MCID: GRY001
MIFTS: 34

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 20 15
B-Cell Lymphoma, Unclassifiable, with Features Intermediate Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma 70

Classifications:



External Ids:

Disease Ontology 12 DOID:5822
NCIt 50 C37869
SNOMED-CT 67 722954005
UMLS 70 C1333878

Summaries for Gray Zone Lymphoma

GARD : 20 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 IgA-Producing B Cells in the Intestine. The drugs tannic acid and Everolimus have been mentioned in the context of this disorder. Affiliated tissues include kidney and testis, and related phenotypes are hematopoietic system and homeostasis/metabolism

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

Wikipedia : 73 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:

(show top 50) (show all 110)
# Related Disease Score Top Affiliating Genes
1 mediastinal gray zone lymphoma 31.4 TNFRSF8 SLC22A2 POU2AF1 PAX5 GPR15 FUT4
2 lymphoma, hodgkin, classic 30.8 TNFRSF8 POU2AF1 PAX5 BCL6
3 lymphoma 30.5 TNFRSF8 PAX5 CD274 BCL6
4 primary mediastinal large b-cell lymphoma 30.1 PDCD1LG2 CD274 BCL6
5 hodgkin's lymphoma, lymphocytic-histiocytic predominance 30.0 TNFRSF8 FUT4 BCL6
6 lymphoma, non-hodgkin, familial 29.7 TNFRSF8 PAX5 MME CD79A BCL6
7 b-cell lymphoma 29.7 TNFRSF8 PAX5 MME CIITA CD274 BCL6
8 hodgkin's lymphoma, nodular sclerosis 29.5 TNFRSF8 PAX5 FUT4 CD79A BCL6
9 immune deficiency disease 29.3 TNFRSF8 GPR15 CIITA CD79A BCL6
10 diffuse large b-cell lymphoma 29.2 TNFRSF8 PAX5 MME CIITA CD79A CD274
11 composite lymphoma 28.6 TNFRSF8 PAX5 MME FUT4 CD79A CD274
12 primary mediastinal b-cell lymphoma 10.4
13 submandibular adenitis 10.3 TNFRSF8 PAX5
14 breast lipoma 10.3 TNFRSF8 PAX5
15 hodgkin's lymphoma, mixed cellularity 10.2 TNFRSF8 FUT4
16 monoclonal paraproteinemia 10.2 PAX5 CD79A
17 pleural lipoma 10.2 TNFRSF8 FUT4
18 infected hydrocele 10.2 TNFRSF8 CD79A
19 uterus leiomyosarcoma 10.2 TNFRSF8 FUT4
20 uterine corpus sarcoma 10.2 TNFRSF8 FUT4
21 primary cutaneous b-cell lymphoma 10.2 PAX5 BCL6
22 retroperitoneal lymphoma 10.2 TNFRSF8 MME
23 lymphosarcoma 10.2 TNFRSF8 BCL6
24 primary central nervous system lymphoma 10.1 PAX5 BCL6
25 orbital cellulitis 10.1 TNFRSF8 MME
26 acute orbital inflammation 10.1 TNFRSF8 MME
27 hilar lung carcinoma 10.1 MME CD79A
28 lung hilum cancer 10.1 MME CD79A
29 b-cell non-hodgkin lymphoma 10.1
30 alpha chain disease 10.1 CD79A BCL6
31 intraocular lymphoma 10.1 PAX5 BCL6
32 liver lymphoma 10.1 MME CD79A
33 multicentric castleman disease 10.1 TNFRSF8 PAX5 CD79A
34 nephrogenic adenoma of the urethra 10.1 MME FUT4
35 mucinous tubular and spindle renal cell carcinoma 10.1 MME FUT4
36 sclerosing hepatic carcinoma 10.1 MME CD79A
37 mental retardation, autosomal dominant 33 10.1 PAX5 MME
38 urethral benign neoplasm 10.1 MME FUT4
39 central epithelioid sarcoma 10.1 TNFRSF8 MME
40 ureteral lymphoma 10.1 MME BCL6
41 vulvar proximal-type epithelioid sarcoma 10.1 TNFRSF8 MME
42 chest wall lymphoma 10.1 MME BCL6
43 epididymis adenocarcinoma 10.1 MME FUT4
44 prostate lymphoma 10.1 MME BCL6
45 epididymis cancer 10.1 MME FUT4
46 heart lymphoma 10.1 MME BCL6
47 ovarian lymphoma 10.1 MME BCL6
48 reticulosarcoma 10.1 TNFRSF8 PAX5 BCL6
49 appendix lymphoma 10.1 MME BCL6
50 intravascular large b-cell lymphoma 10.0 MME BCL6

