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Epstein-Barr virus-associated malignant lymphoproliferative disorder

MedGen UID:: 797800
•Concept ID:: C2363744
•: Disease or Syndrome

Synonyms:	EBV-associated lymphoproliferative disorder; EBV-Related Lymphoproliferative Disorder; Epstein-Barr Virus-Associated Lymphoproliferative Disorder; Epstein-Barr Virus-Related Lymphoproliferative Disorder

Monarch Initiative:	MONDO:0017343
Orphanet:	ORPHA289644

Definition

A lymphoproliferative disorder associated with Epstein-Barr virus. This category includes, but is not limited to, Burkitt lymphoma, classic Hodgkin lymphoma, and lymphomas arising in immunocompromised individuals. [from NCI]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVEpstein-Barr virus-associated malignant lymphoproliferative disorder

Pathological process
- Disease
  - Disorder by Site
    - Hematopoietic and Lymphoid System Disorder
      - Hematologic disorder
        Lymphoproliferative disorder
        Epstein-Barr virus-associated malignant lymphoproliferative disorder
        Burkitt lymphoma
        Adult Burkitt Lymphoma
        Ann Arbor Stage I Adult Burkitt Lymphoma
        Ann Arbor Stage II Adult Burkitt Lymphoma
        Ann Arbor Stage III Adult Burkitt Lymphoma
        Ann Arbor Stage IV Adult Burkitt Lymphoma
        Recurrent Adult Burkitt Lymphoma
        Atypical Burkitt/Burkitt-Like Lymphoma
        Burkitt-Like Lymphoma Post-Transplant Lymphoproliferative Disorder
        Small Intestinal Atypical Burkitt/Burkitt-Like Lymphoma
        Breast Burkitt Lymphoma
        Burkitt Lymphoma by Ann Arbor Stage
        Ann Arbor Stage I Burkitt Lymphoma
        Ann Arbor Stage II Burkitt Lymphoma
        Ann Arbor Stage III Burkitt Lymphoma
        Ann Arbor Stage IV Burkitt Lymphoma
        Burkitt Lymphoma with Plasmacytoid Differentiation
        AIDS-Related Burkitt Lymphoma with Plasmacytoid Differentiation
        Childhood Burkitt Lymphoma
        Ann Arbor Stage I Childhood Burkitt Lymphoma
        Ann Arbor Stage II Childhood Burkitt Lymphoma
        Ann Arbor Stage III Childhood Burkitt Lymphoma
        Ann Arbor Stage IV Childhood Burkitt Lymphoma
        Recurrent Childhood Burkitt Lymphoma
        Classical Burkitt Lymphoma
        Colorectal Burkitt Lymphoma
        Colon Burkitt lymphoma
        Cutaneous Burkitt Lymphoma
        EBV-Related Burkitt Lymphoma
        Endemic Burkitt Lymphoma
        Gastric Burkitt Lymphoma
        Immunodeficiency-Related Burkitt Lymphoma
        AIDS-Related Burkitt Lymphoma
        Burkitt Lymphoma Post-Transplant Lymphoproliferative Disorder
        Recurrent Burkitt Lymphoma
        Refractory Burkitt Lymphoma
        Small intestinal Burkitt lymphoma
        Splenic Burkitt Lymphoma
        Sporadic Burkitt Lymphoma
        Diffuse large B-cell lymphoma with chronic inflammation
        EBV positive mucocutaneous ulcer
        EBV-positive T-cell lymphoproliferative disorder of childhood
        Hydroa vacciniforme-like lymphoma
        Systemic EBV-Positive T-Cell Lymphoma of Childhood
        EBV-Related Lymphoma
        EBV-Related Clonal Post-Transplant Lymphoproliferative Disorder
        Classic Hodgkin Lymphoma Type Post-Transplant Lymphoproliferative Disorder
        Hodgkin-Like Post-Transplant Lymphoproliferative Disorder
        EBV-Related Hodgkin Lymphoma
        EBV-Related Non-Hodgkin Lymphoma
        Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly
        Grade III lymphomatoid granulomatosis
        Primary EBV-Positive Nodal T-Cell or NK-Cell Lymphoma
        Recurrent EBV-Related Lymphoma
        Recurrent EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified
        Recurrent Grade III Lymphomatoid Granulomatosis
        Refractory EBV-Related Lymphoma
        Refractory EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified
        EBV-Related Post-Transplant Lymphoproliferative Disorder
        Extranodal nasal NK/T cell lymphoma
        Adult Nasal Type Extranodal NK/T-Cell Lymphoma
        Childhood Nasal Type Extranodal NK/T-Cell Lymphoma
        Cutaneous Nasal Type Extranodal NK/T-Cell Lymphoma
