Details of Disease

General Information of Disease (ID: DISAUA10)

Disease Name	Mediastinal large B-cell lymphoma
Synonyms	primary mediastinal B-cell lymphoma; primary mediastinal (thymic) large B-cell lymphoma; mediastinal large B-cell lymphoma; primary mediastinal clear cell lymphoma of B-cell type; mediastinal (thymic) large B-cell lymphoma; PMLCL; B-cell diffuse large cell lymphoma of mediastinum; B-cell diffuse large cell lymphoma of the mediastinum; mediastinal B-cell diffuse large cell lymphoma; mediastinal diffuse large-cell lymphoma with sclerosis; large cell lymphoma of the mediastinum; primary mediastinal large B-cell lymphoma; PMBL; mediastinal diffuse large cell lymphoma with sclerosis; Med-DLBCL
Disease Class	2A60: Acute myeloid leukaemia
Definition	A large B-cell non-Hodgkin lymphoma arising in the mediastinum. Morphologically it is characterized by a massive diffuse lymphocytic proliferation associated with compartmentalizing fibrosis. Response to intensive chemotherapy, with or without radiotherapy, is usually good. (WHO, 2001)
Disease Hierarchy	DISJ17C5: Thymus lymphoma DISIH1YQ: B-cell lymphoma DISAUA10: Mediastinal large B-cell lymphoma
ICD Code	ICD-11 ICD-11: 2A60.32 Expand ICD-11 '2A60.32
Disease Identifiers	MONDO ID MONDO_0020323 UMLS CUI C1292754 MedGen ID 714984 Orphanet ID 98838 SNOMED CT ID 128801005

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

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

Drug Name	Drug ID	Highest Status	Drug Type	REF
JCAR014	DMTMVEW	Phase 1/2	CAR T Cell Therapy	[1]
CD19/CD22 Chimeric Antigen Receptor T Cells	DMPADR1	Phase 1	CAR T Cell Therapy	[2]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 8 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
BCL6	TTC9YX5	Strong	Biomarker	[3]
FCER2	TTCH6MU	Strong	Biomarker	[4]
FSCN1	TTTRS9B	Strong	Biomarker	[5]
REL	TT1ZCTH	Strong	Biomarker	[6]
SOCS1	TT8COJM	Strong	Genetic Variation	[7]
MS4A1	TTUE541	Definitive	Biomarker	[8]
PDCD1LG2	TTW14O3	Definitive	Biomarker	[9]
XPO1	TTCJUR4	Definitive	Genetic Variation	[7]
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⏷ Show the Full List of 8 DTT(s)

This Disease Is Related to 7 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CHRDL2	OTJU2I5H	Strong	Biomarker	[10]
CIITA	OTRJNZFO	Strong	Biomarker	[11]
IL4I1	OTK54C63	Strong	Altered Expression	[12]
JCHAIN	OTR8M5TX	Strong	Biomarker	[13]
MEF2B	OT880SE6	Strong	Biomarker	[13]
POU2F2	OTPV0J0C	Strong	Biomarker	[13]
NFKBIE	OTLAYEL9	Definitive	Biomarker	[14]
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⏷ Show the Full List of 7 DOT(s)

References

1	ClinicalTrials.gov (NCT02706405) JCAR014 and Durvalumab in Treating Patients With Relapsed or Refractory B-cell Non-Hodgkin Lymphoma
2	ClinicalTrials.gov (NCT03233854) CD19/CD22 Chimeric Antigen Receptor T Cells and Chemotherapy in Treating Patients With Recurrent or Refractory CD19 Positive Diffuse Large B-Cell Lymphoma or B Acute Lymphoblastic Leukemia
3	Primary Mediastinal Large B-Cell Lymphoma With Translocations Involving BCL6 and MYC (Double-Hit Lymphoma).Am J Clin Pathol. 2016 May;145(5):710-6. doi: 10.1093/ajcp/aqw018. Epub 2016 Mar 27.
4	The utility of CD83, fascin and CD23 in the differential diagnosis of primary mediastinal large B-cell lymphoma versus classic Hodgkin lymphoma.Ann Diagn Pathol. 2019 Jun;40:72-76. doi: 10.1016/j.anndiagpath.2019.04.009. Epub 2019 Apr 23.
5	Dendritic Cell Markers and PD-L1 are Expressed in Mediastinal Gray Zone Lymphoma.Appl Immunohistochem Mol Morphol. 2018 Nov/Dec;26(10):e101-e106. doi: 10.1097/PAI.0000000000000615.
6	NFkappaB activity, function, and target-gene signatures in primary mediastinal large B-cell lymphoma and diffuse large B-cell lymphoma subtypes.Blood. 2005 Aug 15;106(4):1392-9. doi: 10.1182/blood-2004-12-4901. Epub 2005 May 3.
7	Cyclin D1-positive Mediastinal Large B-Cell Lymphoma With Copy Number Gains of CCND1 Gene: A Study of 3 Cases With Nonmediastinal Disease.Am J Surg Pathol. 2019 Jan;43(1):110-120. doi: 10.1097/PAS.0000000000001154.
8	Primary mediastinal large B-cell lymphoma: Outcome of a series of pediatric patients treated with high-dose methotrexate and cytarabine plus anti-CD20.Pediatr Blood Cancer. 2018 Feb;65(2). doi: 10.1002/pbc.26855. Epub 2017 Oct 19.
9	Programmed death-1 ligands PD-L1 and PD-L2 show distinctive and restricted patterns of expression in lymphoma subtypes.Hum Pathol. 2018 Jan;71:91-99. doi: 10.1016/j.humpath.2017.10.029. Epub 2017 Nov 6.
10	Comparative pathologic analysis of mediastinal B-cell lymphomas: selective expression of p63 but no GATA3 optimally differentiates primary mediastinal large B-cell lymphoma from classic Hodgkin lymphoma.Diagn Pathol. 2019 Dec 12;14(1):133. doi: 10.1186/s13000-019-0918-x.
11	Integrative genomic analysis identifies key pathogenic mechanisms in primary mediastinal large B-cell lymphoma.Blood. 2019 Sep 5;134(10):802-813. doi: 10.1182/blood.2019001126. Epub 2019 Jul 10.
12	Interleukin 4-induced gene 1 is activated in primary mediastinal large B-cell lymphoma.Blood. 2003 Apr 1;101(7):2756-61. doi: 10.1182/blood-2002-07-2215. Epub 2002 Nov 21.
13	J chain and myocyte enhancer factor 2B are useful in differentiating classical Hodgkin lymphoma from nodular lymphocyte predominant Hodgkin lymphoma and primary mediastinal large B-cell lymphoma.Hum Pathol. 2017 Oct;68:47-53. doi: 10.1016/j.humpath.2017.08.015. Epub 2017 Aug 26.
14	Frequent NFKBIE deletions are associated with poor outcome in primary mediastinal B-cell lymphoma.Blood. 2016 Dec 8;128(23):2666-2670. doi: 10.1182/blood-2016-03-704528. Epub 2016 Sep 26.