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 9.85 BCL6 CD274 CD79A CIITA FUT4 GPR15
2 homeostasis/metabolism MP:0005376 9.65 BCL6 CD274 CD79A CIITA FUT4 GPR15
3 immune system MP:0005387 9.36 BCL6 CD274 CD79A CIITA FUT4 GPR15

Drugs & Therapeutics for Gray Zone Lymphoma

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

(show all 18)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
tannic acid Approved Phase 2 1401-55-4
2
Everolimus Approved Phase 2 159351-69-6 6442177 70789204
3
rituximab Approved Phase 2 174722-31-7 10201696
4
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
5
Pembrolizumab Approved Phase 2 1374853-91-4
6 Immunologic Factors Phase 2
7 Immunosuppressive Agents Phase 2
8 Antirheumatic Agents Phase 2
9 Antineoplastic Agents, Immunological Phase 2
10 Antibodies Phase 2
11 Immunoglobulins Phase 2
12 Antibodies, Monoclonal Phase 2
13
Lenalidomide Approved Phase 1 191732-72-6 216326
14
Blinatumomab Approved, Investigational Phase 1 853426-35-4
15 Angiogenesis Inhibitors Phase 1
16 Immunoglobulins, Intravenous Phase 1
17 Muromonab-CD3 Phase 1
18 Antibodies, Bispecific Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Trial of Maintenance Rituximab With mTor Inhibition After High-dose Consolidative Therapy in CD20+, B-cell Lymphomas, Gray Zone Lymphoma, and Hodgkin's Lymphoma Completed NCT01665768 Phase 2 Everolimus
2 Phase 2 Trial of Pembrolizumab in Relapsed and Refractory Gray-Zone Lymphoma (GZL), Primary Central Nervous System Lymphoma(PCNSL), and Other Extranodal Diffuse Large B-cell Lymphomas Active, not recruiting NCT03255018 Phase 2
3 A Phase I/Ib Study of the PD-1 Antibody Pembrolizumab in Combination With Ibrutinib in Relapsed/Refractory Non-Hodgkin's Lymphoma (NHL) Completed NCT02950220 Phase 1 Ibrutinib
4 A Phase I Trial of the Combination of Lenalidomide and Blinatumomab in Patients With Relapsed or Refractory Non-Hodgkins Lymphoma (NHL) Recruiting NCT02568553 Phase 1 Lenalidomide

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:

40
Kidney, Testis

Publications for Gray Zone Lymphoma

Articles related to Gray Zone Lymphoma:

