MCID: MDS006
MIFTS: 32

Mediastinal Gray Zone Lymphoma

Categories: Cancer diseases, Immune diseases

Aliases & Classifications for Mediastinal Gray Zone Lymphoma

MalaCards integrated aliases for Mediastinal Gray Zone Lymphoma:

Name: Mediastinal Gray Zone Lymphoma 12 14
Mediastinal B-Cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma 69

Classifications:



External Ids:

Disease Ontology 12 DOID:6867
NCIt 47 C37870
UMLS 69 C1334657

Summaries for Mediastinal Gray Zone Lymphoma

MalaCards based summary : Mediastinal Gray Zone Lymphoma, also known as mediastinal b-cell lymphoma, unclassifiable, with features intermediate between diffuse large b-cell lymphoma and classical hodgkin lymphoma, is related to mediastinitis and vulvovaginal gingival syndrome. An important gene associated with Mediastinal Gray Zone Lymphoma is PAX5 (Paired Box 5), and among its related pathways/superpathways are NF-kappaB Signaling and Interleukin-4 and 13 signaling. The drugs nivolumab and rituximab have been mentioned in the context of this disorder. Affiliated tissues include b cells and t cells, and related phenotypes are Decreased substrate adherent cell growth and cellular

Related Diseases for Mediastinal Gray Zone Lymphoma

Diseases related to Mediastinal Gray Zone Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 32)
id Related Disease Score Top Affiliating Genes
1 mediastinitis 10.5
2 vulvovaginal gingival syndrome 10.5 BCL6 PAX5
3 lymphoma 10.4
4 gray zone lymphoma 10.4
5 ischemic optic neuropathy 10.4 BCL6 PAX5
6 chromosome 2p16.1-p15 deletion syndrome 10.3 BCL11A REL XPO1
7 pediatric lymphoma 10.3 BCL11A BCL6 PAX5
8 b-cell lymphomas 10.3
9 multiple congenital anomalies mental retardation, growth failure and cleft lip palate 10.3 BCL6 PAX5
10 malignant sertoli-leydig cell tumor 10.2 PAX5 TNFRSF8
11 primary mediastinal large b-cell lymphoma 10.2
12 systemic mastocytosis with an associated clonal hematologic non-mast cell lineage disease 10.1 BCL6 CD274 JAK2
13 hodgkin lymphoma 10.1
14 breast lymphoma 10.0 BCL11A CD274 EPHA7 PAX5 SLC22A2
15 primary malignant melanoma of the cervix 10.0 BCL6 PAX5
16 tang hsi ryu syndrome 10.0 BCL6 SLC22A2 TNFRSF8
17 villoglandular endometrial endometrioid adenocarcinoma 9.9 BCL6 PAX5 TNFRSF8
18 penile disease 9.9 BCL6 TNFRSF8
19 inner ear cancer 9.9 FUT4 TNFRSF8
20 hodgkin's lymphoma, lymphocytic-histiocytic predominance 9.9 BCL6 PAX5 TNFRSF8
21 chronic intestinal vascular insufficiency 9.8 FUT4 PAX5 TNFRSF8
22 ovarian cystic teratoma 9.7 FUT4 TNFRSF8
23 chondrosarcoma, extraskeletal myxoid 9.7 CD274 JAK2 PDCD1LG2
24 childhood spinal cord tumor 9.6 BCL6 FUT4 TNFRSF8
25 drug-induced hepatitis 9.6 BCL6 PAX5 REL TNFRSF8
26 autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome 9.6 CD274 JAK2 PDCD1LG2
27 bipolar disorder 9.6 BCL6 PAX5 REL TNFRSF8
28 carnitine-acylcarnitine translocase deficiency 9.5 BCL6 PAX5 REL TNFRSF8
29 hemolytic anemia 9.5 JAK2 PAX5 TNFRSF8
30 pyridoxine-refractory autosomal recessive sideroblastic anemia 9.3 BCL11A BCL6 PAX5 REL TNFRSF8
31 spinal cord neuroblastoma 5.0 BCL11A BCL6 CD274 DAPK1 EPHA7 FUT4
32 hepatoblastoma 4.8 BCL11A BCL6 CD274 DAPK1 EPHA7 FUT4

Graphical network of the top 20 diseases related to Mediastinal Gray Zone Lymphoma:



Diseases related to Mediastinal Gray Zone Lymphoma

Symptoms & Phenotypes for Mediastinal Gray Zone Lymphoma

GenomeRNAi Phenotypes related to Mediastinal Gray Zone Lymphoma according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased substrate adherent cell growth GR00193-A-2 9.35 DAPK1 EPHA7 JAK2
2 Decreased substrate adherent cell growth GR00193-A-3 9.35 DAPK1 JAK2
3 Increased cell death HMECs cells GR00103-A-0 9.02 BCL6 DAPK1 EPHA7 JAK2 XPO1

MGI Mouse Phenotypes related to Mediastinal Gray Zone Lymphoma:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.96 BCL11A BCL6 CD274 DAPK1 FUT4 HMOX1
2 hematopoietic system MP:0005397 9.93 BCL11A BCL6 CD274 FUT4 GPR15 HMOX1
3 homeostasis/metabolism MP:0005376 9.7 MAOA PDCD1LG2 REL SLC22A2 BCL11A BCL6
4 immune system MP:0005387 9.4 BCL6 CD274 FUT4 GPR15 HMOX1 HOXA5

