MCID: GRY001
MIFTS: 29

Gray Zone Lymphoma

Categories: Rare diseases, Cancer diseases, Immune diseases

Aliases & Classifications for Gray Zone Lymphoma

MalaCards integrated aliases for Gray Zone Lymphoma:

Name: Gray Zone Lymphoma 12 53 15
B-Cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma 73

Classifications:



External Ids:

Disease Ontology 12 DOID:5822
NCIt 50 C37869
UMLS 73 C1333878

Summaries for Gray Zone Lymphoma

NIH Rare Diseases : 53 Gray zone lymphoma is a rare type of lymphoma, cancer of a part of the immune system called the lymph system. It is called "gray zone" lymphoma because it has features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma (DLBCL), but cannot be assigned specifically to either type. In many cases, the original diagnosis of gray zone lymphoma is later reclassified as a different type of lymphoma, such as nodular sclerosis classical Hodgkin lymphoma (NScHL). An accurate diagnosis of gray zone lymphoma is challenging, and the clinical characteristics, optimum therapy, and prognosis have not been well-defined. While some features suggest it should be treated like Hodgkin lymphoma, other features suggest it should be treated like DLBCL.

MalaCards based summary : Gray Zone Lymphoma, also known as b-cell lymphoma, unclassifiable, with features intermediate between diffuse large b-cell lymphoma and classical hodgkin lymphoma, is related to mediastinal gray zone lymphoma and lymphoma. An important gene associated with Gray Zone Lymphoma is PAX5 (Paired Box 5), and among its related pathways/superpathways is NF-kappaB Signaling. The drugs rituximab and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include b cells and t cells.

Disease Ontology : 12 A lymphoma that is characterized by having cellular features of both classic Hodgkin's lymphomas and large B-cell Lymphomas.

Wikipedia : 76 Gray zone lymphoma, often presenting as large tumors in the mediastinum, is a type of lymphoma that is... more...

Related Diseases for Gray Zone Lymphoma

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

# Related Disease Score Top Affiliating Genes
1 mediastinal gray zone lymphoma 31.7 CD274 EPHA7 PAX5 SLC22A2
2 lymphoma 10.6
3 mediastinitis 10.5
4 b-cell lymphomas 10.4
5 lymphoma, hodgkin, classic 10.2
6 primary mediastinal large b-cell lymphoma 10.2
7 primary mediastinal b-cell lymphoma 10.0
8 diffuse large b-cell lymphoma 9.8
9 merkel cell carcinoma 9.5 CD274 PAX5
10 mediastinal malignant lymphoma 8.4 BCL11A CD274 EPHA7 PAX5

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



Diseases related to Gray Zone Lymphoma

Symptoms & Phenotypes for Gray Zone Lymphoma

Drugs & Therapeutics for Gray Zone Lymphoma

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

(show all 24)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 2 174722-31-7 10201696
2
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 50-18-0, 6055-19-2 2907
3
Pembrolizumab Approved Phase 2 1374853-91-4
4
nivolumab Approved Phase 1, Phase 2 946414-94-4
5
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
6
Everolimus Approved Phase 2 159351-69-6 6442177
7
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030 46835353
9 tannic acid Approved, Nutraceutical Phase 2
10 Alkylating Agents Phase 1, Phase 2,Phase 2
11 Antibodies Phase 2,Phase 1
12 Immunoglobulins Phase 2,Phase 1
13 Antibodies, Monoclonal Phase 2,Phase 1
14 Immunosuppressive Agents Phase 1, Phase 2,Phase 2
15 Antirheumatic Agents Phase 2,Phase 1
16 Bendamustine Hydrochloride Phase 1, Phase 2,Phase 2
17 Antineoplastic Agents, Alkylating Phase 1, Phase 2,Phase 2
18 Anti-Bacterial Agents Phase 2
19 Antibiotics, Antitubercular Phase 2
20 Anti-Infective Agents Phase 2
21 Antifungal Agents Phase 2
22
Fludarabine Approved Phase 1 21679-14-1, 75607-67-9 30751
23
Cytarabine Approved, Investigational Phase 1 147-94-4 6253
24
Daunorubicin Approved Phase 1 20830-81-3 30323

Interventional clinical trials:

