of donor leukocytes to treat epsteinbarr virusassociated lymphoproliferative disorders after allogeneic bone marrow transplantation. The epsteinbarr virus ebv has been shown to infect t lymphocytes and is associated with two recently recognized human tlymphoproliferative disorder s. Epsteinbarr virusassociated lymphoproliferative disorder the new development of an antib cell monoclonal anti following autografting for chronic myeloid leukemia. However, in some populations, the epstein barr virus ebv has been involved in the occurrence of a wide range of bcell lymphoproliferative disorders lpds. Chronic active epsteinbarr virus infection caebv is a systemic ebvpositive lymphoproliferative disorder ebvlpd, which is marked by. Epsteinbarr virusassociated lymphoproliferative diseases. Preemptive therapy with rituximab for prevention of. Advances in understanding the pathogenesis of epsteinbarr. Adoptive immunotherapy for ebvassociated lymphoproliferative disorders 457 140. Epstein barr virus ebv has been strongly associated with bcell cancers including endemic burkitts lymphoma, hodgkins disease, and posttransplant lymphoproliferative disease ptld. Chronic active epstein barr virus infection caebv is a systemic ebv positive lymphoproliferative disorder ebv lpd, which is marked by. A 16yearold boy with severe hydroa vacciniformelike eruptions associated with facial swelling, ulcerative skin lesions, fever, and liver damage case 26.
The management of epsteinbarr virus associated post. Ebvpositive lymphoproliferative disorders are heterogeneous, with variable clinical features and prognoses depending on the context in which. Over 90% of humans are infected with the epstein barr virus ebv and the infection persists for life. Clinicopathologic manifestations of epsteinbarr virus\pm\associatedcutaneous lymphoproliferative disorders keiji iwatsuki, md. Cd30positive tcell lymphoproliferative disease of the oral. Frontiers epstein barr virus associated bcell lymphomas. An adult with crohn disease cd taking azathioprine who later developed fatal ebv infection with bcell lymphoproliferative disorder lpd has been reported 7. Presence of epsteinbarr virus latency type iii at the single cell level in posttransplantation lymphoproliferative disorders and aids related lymphomas j clin pathol, 50 1997, pp. B cells, t cells, nk cells, and histiocyticdendritic cells, are infected with the epsteinbarr virus ebv.
Hemophagocytic syndrome in epsteinbarr virusassociated t. Dw kingma, wb weiss, es jaffe, s kumar, k frekko, m raffeld. Epsteinbarr virusassociated bcell lymphoproliferative. Pdf epsteinbarr virusassociated lymphoproliferative disorders. Infusions of donor leukocytes to treat epsteinbarr virus. Jan 01, 2020 epsteinbarr virus ebvpositive tcell and natural killer nkcell lymphoproliferative diseases ebvtnklpd are a group of uncommon disorders character ised by ebv infection of t and nkcells. Ebvassociated t or nkcell lymphoproliferations represent a continuous spectrum of diseases ranging from asymptomatic infection, infectious mononucleosis im, to clonal. The epsteinbarr virus ebv, formally called human gammaherpesvirus 4, is one of the nine known human herpesvirus types in the herpes family, and is one of the most common viruses in humans. Epsteinbarr virus ebv is a herpesvirus usually infecting bcells but may occasionally infect t or natural killer nkcells. Review article epstein barr virusassociated tnatural killer. Pdf epsteinbarr virus associated bcell lymphoma after.
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