Overview

NCI Definition: An aggressive and life-threatening, EBV-positive T-cell lymphoproliferative disorder 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. [1]

Significant Genes in Systemic EBV-Positive T-Cell Lymphoma of Childhood

ALK +

Disease Details

Synonyms
Systemic EBV-Positive T-Cell Lymphoproliferative Disease of Childhood, Systemic EBV Positive T-Cell Lymphoma of Childhood, Systemic EBV-Positive T-Cell Lymphoproliferative Disorder of Childhood
Parent(s)
Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
OncoTree Name
Systemic EBV Positive T-Cell Lymphoma of Childhood
OncoTree Code
SEBVTLC

References

1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.