        Nasal Type NK/T-Cell Lymphoma by Ann Arbor Stage
        Ann Arbor Stage I Nasal Type NK/T-Cell Lymphoma
        Ann Arbor Stage II Nasal Type NK/T-Cell Lymphoma
        Ann Arbor Stage III Nasal Type NK/T-Cell Lymphoma
        Ann Arbor Stage IV Nasal Type NK/T-Cell Lymphoma
        Recurrent Nasal Type Extranodal NK/T-Cell Lymphoma
        Refractory Nasal Type Extranodal NK/T-Cell Lymphoma
        Sinonasal Extranodal NK/T-Cell Lymphoma
        Nasal Cavity Extranodal NK/T-Cell Lymphoma
        Testicular Nasal Type Extranodal NK/T-Cell Lymphoma
        HHV8-Positive Germinotropic Lymphoproliferative Disorder
        Lymphomatoid granulomatosis
        Childhood Lymphomatoid Granulomatosis
        Childhood Grade III Lymphomatoid Granulomatosis
        Recurrent Childhood Lymphomatoid Granulomatosis
        Cutaneous Lymphomatoid Granulomatosis
        Grade I lymphomatoid granulomatosis
        Recurrent Grade I Lymphomatoid Granulomatosis
        Grade II lymphomatoid granulomatosis
        Recurrent Grade II Lymphomatoid Granulomatosis
        Recurrent Lymphomatoid Granulomatosis
        Plasmablastic lymphoma
        AIDS-Related Plasmablastic Lymphoma
        AIDS-Related Plasmablastic Lymphoma of Mucosa Site
        Plasmablastic Lymphoma of Mucosa Site
        Plasmablastic Lymphoma of the Oral Mucosa
        Primary Cutaneous Plasmablastic Lymphoma
        Recurrent Plasmablastic Lymphoma
        Refractory Plasmablastic Lymphoma
        Post-transplant lymphoproliferative disease
        Neoplastic Post-Transplant Lymphoproliferative Disorder
        Monomorphic Post-Transplant Lymphoproliferative Disorder
        Polymorphic Post-Transplant Lymphoproliferative Disorder
        Non-Destructive Post-Transplant Lymphoproliferative Disorder
        Florid Follicular Hyperplasia Post-Transplant Lymphoproliferative Disorder
        Infectious mononucleosis-like post-transplant lymphoproliferative disorder
        Plasmacytic Hyperplasia Post-Transplant Lymphoproliferative Disorder
        Primary central nervous system lymphoma
        AIDS-related primary central nervous system lymphoma
        Central Nervous System Hodgkin Lymphoma
        Cerebral Hodgkin Lymphoma
        Central nervous system non-hodgkin lymphoma
        Central Nervous System B-Cell Non-Hodgkin Lymphoma
        Central Nervous System T-Cell Non-Hodgkin Lymphoma
        Cerebral Non-Hodgkin Lymphoma
        Pituitary Gland Non-Hodgkin Lymphoma
        Primary Vitreoretinal Non-Hodgkin Lymphoma
        Recurrent Central Nervous System Non-Hodgkin Lymphoma
        Refractory Central Nervous System Non-Hodgkin Lymphoma
        Cerebral lymphoma
        Cerebral Lymphoma in Immunocompetent Host
        Meningeal Lymphoma
        Dural Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue
        Recurrent Central Nervous System Lymphoma
        Refractory Central Nervous System Lymphoma
        Spinal cord lymphoma
        Primary effusion lymphoma
        AIDS-Related Primary Effusion Lymphoma
        Body Cavity Primary Effusion Lymphoma
        Extracavitary Primary Effusion Lymphoma
        Recurrent Primary Effusion Lymphoma
        Refractory Primary Effusion Lymphoma
        Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

Follow this link to review classifications for Epstein-Barr virus-associated malignant lymphoproliferative disorder in Orphanet.

Professional guidelines

PubMed

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.

Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248

The treatment of Burkitt lymphoma in adults.

Crombie J, LaCasce A
Blood 2021 Feb 11;137(6):743-750. doi: 10.1182/blood.2019004099. PMID: 33171490

MALT lymphoma: epidemiology, clinical diagnosis and treatment.

Violeta Filip P, Cuciureanu D, Sorina Diaconu L, Maria Vladareanu A, Silvia Pop C
J Med Life 2018 Jul-Sep;11(3):187-193. doi: 10.25122/jml-2018-0035. PMID: 30364585 Free PMC Article

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Recent clinical studies

Etiology

New concepts in EBV-associated B, T, and NK cell lymphoproliferative disorders.

