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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
      - Disorder of lymphoid system
        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
        EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified
        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
        Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly
        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

Hematologic cancers in individuals infected by HIV.

Carbone A, Vaccher E, Gloghini A
Blood 2022 Feb 17;139(7):995-1012. doi: 10.1182/blood.2020005469. PMID: 34469512

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.

Haemophagocytic lymphohistiocytosis and Epstein-Barr virus: a complex relationship with diverse origins, expression and outcomes.

El-Mallawany NK, Curry CV, Allen CE
Br J Haematol 2022 Jan;196(1):31-44. Epub 2021 Jun 24 doi: 10.1111/bjh.17638. PMID: 34169507

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

Biomarkers for PTLD diagnosis and therapies.

Martinez OM
Pediatr Nephrol 2020 Jul;35(7):1173-1181. Epub 2019 Jun 25 doi: 10.1007/s00467-019-04284-w. PMID: 31240394

Vaccine Development for Epstein-Barr Virus.

Cohen JI
Adv Exp Med Biol 2018;1045:477-493. doi: 10.1007/978-981-10-7230-7_22. PMID: 29896681 Free PMC Article

Post-transplant lymphoproliferative disorder.

Kalinova L, Indrakova J, Bachleda P
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2009 Dec;153(4):251-7. doi: 10.5507/bp.2009.043. PMID: 20208963

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

See all (1054)

Prognosis

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

EBV-positive B-cell lymphomas and lymphoproliferative disorders: Review from the perspective of immune escape and immunodeficiency.

Satou A, Nakamura S
Cancer Med 2021 Oct;10(19):6777-6785. Epub 2021 Aug 13 doi: 10.1002/cam4.4198. PMID: 34387382 Free PMC Article

Lymphadenopathy at the crossroad between immunodeficiency and autoinflammation: An intriguing challenge.

Costagliola G, Consolini R
Clin Exp Immunol 2021 Sep;205(3):288-305. Epub 2021 Jun 20 doi: 10.1111/cei.13620. PMID: 34008169 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

See all (1281)

Clinical prediction guides

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

Lymphadenopathy at the crossroad between immunodeficiency and autoinflammation: An intriguing challenge.

Costagliola G, Consolini R
Clin Exp Immunol 2021 Sep;205(3):288-305. Epub 2021 Jun 20 doi: 10.1111/cei.13620. PMID: 34008169 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

The Epstein-Barr virus and the pathogenesis of lymphoma.

Vockerodt M, Yap LF, Shannon-Lowe C, Curley H, Wei W, Vrzalikova K, Murray PG
J Pathol 2015 Jan;235(2):312-22. doi: 10.1002/path.4459. PMID: 25294567

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Recent systematic reviews

Other iatrogenic immunodeficiency-associated lymphoproliferative disorders in a patient with anti-melanoma differentiation-associated gene 5-positive dermatomyositis: A case report and systematic literature review.

Nishimura N, Niwamoto T, Arai Y, Nakashima R, Terada K, Yoshizawa A, Sakamoto R, Tanizawa K, Handa T, Morinobu A, Hirai T
Int J Rheum Dis 2023 Jun;26(6):1172-1177. Epub 2023 Feb 15 doi: 10.1111/1756-185X.14608. PMID: 36789793

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

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

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