Drugs & Therapeutics for Mediastinal Gray Zone Lymphoma

Drugs for Mediastinal Gray Zone Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 10)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
nivolumab Approved Phase 1, Phase 2 946414-94-4
2
rituximab Approved Phase 2 174722-31-7 10201696
3 Antibodies Phase 1, Phase 2
4 Antibodies, Monoclonal Phase 1, Phase 2
5 Immunoglobulins Phase 1, Phase 2
6 Antirheumatic Agents Phase 2,Phase 1
7
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
8
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
9 Alkylating Agents Phase 1
10 Immunosuppressive Agents Phase 1

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 An Investigational Immuno-therapy Safety and Effectiveness Study of Nivolumab in Combination With Brentuximab Vedotin to Treat Non-Hodgkin Lymphomas Recruiting NCT02581631 Phase 1, Phase 2 Brentuximab Vedotin
2 Dose-Adjusted EPOCH Chemotherapy and Rituximab (CD20+) in Previously Untreated Aggressive Non-Hodgkin's Lymphoma Recruiting NCT00001337 Phase 2 EPOCH
3 A Phase I/II Study Of Brentuximab Vedotin In Combination With Multi-Agent Chemotherapy As Front-Line Treatment In Patients With CD30 Positive Primary Mediastinal Large B-Cell, Diffuse Large B-Cell, And Grey Zone Lymphomas Active, not recruiting NCT01994850 Phase 1, Phase 2 brentuximab vedotin
4 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
5 Mediastinal Grey Zone Lymphoma From the LYSA Completed NCT03037177

Search NIH Clinical Center for Mediastinal Gray Zone Lymphoma

Genetic Tests for Mediastinal Gray Zone Lymphoma

Anatomical Context for Mediastinal Gray Zone Lymphoma

MalaCards organs/tissues related to Mediastinal Gray Zone Lymphoma:

39
B Cells, T Cells

Publications for Mediastinal Gray Zone Lymphoma

Articles related to Mediastinal Gray Zone Lymphoma:

(show all 15)
id Title Authors Year
1
PD-1 Blockade in Mediastinal Gray-Zone Lymphoma. ( 28679093 )
2017
2
Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association. ( 27758822 )
2017
3
Mediastinal Gray Zone Lymphoma with Features Intermediate between Classical Hodgkin Lymphoma and Primary Mediastinal B-Cell Lymphoma. ( 27623181 )
2016
4
Mediastinal Gray Zone Lymphoma: A Wider Category Than We Think? ( 26957476 )
2016
5
Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach? ( 25499450 )
2015
6
Mediastinal Gray Zone Lymphoma. ( 27164737 )
2015
7
A prospective study of mediastinal gray-zone lymphoma. ( 25024303 )
2014
8
Clinical, pathological and genetic features of primary mediastinal large B-cell lymphomas and mediastinal gray zone lymphomas in children. ( 20971819 )
2011
9
Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma. ( 21366467 )
2011
10
Primary mediastinal large B-cell lymphoma, classic Hodgkin lymphoma presenting in the mediastinum, and mediastinal gray zone lymphoma: what is the oncologist to do? ( 21590365 )
2011
11
Methylation profiling of mediastinal gray zone lymphoma reveals a distinctive signature with elements shared by classical Hodgkin's lymphoma and primary mediastinal large B-cell lymphoma. ( 21454882 )
2011
12
Mediastinal gray zone lymphoma. ( 21454881 )
2011
13
Two cases of mediastinal gray zone lymphoma. ( 21123972 )
2010
14
Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. ( 16224207 )
2005
15
Molecular profiling provides evidence of primary mediastinal large B-cell lymphoma as a distinct entity related to classic Hodgkin lymphoma: implications for mediastinal gray zone lymphomas as an intermediate form of B-cell lymphoma. ( 15322489 )
2004

Variations for Mediastinal Gray Zone Lymphoma

Expression for Mediastinal Gray Zone Lymphoma

Search GEO for disease gene expression data for Mediastinal Gray Zone Lymphoma.

Pathways for Mediastinal Gray Zone Lymphoma

GO Terms for Mediastinal Gray Zone Lymphoma

Cellular components related to Mediastinal Gray Zone Lymphoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 endomembrane system GO:0012505 8.8 CD274 JAK2 PDCD1LG2

Biological processes related to Mediastinal Gray Zone Lymphoma according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription from RNA polymerase II promoter GO:0000122 9.72 BCL11A BCL6 PAX5 REL XPO1
2 negative regulation of interferon-gamma production GO:0032689 9.37 CD274 PDCD1LG2
3 negative regulation of interleukin-10 production GO:0032693 9.26 CD274 PDCD1LG2
4 negative regulation of activated T cell proliferation GO:0046007 9.16 CD274 PDCD1LG2
5 positive regulation of apoptotic process GO:0043065 9.1 BCL6 EPHA7 HMOX1 HOXA5 JAK2 TNFRSF8
6 negative regulation of mast cell cytokine production GO:0032764 8.96 BCL6 HMOX1

Sources for Mediastinal Gray Zone Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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