Details of Disease

General Information of Disease (ID: DISZPFTL)

• Disease Name: Angioimmunoblastic T-cell Lymphoma
• Synonyms: AITL; T-cell lymphoma, AILD type; AILD; angioimmunoblastic lymphadenopathy; angioimmunoblastic lymphadenopathy type T-cell lymphoma; angioimmunoblastic lymphadenopathy with Dysproteinemia; lymphogranulomatosis X; angioimmunoblastic T-cell lymphoma; immunoblastic lymphadenopathy; AILT
• Disease Class: 2A90: Mature T-cell lymphoma
• Definition: A mature T-cell non-Hodgkin lymphoma, characterized by systemic disease and a polymorphous infiltrate involving lymph nodes and extranodal sites. The clinical course is typically aggressive.
• Disease Hierarchy:
  DISCDT4E: Peripheral T-cell lymphoma
  DISZPFTL: Angioimmunoblastic T-cell Lymphoma
• ICD Code:
  ICD-11: ICD-11: 2A90.9
  ICD-10: ICD-10: C86.5
  ICD-9: ICD-9: 2A90.9
  Expand ICD-11: '2A90.9
  Expand ICD-10: 'C86.5
  Expand ICD-9: 2A90.9
• Disease Identifiers:
  MONDO ID: MONDO_0004977
  MESH ID: D007119
  UMLS CUI: C0020981
  MedGen ID: 7025
  Orphanet ID: 86886
  SNOMED CT ID: 413537009

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Plitidepsin	DMJ8AUE	Phase 3	Small molecular drug	[1]
TRBC1	DM1ZK4W	Phase 1/2	CAR T Cell Therapy	[2]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 12 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CR2	TT0HUN7	Limited	Biomarker	[3]
ITK	TT3C80U	Limited	Genetic Variation	[4]
TNFRSF21	TT8UA0T	Limited	Genetic Variation	[5]
TNFRSF8	TT2GM5R	Limited	Biomarker	[6]
ALK	TTPMQSO	Strong	Biomarker	[7]
BCL6	TTC9YX5	Strong	Biomarker	[3]
CXCL13	TT0NIZ1	Strong	Biomarker	[3]
DNMT3A	TTJUALD	Strong	Genetic Variation	[8]
ICOS	TTE5VP6	Strong	Biomarker	[3]
MME	TT5TKPM	Strong	Biomarker	[3]
RHOA	TTP2U16	Strong	Genetic Variation	[9]
PLCG1	TT6T4JI	Definitive	Altered Expression	[10]

This Disease Is Related to 15 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CMIP	OTZN8Z4A	Limited	Altered Expression	[11]
DLAT	OT9LBJVN	Limited	Biomarker	[12]
FBL	OTRODIE5	Limited	Biomarker	[13]
GTF2I	OTUYL1TQ	Limited	Genetic Variation	[14]
PWWP3A	OTXQVL4U	Limited	Biomarker	[15]
SAMSN1	OT7N88T1	Limited	Biomarker	[5]
SRSF2	OTVDHO6U	Limited	Genetic Variation	[16]
ALLC	OTZE4XV7	Strong	Genetic Variation	[17]
DUSP22	OTEZ3U85	Strong	Genetic Variation	[18]
FIP1L1	OTF91GTL	Strong	Genetic Variation	[19]
IDH2	OTTQA4PB	Strong	Genetic Variation	[8]
NCKIPSD	OT24UORN	Strong	Biomarker	[20]
NOCT	OTVSYP2D	Strong	Genetic Variation	[21]
TET2	OTKKT03T	Strong	Genetic Variation	[8]
RC3H1	OTCU6CYA	Definitive	Altered Expression	[22]