(show all 11)
# Name Status NCT ID Phase Drugs
1 Pembrolizumab in Relapsed and Refractory Gray-Zone Lymphoma (GZL), Primary Central Nervous System Lymphoma (PCNSL), and Other Extranodal Diffuse Large B-cell Lymphomas Recruiting NCT03255018 Phase 2 Pembrolizumab
2 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
3 Dose-Adjusted EPOCH Chemotherapy and Rituximab (CD20+) in Previously Untreated Aggressive Non-Hodgkin's Lymphoma Recruiting NCT00001337 Phase 2 EPOCH
4 Haploidentical BMT With Post-Transplant Cyclophosphamide and Bendamustine Recruiting NCT02996773 Phase 1, Phase 2 Bendamustine;Cyclophosphamide
5 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
6 Maintenance Rituximab With mTor Inhibition After High-dose Consolidative Therapy in Lymphoma Active, not recruiting NCT01665768 Phase 2 Everolimus and Rituximab
7 Brentuximab Vedotin, Bendamustine, and Rituximab in Patients With Relapsed or Refractory B-cell Non-Hodgkin Lymphoma Withdrawn NCT02623920 Phase 2 Brentuximab;Bendamustine;Rituximab
8 Durvalumab in DLBCL After Autologous Transplant Withdrawn NCT03241017 Phase 2 Durvalumab
9 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
10 Safety Study of CPX-351 in Children With Relapsed Leukemia or Lymphoma Active, not recruiting NCT01943682 Phase 1 CPX-351
11 Mediastinal Grey Zone Lymphoma From the LYSA Completed NCT03037177

Search NIH Clinical Center for Gray Zone Lymphoma

Genetic Tests for Gray Zone Lymphoma

Anatomical Context for Gray Zone Lymphoma

MalaCards organs/tissues related to Gray Zone Lymphoma:

41
B Cells, T Cells

Publications for Gray Zone Lymphoma

Articles related to Gray Zone Lymphoma:

(show all 31)
# Title Authors Year
1
Long-term complete remission following tandem autologous stem cell transplantation and consolidative radiotherapy for refractory mediastinal gray-zone lymphoma. ( 29786758 )
2018
2
Favorable response of relapsed/refractory gray-zone lymphoma to brentuximab vedotin. ( 29198035 )
2018
3
Expression pattern of PD-L1 and PD-L2 in classical Hodgkin lymphoma, primary mediastinal large B-cell lymphoma, and gray zone lymphoma. ( 29377256 )
2018
4
PD-1 Blockade in Mediastinal Gray-Zone Lymphoma. ( 28679093 )
2017
5
Efficacy of High-Dose Therapy and Autologous Hematopoietic Cell Transplantation in Gray Zone Lymphoma: A US Multicenter Collaborative Study. ( 29225164 )
2017
6
Dendritic Cell Markers and PD-L1 are Expressed in Mediastinal Gray Zone Lymphoma. ( 29189264 )
2017
7
Paraneoplastic myelopathy and ophthalmoplegia secondary to gray zone lymphoma with excellent response to immuno-chemotherapy: Case report. ( 28522368 )
2017
8
Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association. ( 27758822 )
2017
9
Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL. ( 29296913 )
2017
10
Management of Primary Mediastinal B-Cell Lymphoma and Gray Zone Lymphoma. ( 28620906 )
2017
11
Gray Zone Lymphoma: Current Diagnosis and Treatment Options. ( 27888879 )
2016
12
Mediastinal Gray Zone Lymphoma with Features Intermediate between Classical Hodgkin Lymphoma and Primary Mediastinal B-Cell Lymphoma. ( 27623181 )
2016
13
Mediastinal Gray Zone Lymphoma: A Wider Category Than We Think? ( 26957476 )
2016
14
Gray-zone lymphoma: a complex reality. ( 28092884 )
2016
15
Dose adjusted-EPOCH-R and mediastinal disease may improve outcomes for patients with gray-zone lymphoma. ( 27378601 )
2016
16
The t(14;18)(q32;q21) with extra MYC signal - is it a gray zone lymphoma? ( 26464726 )
2015
17
Gray zone lymphoma with features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma: characteristics, outcomes, and prognostication among a large multicenter cohort. ( 26044261 )
2015
18
Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach? ( 25499450 )
2015
19
Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A Proposed Scoring System. ( 26447896 )
2015
20
Mediastinal Gray Zone Lymphoma. ( 27164737 )
2015
21
A prospective study of mediastinal gray-zone lymphoma. ( 25024303 )
2014
22
Gray zone lymphoma: better treated like hodgkin lymphoma or mediastinal large B-cell lymphoma? ( 22833351 )
2012
23
Mediastinal gray zone lymphoma. ( 21454881 )
2011
24
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
25
Clinical, pathological and genetic features of primary mediastinal large B-cell lymphomas and mediastinal gray zone lymphomas in children. ( 20971819 )
2011
26
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
27
Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma. ( 21366467 )
2011
28
Gray zone lymphoma: chromosomal aberrations with immunophenotypic and clinical correlations. ( 21822207 )
2011
29
Two cases of mediastinal gray zone lymphoma. ( 21123972 )
2010
30
Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. ( 16224207 )
2005
31
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 Gray Zone Lymphoma

Cosmic variations for Gray Zone Lymphoma:

9
(show top 50) (show all 80)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM96797 XPO1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1711G>A p.E571K 2:61492337-61492337 3
2 COSM45892 TP53 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.857A>C p.E286A 17:7673763-7673763 3
3 COSM45652 TP53 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.914A>C p.K305T 17:7673706-7673706 3
4 COSM35925 TNFAIP3 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1004T>G p.L335* 6:137878449-137878449 3
5 COSM35928 TNFAIP3 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2343A>T p.E781D 6:137881289-137881289 3
6 COSM35930 TNFAIP3 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.569C>A p.S190* 6:137875770-137875770 3
7 COSM6932498 SOX17 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.146C>T p.P49L 8:54458284-54458284 3
8 COSM6986929 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.14A>T p.N5I 16:11255465-11255465 3
9 COSM6956542 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,Hodgkin lymphoma c.529C>A p.L177M 16:11254950-11254950 3
10 COSM6986928 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.182A>T p.H61L 16:11255297-11255297 3
11 COSM6986927 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.533G>A p.C178Y 16:11254946-11254946 3
12 COSM6932501 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.274C>G p.R92G 16:11255205-11255205 3
13 COSM6932499 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.550G>A p.A184T 16:11254929-11254929 3
14 COSM6936864 RAD50 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2443A>G p.I815V 5:132609147-132609147 3
15 COSM6936879 RAB35 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.262T>G p.Y88D 12:120099120-120099120 3
16 COSM6932496 PIM1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.427C>A p.L143M 6:37171311-37171311 3
17 COSM6936865 PIM1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.202C>T p.H68Y 6:37170993-37170993 3
18 COSM1581454 PIK3CD haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.3061G>A p.E1021K 1:9726972-9726972 3
19 COSM6936881 PIK3C3 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.452C>T p.A151V 18:41970377-41970377 3
20 COSM6936872 PIK3C2G haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1175T>G p.L392R 12:18320999-18320999 3
21 COSM6936878 PIK3C2G haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2366C>A p.A789D 12:18424024-18424024 3
22 COSM6936875 PIK3C2G haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2105A>C p.D702A 12:18399760-18399760 3
23 COSM6929577 NTRK2 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1912C>T p.H638Y 9:84948609-84948609 3
24 COSM6932493 MYCN haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1178G>A p.R393H 2:15945880-15945880 3
25 COSM6929580 MRE11 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.757C>T p.P253S 11:94471662-94471662 3
26 COSM6936868 KMT2C haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.34C>T p.P12S 7:152435753-152435753 3
27 COSM12600 JAK2 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1849G>T p.V617F 9:5073770-5073770 3
28 COSM6936858 JAK1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1208T>G p.L403W 1:64860231-64860231 3
29 COSM3410849 HIST1H3F haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.191G>A p.R64H 6:26250415-26250415 3
30 COSM6932497 HGF haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1978G>T p.G660W 7:81705422-81705422 3
31 COSM6936880 FOXA1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1219T>C p.S407P 14:37591565-37591565 3
32 COSM6929573 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.8456T>C p.V2819A 4:186618130-186618130 3
33 COSM6932495 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.12674C>T p.T4225M 4:186596866-186596866 3
34 COSM6932494 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.12674C>T p.T4225M 4:186596866-186596866 3
35 COSM6929574 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.8456T>C p.V2819A 4:186618130-186618130 3
36 COSM37028 EZH2 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1937A>T p.Y646F 7:148811635-148811635 3
37 COSM6936862 ERBB4 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.1904A>G p.Y635C 2:211657796-211657796 3
38 COSM231560 DNMT3A haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2206C>T p.R736C 2:25240418-25240418 3
39 COSM6986923 CDKN2B haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.68C>G p.A23G 9:22008886-22008886 3
40 COSM12475 CDKN2A haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.238C>T p.R80* 9:21971121-21971121 3
41 COSM6929581 CDKN1B haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.322G>A p.D108N 12:12718161-12718161 3
42 COSM6929588 BCL2 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.40A>C p.I14L 18:63318627-63318627 3
43 COSM4170969 BCL2 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.4G>C p.A2P 18:63318663-63318663 3
44 COSM144522 B2M haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2T>A p.M1K 15:44711548-44711548 3
45 COSM5383384 B2M haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.67+2T>C p.? 15:44711615-44711615 3
46 COSM6929579 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.757C>T p.P253S 11:94471662-94471662 3
47 COSM99935 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.404C>T p.P135L 9:21971121-21971121 3
48 COSM4243207 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.3133G>A p.E1045K 1:9726972-9726972 3
49 COSM99191 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.238C>T p.R80* 9:21971121-21971121 3
50 COSM3077774 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.295T>C p.C99R 6:394899-394899 3

Expression for Gray Zone Lymphoma

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

Pathways for Gray Zone Lymphoma

Pathways related to Gray Zone Lymphoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.31 CD274 CIITA PAX5

GO Terms for Gray Zone Lymphoma

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

# Name GO ID Score Top Affiliating Genes
1 aging GO:0007568 8.96 CIITA PAX5
2 negative regulation of transcription by RNA polymerase II GO:0000122 8.8 BCL11A CIITA PAX5

Sources for Gray Zone Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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