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EBV-positive T-cell lymphoproliferative disorder of childhood

MedGen UID:
397515
•Concept ID:
C2699838
•
Neoplastic Process
Synonyms: EBV-Positive T-Cell Lymphoproliferative Disease of Childhood; EBV-positive T-cell lymphoproliferative disease of childhood; EBV-Positive T-Cell Lymphoproliferative Disorder of Childhood; EBV-Positive T-Cell/NK-Cell Lymphoproliferative Disorder of Childhood
 
Monarch Initiative: MONDO:0006188

Definition

An Epstein-Barr virus-associated mature T-cell/NK-cell lymphoproliferative group of disorders affecting children. It occurs with increased frequency in Asians and Native Americans. It includes the systemic EBV-positive T-cell lymphoma of childhood and the hydroa vacciniforme-like lymphoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVEBV-positive T-cell lymphoproliferative disorder of childhood

Professional guidelines

PubMed

Successful treatment of systemic EBV-positive T-cell lymphoma of childhood using the ruxolitinib combined with L-DEP regimen chemotherapy followed by haploidentical allogeneic hematopoietic stem cell transplantation.
Li J, Cao C, Lu S, Rong L, Kang M, Fang Y
Ann Hematol 2023 Oct;102(10):2955-2957. Epub 2023 Jul 25 doi: 10.1007/s00277-023-05298-z. PMID: 37490113
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis.
Montes-Mojarro IA, Kim WY, Fend F, Quintanilla-Martinez L
Semin Diagn Pathol 2020 Jan;37(1):32-46. Epub 2019 Dec 17 doi: 10.1053/j.semdp.2019.12.004. PMID: 31889602

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: 36216980Free PMC Article
How I Diagnose EBV-Positive B- and T-Cell Lymphoproliferative Disorders.
Dojcinov SD, Quintanilla-Martinez L
Am J Clin Pathol 2023 Jan 4;159(1):14-33. doi: 10.1093/ajcp/aqac105. PMID: 36214507
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
Cutaneous EBV-related lymphoproliferative disorders.
Gru AA, Jaffe ES
Semin Diagn Pathol 2017 Jan;34(1):60-75. Epub 2016 Dec 7 doi: 10.1053/j.semdp.2016.11.003. PMID: 27988064Free PMC Article
Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.
Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145

Diagnosis

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: 36216980Free PMC Article
How I Diagnose EBV-Positive B- and T-Cell Lymphoproliferative Disorders.
Dojcinov SD, Quintanilla-Martinez L
Am J Clin Pathol 2023 Jan 4;159(1):14-33. doi: 10.1093/ajcp/aqac105. PMID: 36214507
EBV associated T- and NK-cell lymphoproliferative diseases: A comprehensive overview of clinical manifestations and novel therapeutic insights.
Shafiee A, Shamsi S, Kohandel Gargari O, Beiky M, Allahkarami MM, Miyanaji AB, Aghajanian S, Mozhgani SH
Rev Med Virol 2022 Jul;32(4):e2328. Epub 2022 Feb 4 doi: 10.1002/rmv.2328. PMID: 35122349
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
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

Therapy

Unrelated cord blood transplantation for adult-onset EBV-associated T-cell and NK-cell lymphoproliferative disorders.
Onishi Y, Onodera K, Fukuhara N, Kato H, Ichikawa S, Fujiwara T, Yokoyama H, Yamada-Fujiwara M, Harigae H
Int J Hematol 2022 Jun;115(6):873-881. Epub 2022 Mar 10 doi: 10.1007/s12185-022-03313-z. PMID: 35274195
Hydroa Vacciniforme and Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Spectrum of Disease Phenotypes Associated with Ultraviolet Irradiation and Chronic Epstein-Barr Virus Infection.
Chen CC, Chang KC, Medeiros LJ, Lee JY
Int J Mol Sci 2020 Dec 7;21(23) doi: 10.3390/ijms21239314. PMID: 33297336Free PMC Article
Systemic EBV-positive T-cell lymphoma of childhood mimicking anaplastic large cell lymphoma.
Chen Z, Liu W, Zhao S
Br J Haematol 2019 Jun;185(6):1016. doi: 10.1111/bjh.16054. PMID: 31172518
Mature T- and NK-cell non-Hodgkin lymphoma in children and young adolescents.
Pillai V, Tallarico M, Bishop MR, Lim MS
Br J Haematol 2016 May;173(4):573-81. Epub 2016 Mar 15 doi: 10.1111/bjh.14044. PMID: 26992145
Post-transplant lymphoproliferative disease in pediatric solid organ transplant recipients.
Wistinghausen B, Gross TG, Bollard C
Pediatr Hematol Oncol 2013 Sep;30(6):520-31. Epub 2013 Jun 26 doi: 10.3109/08880018.2013.798844. PMID: 23802715

