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Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

MedGen UID:
397492
•Concept ID:
C2699747
•
Neoplastic Process
Synonyms: Systemic EBV positive T-cell lymphoproliferative disease of childhood; Systemic EBV+ T-cell LPD of childhood; systemic EBV+ T-cell LPD of childhood; Systemic EBV-Positive T-Cell Lymphoma of Childhood; systemic EBV-positive T-cell lymphoma of childhood; Systemic EBV-positive T-cell lymphoproliferative disease of childhood; systemic EBV-positive T-cell lymphoproliferative disease of childhood; systemic EBV-positive T-cell lymphoproliferative disorder of childhood; Systemic Epstein Barr virus positive T-cell lymphoproliferative disease of childhood; Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood; Systemic Epstein-Barr Virus-Positive T-Cell Lymphoma 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); Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood (721311006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018223
Orphanet: ORPHA364033

Definition

A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSystemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

Professional guidelines

PubMed

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

Systemic Epstein-Barr Virus-positive T-Cell Lymphoproliferative Disease of Childhood With Good Response to Steroid Therapy.
Kim DH, Kim M, Kim Y, Han K, Han E, Lee JW, Chung NG, Cho B
J Pediatr Hematol Oncol 2017 Nov;39(8):e497-e500. doi: 10.1097/MPH.0000000000000909. PMID: 28697169
Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome.
Chen G, Chen L, Qin X, Huang Z, Xie X, Li G, Xu B
Int J Clin Exp Pathol 2014;7(10):7110-3. Epub 2014 Sep 15 PMID: 25400806Free PMC Article

Therapy

Systemic Epstein-Barr Virus-positive T-Cell Lymphoproliferative Disease of Childhood With Good Response to Steroid Therapy.
Kim DH, Kim M, Kim Y, Han K, Han E, Lee JW, Chung NG, Cho B
J Pediatr Hematol Oncol 2017 Nov;39(8):e497-e500. doi: 10.1097/MPH.0000000000000909. PMID: 28697169
Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome.
Chen G, Chen L, Qin X, Huang Z, Xie X, Li G, Xu B
Int J Clin Exp Pathol 2014;7(10):7110-3. Epub 2014 Sep 15 PMID: 25400806Free PMC Article

Prognosis

Systemic Epstein-Barr Virus-positive T-Cell Lymphoproliferative Disease of Childhood With Good Response to Steroid Therapy.
Kim DH, Kim M, Kim Y, Han K, Han E, Lee JW, Chung NG, Cho B
J Pediatr Hematol Oncol 2017 Nov;39(8):e497-e500. doi: 10.1097/MPH.0000000000000909. PMID: 28697169
Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome.
Chen G, Chen L, Qin X, Huang Z, Xie X, Li G, Xu B
Int J Clin Exp Pathol 2014;7(10):7110-3. Epub 2014 Sep 15 PMID: 25400806Free PMC Article

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