Details of Disease

Disease Name

Hepatosplenic T-cell lymphoma

HSTCL; Hepatosplenic gamma/Delta T-cell lymphoma

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.

Disease Hierarchy

DISSXRTQ: T-cell lymphoma

DIS7KMY9: Hepatosplenic T-cell lymphoma

Disease Identifiers

MONDO ID

MONDO_0019474

UMLS CUI

C1333984

MedGen ID

272711

Orphanet ID

86882

SNOMED CT ID

445406001

General Information of Disease (ID: DIS7KMY9)

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 8 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Fludarabine	DMVRLT7	Approved	Small molecular drug	[1]
Lenalidomide	DM6Q7U4	Approved	Small molecular drug	[2]
Mycophenolate mofetil	DMPQAGE	Approved	Small molecular drug	[3]
Mycophenolic acid	DMRBMAU	Approved	NA	[4]
Rituximab	DM1YVZT	Approved	Antibody	[5]
Tacrolimus	DMZ7XNQ	Approved	Small molecular drug	[6]
Thalidomide	DM70BU5	Approved	Small molecular drug	[7]
Vidarabine	DM0N85H	Approved	Small molecular drug	[8]
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⏷ Show the Full List of 8 Drug(s)

Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 1 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CHN2	TT39YVO	Strong	Genetic Variation	[9]
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This Disease Is Related to 5 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
RHOJ	OTWI65OA	Disputed	Biomarker	[10]
AUTS2	OTAEXHSC	Strong	Biomarker	[11]
DERL2	OTI3TUUZ	Strong	Biomarker	[11]
GLIS2	OTOUUV1X	Strong	Biomarker	[11]
PPP1R9A	OTJL2TOC	Strong	Biomarker	[9]
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References

1	Fludarabine FDA Label
2	Lenalidomide FDA Label
3	Mycophenolate mofetil FDA Label
4	A standard database for drug repositioning. Sci Data. 2017 Mar 14;4:170029.
5	Coexistent hairy cell leukaemia and hepatosplenic T-cell lymphoma: a case report. Diagn Pathol. 2014 Mar 12;9:58.
6	Tacrolimus FDA Label
7	Thalidomide FDA Label
8	Vidarabine FDA Label
9	Integrative genomic and transcriptomic analysis identified candidate genes implicated in the pathogenesis of hepatosplenic T-cell lymphoma.PLoS One. 2014 Jul 24;9(7):e102977. doi: 10.1371/journal.pone.0102977. eCollection 2014.
10	Fluorescence in situ hybridization study of chromosome 7 aberrations in hepatosplenic T-cell lymphoma: isochromosome 7q as a common abnormality accumulating in forms with features of cytologic progression.Genes Chromosomes Cancer. 2002 Mar;33(3):243-51. doi: 10.1002/gcc.10021.
11	Deregulated expression of NKL homeobox genes in T-cell lymphomas.Oncotarget. 2019 May 14;10(35):3227-3247. doi: 10.18632/oncotarget.26929. eCollection 2019 May 14.