Prognosis

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: 36216980Free PMC Article
Unrelated cord blood transplantation for adult-onset EBV-associated T-cell and NK-cell lymphoproliferative disorders.
Onishi Y, Onodera K, Fukuhara N, Kato H, Ichikawa S, Fujiwara T, Yokoyama H, Yamada-Fujiwara M, Harigae H
Int J Hematol 2022 Jun;115(6):873-881. Epub 2022 Mar 10 doi: 10.1007/s12185-022-03313-z. PMID: 35274195
Hydroa vacciniforme-like lymphoproliferative disorder in Korea.
Han B, Hur K, Ohn J, Kim TM, Jeon YK, Kim YC, Mun JH
Sci Rep 2020 Nov 9;10(1):19294. doi: 10.1038/s41598-020-76345-2. PMID: 33168864Free PMC Article
Comparison of Systemic EBV-positive T-Cell and NK-Cell Lymphoproliferative Diseases of Childhood Based on Classification Evolution: New Classification, Old Problems.
Chen Z, Wang M, Guan P, Zhao S, Zhang W, Gao L, Tang Y, Li L, Liu W
Am J Surg Pathol 2020 Aug;44(8):1061-1072. doi: 10.1097/PAS.0000000000001495. PMID: 32317607
Epstein - Barr virus positive T and NK-cell lymphoproliferations: Morphological features and differential diagnosis.
Montes-Mojarro IA, Kim WY, Fend F, Quintanilla-Martinez L
Semin Diagn Pathol 2020 Jan;37(1):32-46. Epub 2019 Dec 17 doi: 10.1053/j.semdp.2019.12.004. PMID: 31889602

Clinical prediction guides

Characteristics of genetic alterations of peripheral T-cell lymphoma in childhood including identification of novel fusion genes: the Japan Children's Cancer Group (JCCG).
Ohki K, Kiyokawa N, Watanabe S, Iwafuchi H, Nakazawa A, Ishiwata K, Ogata-Kawata H, Nakabayashi K, Okamura K, Tanaka F, Fukano R, Hata K, Mori T, Moriya Saito A, Hayashi Y, Taga T, Sekimizu M, Kobayashi R; Japan Children’s Cancer Study Group (JCCG)
Br J Haematol 2021 Aug;194(4):718-729. Epub 2021 Jul 13 doi: 10.1111/bjh.17639. PMID: 34258755
Comparison of Systemic EBV-positive T-Cell and NK-Cell Lymphoproliferative Diseases of Childhood Based on Classification Evolution: New Classification, Old Problems.
Chen Z, Wang M, Guan P, Zhao S, Zhang W, Gao L, Tang Y, Li L, Liu W
Am J Surg Pathol 2020 Aug;44(8):1061-1072. doi: 10.1097/PAS.0000000000001495. PMID: 32317607
The ambiguous boundary between EBV-related hemophagocytic lymphohistiocytosis and systemic EBV-driven T cell lymphoproliferative disorder.
Smith MC, Cohen DN, Greig B, Yenamandra A, Vnencak-Jones C, Thompson MA, Kim AS
Int J Clin Exp Pathol 2014;7(9):5738-49. Epub 2014 Aug 15 PMID: 25337215Free PMC Article
Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma.
Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R, Alderete G, Grube P, Lome-Maldonado C, Bonzheim I, Fend F
Blood 2013 Oct 31;122(18):3101-10. Epub 2013 Aug 27 doi: 10.1182/blood-2013-05-502203. PMID: 23982171
On the aetiology of Hodgkin lymphoma.
Hjalgrim H
Dan Med J 2012 Jul;59(7):B4485. PMID: 22759852

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