Overview

NCI Definition: An extranodal, mature T-cell non-Hodgkin lymphoma that originates from cytotoxic T-cells, usually of gamma/delta T-cell type. It is characterized by the presence of medium-size neoplastic lymphocytes infiltrating the hepatic sinusoids. A similar infiltrating pattern is also present in the spleen and bone marrow that are usually involved at the time of the diagnosis. [1]

Hepatosplenic T-cell lymphomas most frequently harbor alterations in PTEN, POT1, and FBXW7 [2].

Most Commonly Altered Genes in Hepatosplenic T-Cell Lymphoma

PTEN Mutation, PTEN G127E, POT1 Mutation, POT1 H264N, and FBXW7 R479Q are the most common alterations in hepatosplenic T-cell lymphoma [2].

Top Alterations in Hepatosplenic T-Cell Lymphoma

Significant Genes in Hepatosplenic T-Cell Lymphoma

ALK +

FLT3 +

Disease Details

Synonyms
Bone marrow hepatosplenic T-cell lymphoma, Hepatosplenic Gamma/Delta T-Cell Lymphoma
Parent(s)
Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
OncoTree Name
Hepatosplenic T-Cell Lymphoma
OncoTree Code
HSTCL

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 8. 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.