U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination
  • C4303422 has been replaced by C2699747, showing C2699747

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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...