MCID: GRY001
MIFTS: 33

Gray Zone Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare 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, hodgkin, classic. An important gene associated with Gray Zone Lymphoma is PAX5 (Paired Box 5), and among its related pathways/superpathways are NF-kappaB Signaling and Primary immunodeficiency. The drugs Pembrolizumab and Nivolumab have been mentioned in the context of this disorder. Affiliated tissues include b cells and lung, and related phenotypes are hematopoietic system and immune system

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

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



Diseases related to Gray Zone Lymphoma

Symptoms & Phenotypes for Gray Zone Lymphoma

MGI Mouse Phenotypes related to Gray Zone Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.55 BCL11A CD274 CD79A CIITA PAX5
2 immune system MP:0005387 9.35 BCL11A CD274 CD79A CIITA PAX5
3 normal MP:0002873 8.92 CD79A CIITA PAX5 SLC22A2

Drugs & Therapeutics for Gray Zone Lymphoma

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

(show all 27)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Pembrolizumab Approved Phase 2 1374853-91-4
2
Nivolumab Approved Phase 1, Phase 2 946414-94-4
3
rituximab Approved Phase 2 174722-31-7 10201696
4
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 6055-19-2, 50-18-0 2907
5 tannic acid Approved Phase 2
6
Benzocaine Approved, Investigational Phase 2 94-09-7, 1994-09-7 2337
7
Everolimus Approved Phase 2 159351-69-6 6442177
8
Sirolimus Approved, Investigational Phase 2 53123-88-9 46835353 6436030 5284616
9
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
10 Antibodies, Monoclonal Phase 2,Phase 1
11 Immunoglobulins Phase 2,Phase 1
12 Antibodies Phase 2,Phase 1
13 Immunologic Factors Phase 1, Phase 2,Phase 2
14 Antirheumatic Agents Phase 2,Phase 1
15 Antineoplastic Agents, Alkylating Phase 1, Phase 2,Phase 2
16 Bendamustine Hydrochloride Phase 1, Phase 2,Phase 2
17 Immunosuppressive Agents Phase 1, Phase 2,Phase 2
18 Alkylating Agents Phase 1, Phase 2,Phase 2
19 Anti-Bacterial Agents Phase 2
20 Antibiotics, Antitubercular Phase 2
21 Antifungal Agents Phase 2
22 Anti-Infective Agents Phase 2
23
Fludarabine Approved Phase 1 75607-67-9, 21679-14-1 30751
24
Daunorubicin Approved Phase 1 20830-81-3 30323
25
Cytarabine Approved, Investigational Phase 1 147-94-4 6253
26 Antimetabolites, Antineoplastic Phase 1
27 Antimetabolites Phase 1

Interventional clinical trials:

(show all 12)
# 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
7 Phase II Open Label Trial to Determine Safety & Efficacy of Tisagenlecleucel in Pediatric Non-Hodgkin Lymphoma Patients Not yet recruiting NCT03610724 Phase 2
8 Brentuximab Vedotin, Bendamustine, and Rituximab in Patients With Relapsed or Refractory B-cell Non-Hodgkin Lymphoma Withdrawn NCT02623920 Phase 2 Brentuximab;Bendamustine;Rituximab
9 Durvalumab in DLBCL After Autologous Transplant Withdrawn NCT03241017 Phase 2 Durvalumab
10 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
11 Safety Study of CPX-351 in Children With Relapsed Leukemia or Lymphoma Active, not recruiting NCT01943682 Phase 1 CPX-351
12 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, Lung

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
Gray Zone Lymphoma Arising in the Neck of a Teenager With a Germline Mutation in TP53. ( 30299350 )
2018
5
Gray-zone Lymphoma Between cHL and Large B-Cell Lymphoma: A Histopathologic Series From the LYSA. ( 30540571 )
2018
6
PD-1 Blockade in Mediastinal Gray-Zone Lymphoma. ( 28679093 )
2017
7
Efficacy of High-Dose Therapy and Autologous Hematopoietic Cell Transplantation in Gray Zone Lymphoma: A US Multicenter Collaborative Study. ( 29225164 )
2017
8
Dendritic Cell Markers and PD-L1 are Expressed in Mediastinal Gray Zone Lymphoma. ( 29189264 )
2017
9
Paraneoplastic myelopathy and ophthalmoplegia secondary to gray zone lymphoma with excellent response to immuno-chemotherapy: Case report. ( 28522368 )
2017
10
Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association. ( 27758822 )
2017
11
Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL. ( 29296913 )
2017
12
Management of Primary Mediastinal B-Cell Lymphoma and Gray Zone Lymphoma. ( 28620906 )
2017
13
Gray Zone Lymphoma: Current Diagnosis and Treatment Options. ( 27888879 )
2016
14
Mediastinal Gray Zone Lymphoma with Features Intermediate between Classical Hodgkin Lymphoma and Primary Mediastinal B-Cell Lymphoma. ( 27623181 )
2016
15
Mediastinal Gray Zone Lymphoma: A Wider Category Than We Think? ( 26957476 )
2016
16
Gray-zone lymphoma: a complex reality. ( 28092884 )
2016
17
Dose adjusted-EPOCH-R and mediastinal disease may improve outcomes for patients with gray-zone lymphoma. ( 27378601 )
2016
18
The t(14;18)(q32;q21) with extra MYC signal - is it a gray zone lymphoma? ( 26464726 )
2015
19
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
20
Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach? ( 25499450 )
2015
21
Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A Proposed Scoring System. ( 26447896 )
2015
22
Mediastinal Gray Zone Lymphoma. ( 27164737 )
2015
23
A prospective study of mediastinal gray-zone lymphoma. ( 25024303 )
2014
24
Gray zone lymphoma: better treated like hodgkin lymphoma or mediastinal large B-cell lymphoma? ( 22833351 )
2012
25
Mediastinal gray zone lymphoma. ( 21454881 )
2011
26
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
27
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
28
Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma. ( 21366467 )
2011
29
Gray zone lymphoma: chromosomal aberrations with immunophenotypic and clinical correlations. ( 21822207 )
2011
30
Two cases of mediastinal gray zone lymphoma. ( 21123972 )
2010
31
Mediastinal gray zone lymphoma: the missing link between classic Hodgkin's lymphoma and mediastinal large B-cell lymphoma. ( 16224207 )
2005

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 COSM6956542 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,Hodgkin lymphoma c.529C>A p.L177M 16:11254950-11254950 3
9 COSM6986928 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.182A>T p.H61L 16:11255297-11255297 3
10 COSM6986927 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.533G>A p.C178Y 16:11254946-11254946 3
11 COSM6932501 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.274C>G p.R92G 16:11255205-11255205 3
12 COSM6986929 SOCS1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.14A>T p.N5I 16:11255465-11255465 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 COSM6932495 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.12674C>T p.T4225M 4:186596866-186596866 3
33 COSM6932494 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.12674C>T p.T4225M 4:186596866-186596866 3
34 COSM6929573 FAT1 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.8456T>C p.V2819A 4:186618130-186618130 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 COSM5383385 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.67+2T>C p.? 15:44711615-44711615 3
49 COSM3077774 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.295T>C p.C99R 6:394899-394899 3
50 COSM6936874 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,diffuse large B cell lymphoma c.2228A>C p.D743A 12:18399760-18399760 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 11.44 CD274 CD79A CIITA PAX5
2 10.52 CD79A CIITA

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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