Quintanilla-Martinez L, Swerdlow SH, Tousseyn T, Barrionuevo C, Nakamura S, Jaffe ES
Virchows Arch 2023 Jan;482(1):227-244. Epub 2022 Oct 11 doi: 10.1007/s00428-022-03414-4. PMID: 36216980 Free PMC Article

Impact of Epstein-Barr virus infection in patients with inflammatory bowel disease.

Zhang H, Zhao S, Cao Z
Front Immunol 2022;13:1001055. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1001055. PMID: 36389673 Free PMC Article

Epidemiology of Non-Hodgkin's Lymphoma.

Thandra KC, Barsouk A, Saginala K, Padala SA, Barsouk A, Rawla P
Med Sci (Basel) 2021 Jan 30;9(1) doi: 10.3390/medsci9010005. PMID: 33573146 Free PMC Article

Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach.

Hue SS, Oon ML, Wang S, Tan SY, Ng SB
Pathology 2020 Jan;52(1):111-127. Epub 2019 Nov 22 doi: 10.1016/j.pathol.2019.09.011. PMID: 31767131

Epstein-Barr virus and its association with disease - a review of relevance to general practice.

Fugl A, Andersen CL
BMC Fam Pract 2019 May 14;20(1):62. doi: 10.1186/s12875-019-0954-3. PMID: 31088382 Free PMC Article

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Diagnosis

New concepts in EBV-associated B, T, and NK cell lymphoproliferative disorders.

Impact of Epstein-Barr virus infection in patients with inflammatory bowel disease.

Zhang H, Zhao S, Cao Z
Front Immunol 2022;13:1001055. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1001055. PMID: 36389673 Free PMC Article

Epidemiology of Non-Hodgkin's Lymphoma.

Thandra KC, Barsouk A, Saginala K, Padala SA, Barsouk A, Rawla P
Med Sci (Basel) 2021 Jan 30;9(1) doi: 10.3390/medsci9010005. PMID: 33573146 Free PMC Article

Epstein-Barr virus-associated T- and NK-cell lymphoproliferative diseases: an update and diagnostic approach.

Hue SS, Oon ML, Wang S, Tan SY, Ng SB
Pathology 2020 Jan;52(1):111-127. Epub 2019 Nov 22 doi: 10.1016/j.pathol.2019.09.011. PMID: 31767131

Lymphoproliferative Disorders of the Lung.

Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772

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Therapy

Estimating the global burden of Epstein-Barr virus-related cancers.

Wong Y, Meehan MT, Burrows SR, Doolan DL, Miles JJ
J Cancer Res Clin Oncol 2022 Jan;148(1):31-46. Epub 2021 Oct 27 doi: 10.1007/s00432-021-03824-y. PMID: 34705104 Free PMC Article

Human Immunodeficiency Virus-Associated Lymphoproliferative Disorders.

Lilly AJ, Fedoriw Y
Surg Pathol Clin 2019 Sep;12(3):771-782. Epub 2019 May 17 doi: 10.1016/j.path.2019.03.005. PMID: 31352987

Therapeutic Strategies against Epstein-Barr Virus-Associated Cancers Using Proteasome Inhibitors.

Hui KF, Tam KP, Chiang AKS
Viruses 2017 Nov 21;9(11) doi: 10.3390/v9110352. PMID: 29160853 Free PMC Article

Viral lymphomagenesis.

Ahmed N, Heslop HE
Curr Opin Hematol 2006 Jul;13(4):254-9. doi: 10.1097/01.moh.0000231423.38525.fe. PMID: 16755222

Immunodeficiency-associated lymphoproliferative disorders.

Knowles DM
Mod Pathol 1999 Feb;12(2):200-17. PMID: 10071343

See all (1069)

Prognosis

Epstein-Barr virus-associated lymphomas decoded.

Bednarska K, Chowdhury R, Tobin JWD, Swain F, Keane C, Boyle S, Khanna R, Gandhi MK
Br J Haematol 2024 Feb;204(2):415-433. Epub 2023 Dec 28 doi: 10.1111/bjh.19255. PMID: 38155519

New concepts in EBV-associated B, T, and NK cell lymphoproliferative disorders.

Impact of Epstein-Barr virus infection in patients with inflammatory bowel disease.

Zhang H, Zhao S, Cao Z
Front Immunol 2022;13:1001055. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1001055. PMID: 36389673 Free PMC Article

Lymphoproliferative Disorders of the Lung.

Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772

Epstein-Barr Virus-associated Lymphoproliferative Disorders in the Skin.

