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Systemic EBV-Positive T-Cell Lymphoma of Childhood

MedGen UID:
397492
•Concept ID:
C2699747
•
Neoplastic Process
Synonyms: Systemic EBV positive T-cell lymphoproliferative disease of childhood; Systemic Epstein Barr virus positive T-cell lymphoproliferative disease of childhood
SNOMED CT: Systemic EBV positive T-cell lymphoproliferative disease of childhood (450906003); Systemic Epstein Barr virus positive T-cell lymphoproliferative disease of childhood (450906003)

Definition

An aggressive and life-threatening, EBV-positive T-cell lymphoma affecting children. It is more prevalent in Taiwan and Japan. Clinically, it presents with acute onset of fever and generalized malaise, followed by hepatosplenomegaly and liver failure. Morphologically it is characterized by the presence of infiltrating T-lymphocytes which are usually small and erythrophagocytosis. Most patients have a fulminant clinical course. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSystemic EBV-Positive T-Cell Lymphoma 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

Diagnosis

Possible Concomitant Aggressive NK Cell Leukemia and EBV-positive T-cell lymphoma; Using the online beta version of WHO-HAEM5 and videoconferencing software to make diagnoses accessible in an emerging economy.
Dayton VJ, Hoang Thien D, Xinh PT, Arries C, Sang NN, Linh NNN, Lien NP, Dung PC
Diagn Pathol 2023 Oct 6;18(1):111. doi: 10.1186/s13000-023-01395-9. PMID: 37803436Free PMC Article

Therapy

Successful treatment of systemic EBV positive T-cell lymphoma of childhood using the SMILE regimen.
Yoshida M, Osumi T, Imadome KI, Tomizawa D, Kato M, Miyazawa N, Ito R, Nakazawa A, Matsumoto K
Pediatr Hematol Oncol 2018 Mar;35(2):121-124. Epub 2018 Apr 12 doi: 10.1080/08880018.2018.1459982. PMID: 29648917

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