References

• [1] Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
• [2] ClinicalTrials.gov (NCT03590574) Phase I/II Study Evaluating AUTO4 in Patients With TRBC1 Positive T Cell Lymphoma
• [3] Application of a 5 Marker Panel to the Routine Diagnosis of Peripheral T-Cell Lymphoma With T-Follicular Helper Phenotype.Am J Surg Pathol. 2019 Sep;43(9):1282-1290. doi: 10.1097/PAS.0000000000001315.
• [4] Angioimmunoblastic T-cell lymphoma in Taiwan shows a frequent gain of ITK gene.Int J Clin Exp Pathol. 2014 Aug 15;7(9):6097-107. eCollection 2014.
• [5] Angioimmunoblastic T cell lymphoma: novel molecular insights by mutation profiling.Oncotarget. 2017 Mar 14;8(11):17763-17770. doi: 10.18632/oncotarget.14846.
• [6] FDA Approval Summary: Brentuximab Vedotin in First-Line Treatment of Peripheral T-Cell Lymphoma.Oncologist. 2019 May;24(5):e180-e187. doi: 10.1634/theoncologist.2019-0098. Epub 2019 Mar 26.
• [7] Outcomes following front-line chemotherapy in peripheral T-cell lymphoma: 10-year experience at The Royal Marsden and The Christie Hospital.Leuk Lymphoma. 2018 Jul;59(7):1586-1595. doi: 10.1080/10428194.2017.1393671. Epub 2017 Nov 9.
• [8] The pathological features of angioimmunoblastic T-cell lymphomas with IDH2(R172) mutations.Mod Pathol. 2019 Jul;32(8):1123-1134. doi: 10.1038/s41379-019-0254-4. Epub 2019 Apr 5.
• [9] RHOA G17V mutation in angioimmunoblastic T-cell lymphoma: A potential biomarker for cytological assessment.Exp Mol Pathol. 2019 Oct;110:104294. doi: 10.1016/j.yexmp.2019.104294. Epub 2019 Aug 5.
• [10] ITK inhibition induced in vitro and in vivo anti-tumor activity through downregulating TCR signaling pathway in malignant T cell lymphoma.Cancer Cell Int. 2019 Feb 14;19:32. doi: 10.1186/s12935-019-0754-9. eCollection 2019.
• [11] CMIP is oncogenic in human gastric cancer cells.Mol Med Rep. 2017 Nov;16(5):7277-7286. doi: 10.3892/mmr.2017.7541. Epub 2017 Sep 20.
• [12] The Pathogenesis of Autoimmune Liver Disease.Dig Dis. 2016;34(4):327-33. doi: 10.1159/000444471. Epub 2016 May 11.
• [13] Diagnostic performance of a point shear wave elastography (pSWE) for hepatic fibrosis in patients with autoimmune liver disease.PLoS One. 2019 Mar 11;14(3):e0212771. doi: 10.1371/journal.pone.0212771. eCollection 2019.
• [14] Activating mutations in genes related to TCR signaling in angioimmunoblastic and other follicular helper T-cell-derived lymphomas.Blood. 2016 Sep 15;128(11):1490-502. doi: 10.1182/blood-2016-02-698977. Epub 2016 Jul 1.
• [15] MUM-1 expression differentiates AITL with HRS-like cells from cHL.Int J Clin Exp Pathol. 2015 Sep 1;8(9):11372-8. eCollection 2015.
• [16] Response to 5-azacytidine in a patient with TET2-mutated angioimmunoblastic T-cell lymphoma and chronic myelomonocytic leukaemia preceded by an EBV-positive large B-cell lymphoma.Hematol Oncol. 2017 Dec;35(4):864-868. doi: 10.1002/hon.2319. Epub 2016 Jun 29.
• [17] Expression of human recombination activating genes (RAG-1 and RAG-2) in angioimmunoblastic lymphadenopathy and anaplastic large cell lymphoma of T-type.Br J Haematol. 1993 Apr;83(4):655-9. doi: 10.1111/j.1365-2141.1993.tb04706.x.
• [18] Molecular Pathogenesis of Peripheral T Cell Lymphoma.Curr Hematol Malig Rep. 2015 Dec;10(4):429-37. doi: 10.1007/s11899-015-0289-7.
• [19] Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report.Medicine (Baltimore). 2017 Sep;96(36):e8001. doi: 10.1097/MD.0000000000008001.
• [20] Human herpesvirus 6 (HHV-6) ORF-1 transactivating gene exhibits malignant transforming activity and its protein binds to p53.Oncogene. 1997 Jan 23;14(3):359-67. doi: 10.1038/sj.onc.1200840.
• [21] Molecular etiology of mature T-cell non-Hodgkin's lymphomas.Front Biosci. 2003 Jan 1;8:d156-75. doi: 10.2741/922.
• [22] ROQUIN/RC3H1 alterations are not found in angioimmunoblastic T-cell lymphoma.PLoS One. 2013 Jun 25;8(6):e64536. doi: 10.1371/journal.pone.0064536. Print 2013.