Goodlad JR
Surg Pathol Clin 2017 Jun;10(2):429-453. Epub 2017 Mar 18 doi: 10.1016/j.path.2017.01.001. PMID: 28477890

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Clinical prediction guides

The Clinical Significance and Prognostic Role of Whole-Blood Epstein-Barr Virus DNA in Lymphoma-Associated Hemophagocytic Lymphohistiocytosis.

Zhang J, Qin S, Jin Z, Chen Q, Xing L, Qiu T, Xia Y, Liang J, Zhu H, Wang L, Fan L, Xu W, Li J, Miao Y
J Clin Immunol 2023 Aug;43(6):1302-1310. Epub 2023 Apr 24 doi: 10.1007/s10875-023-01493-9. PMID: 37093406

EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2022 update on diagnosis, risk-stratification, and management.

Malpica L, Marques-Piubelli ML, Beltran BE, Chavez JC, Miranda RN, Castillo JJ
Am J Hematol 2022 Jul;97(7):951-965. Epub 2022 May 3 doi: 10.1002/ajh.26579. PMID: 35472248

Estimating the global burden of Epstein-Barr virus-related cancers.

Wong Y, Meehan MT, Burrows SR, Doolan DL, Miles JJ
J Cancer Res Clin Oncol 2022 Jan;148(1):31-46. Epub 2021 Oct 27 doi: 10.1007/s00432-021-03824-y. PMID: 34705104 Free PMC Article

Defective Epstein-Barr virus in chronic active infection and haematological malignancy.

Okuno Y, Murata T, Sato Y, Muramatsu H, Ito Y, Watanabe T, Okuno T, Murakami N, Yoshida K, Sawada A, Inoue M, Kawa K, Seto M, Ohshima K, Shiraishi Y, Chiba K, Tanaka H, Miyano S, Narita Y, Yoshida M, Goshima F, Kawada JI, Nishida T, Kiyoi H, Kato S, Nakamura S, Morishima S, Yoshikawa T, Fujiwara S, Shimizu N, Isobe Y, Noguchi M, Kikuta A, Iwatsuki K, Takahashi Y, Kojima S, Ogawa S, Kimura H
Nat Microbiol 2019 Mar;4(3):404-413. Epub 2019 Jan 21 doi: 10.1038/s41564-018-0334-0. PMID: 30664667

Epstein-Barr virus in the pathogenesis of oral cancers.

Guidry JT, Birdwell CE, Scott RS
Oral Dis 2018 May;24(4):497-508. Epub 2017 Apr 18 doi: 10.1111/odi.12656. PMID: 28190296 Free PMC Article

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Recent systematic reviews

Non-invasive monitoring associated with B lymphoma cells in post-transplant lymphoproliferative disorder (PTLD) patients: Systematic review.

Honar N, Shahramian I, Imanieh MH, Ataollahi M, Tahani M, Rakhshaninasab S, Javadifar A
Hum Antibodies 2022;30(4):183-194. doi: 10.3233/HAB-220016. PMID: 37005883

Is Hodgkin Lymphoma Associated with Hepatitis B and C Viruses? A Systematic Review and Meta-analysis.

Mullen CJR, Volesky KD, Greenwald ZR, El-Zein M, Franco EL
Cancer Epidemiol Biomarkers Prev 2021 Dec;30(12):2167-2175. Epub 2021 Sep 20 doi: 10.1158/1055-9965.EPI-21-0548. PMID: 34548328

Risk factors for Epstein Barr virus-associated cancers: a systematic review, critical appraisal, and mapping of the epidemiological evidence.

Bakkalci D, Jia Y, Winter JR, Lewis JE, Taylor GS, Stagg HR
J Glob Health 2020 Jun;10(1):010405. doi: 10.7189/jogh.10.010405. PMID: 32257153 Free PMC Article

Primary immunodeficiencies and lymphoma: a systematic review of literature.

Herber M, Mertz P, Dieudonné Y, Guffroy B, Jung S, Gies V, Korganow AS, Guffroy A
Leuk Lymphoma 2020 Feb;61(2):274-284. Epub 2019 Oct 3 doi: 10.1080/10428194.2019.1672056. PMID: 31580160

Genetic associations in classical hodgkin lymphoma: a systematic review and insights into susceptibility mechanisms.

Kushekhar K, van den Berg A, Nolte I, Hepkema B, Visser L, Diepstra A
Cancer Epidemiol Biomarkers Prev 2014 Dec;23(12):2737-47. Epub 2014 Sep 9 doi: 10.1158/1055-9965.EPI-14-0683. PMID: 25205514

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Epstein-Barr virus-associated malignant lymphoproliferative disorder

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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