(show top 50) (show all 88)
# Title Authors PMID Year
1
Mutational landscape of gray zone lymphoma. 61
32961552 2021
2
Secondary cutaneous involvement by direct extension in high-grade B-cell lymphomas. 61
32639608 2021
3
Unraveling the Mystery of Gray Zone Lymphoma in Human Immunodeficiency Virus-Seropositive Patients: Two Cases. 61
33224393 2020
4
Primary Mediastinal (Thymic) Large B-Cell Lymphoma: Fidelity of Diagnosis Using WHO Criteria. 61
33487576 2020
5
EBV-Positive Primary Large B-Cell Lymphoma: The Role of Immunohistochemistry and XPO1 in the Diagnosis of Mediastinal Lymphomas. 61
31789821 2020
6
Pediatric Lymphoma and Solid Tumors Associated With Cancer Susceptibility Syndromes. 61
32282651 2020
7
Nodal diffuse large B-cell lymphoma with neoplastic PD-L1 positivity, but without EBV association: Three cases highlighting an aspect of gray zone lymphoma. 61
32623805 2020
8
Gene Expression Profiling of Mediastinal Gray Zone Lymphoma and Its Relationship to Primary Mediastinal B-cell Lymphoma and Classical Hodgkin Lymphoma. 61
32914098 2020
9
Lenograstim-Induced Nodal Extramedullary Hematopoiesis: A Challenging Diagnosis in Lymphoma Evaluation With 18F-FDG PET/CT. 61
32558708 2020
10
Use of checkpoint inhibitors in gray zone lymphoma. 61
32589932 2020
11
Immune checkpoint inhibitors in malignant lymphoma: Advances and perspectives. 61
32694896 2020
12
Gene expression profiling of gray zone lymphoma. 61
32516416 2020
13
Brentuximab vedotin in combination with rituximab, cyclophosphamide, doxorubicin, and prednisone as frontline treatment for patients with CD30-positive B-cell lymphomas. 61
32414850 2020
14
Management of children and adolescents with gray zone lymphoma: A case series. 61
32037692 2020
15
Diagnostic utility of STAT6YE361 expression in classical Hodgkin lymphoma and related entities. 61
31822802 2020
16
Pruritic cutaneous lesions mimicking dermatitis artefacta in the course of mediastinal gray-zone lymphoma. 61
32489369 2020
17
Activating the Antitumor Immune Response in Non-Hodgkin Lymphoma Using Immune Checkpoint Inhibitors. 61
33294467 2020
18
"Do Not Ignore Musculoskeletal Pain"-Initial Presentation of a Rare Malignant Disease: Case Report and Literature Review. 61
32660277 2020
19
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. 61
31831043 2019
20
Anaplastic variant of diffuse large B-cell lymphoma: Reappraisal as a nodal disease with sinusoidal involvement. 61
31872533 2019
21
Primary Mediastinal Nodal and Extranodal Non-Hodgkin Lymphomas: Current Concepts, Historical Evolution, and Useful Diagnostic Approach: Part 1. 61
31567132 2019
22
Primary Mediastinal Nodal and Extranodal Non-Hodgkin Lymphomas: Current Concepts, Historical Evolution, and Useful Diagnostic Approach: Part 2. 61
31567129 2019
23
Long-term Remission by Brentuximab Vedotin for Non-mediastinal Gray Zone Lymphoma Refractory to Autologous Stem Cell Transplantation. 61
31551171 2019
24
Transformation of monoclonal B lymphocytosis to Epstein-Barr virus-positive large B-cell lymphoma with intermediate features between diffuse large B-cell lymphoma and classic Hodgkin lymphoma. 61
33437870 2019
25
Challenges in the Diagnosis of Gray Zone Lymphomas. 61
31352983 2019
26
Gray Zone Lymphoma Arising in the Neck of a Teenager With a Germline Mutation in TP53. 61
30299350 2019
27
The utility of CD83, fascin and CD23 in the differential diagnosis of primary mediastinal large B-cell lymphoma versus classic Hodgkin lymphoma. 61
31075666 2019
28
Gray-zone lymphoma. Examples of rare clinical manifestation. 61
31094484 2019
29
GATA3 Immunohistochemical Staining in Hodgkin Lymphoma: Diagnostic Utility in Differentiating Classic Hodgkin Lymphoma From Nodular Lymphocyte Predominant Hodgkin Lymphoma and Other Mimicking Entities. 61
28877074 2019
30
Gray-zone Lymphoma Between cHL and Large B-Cell Lymphoma: A Histopathologic Series From the LYSA. 61
30540571 2019
31
Dendritic Cell Markers and PD-L1 are Expressed in Mediastinal Gray Zone Lymphoma. 61
29189264 2018
32
Long-term complete remission following tandem autologous stem cell transplantation and consolidative radiotherapy for refractory mediastinal gray-zone lymphoma. 61
29786758 2018
33
Expression pattern of PD-L1 and PD-L2 in classical Hodgkin lymphoma, primary mediastinal large B-cell lymphoma, and gray zone lymphoma. 61
29377256 2018
34
Favorable response of relapsed/refractory gray-zone lymphoma to brentuximab vedotin. 61
29198035 2018
35
Efficacy of High-Dose Therapy and Autologous Hematopoietic Cell Transplantation in Gray Zone Lymphoma: A US Multicenter Collaborative Study. 61
29225164 2018
36
Primary mediastinal B-cell lymphoma: biology and evolving therapeutic strategies. 61
29222270 2017
37
Clinicopathologic consensus study of gray zone lymphoma with features intermediate between DLBCL and classical HL. 61
29296913 2017
38
Dose adjusted-EPOCH-R and mediastinal disease may improve outcomes for patients with gray-zone lymphoma. 61
27378601 2017
39
Monoamine oxidase A is highly expressed in classical Hodgkin lymphoma. 61
28722111 2017
40
Paraneoplastic myelopathy and ophthalmoplegia secondary to gray zone lymphoma with excellent response to immuno-chemotherapy: Case report. 61
28522368 2017
41
PD-1 Blockade in Mediastinal Gray-Zone Lymphoma. 61
28679093 2017
42
Management of Primary Mediastinal B-Cell Lymphoma and Gray Zone Lymphoma. 61
28620906 2017
43
A case of composite classical and nodular lymphocyte predominant Hodgkin lymphoma with progression to diffuse large B-cell non-Hodgkin lymphoma: Diagnostic difficulty in fine-needle aspiration cytology. 61
27888658 2017
44
Mediastinal gray zone lymphoma: clinico-pathological characteristics and outcomes of 99 patients from the Lymphoma Study Association. 61
27758822 2017
45
Gray Zone Lymphoma: Current Diagnosis and Treatment Options. 61
27888879 2016
46
The immunophenotypic spectrum of primary mediastinal large B-cell lymphoma reveals prognostic biomarkers associated with outcome. 61
27419920 2016
47
Recurrent mutations of the exportin 1 gene (XPO1) and their impact on selective inhibitor of nuclear export compounds sensitivity in primary mediastinal B-cell lymphoma. 61
27312795 2016
48
Primary splenic B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: A case report. 61
27602118 2016
49
Distinguishing Classical Hodgkin Lymphoma, Gray Zone Lymphoma, and Large B-cell Lymphoma: A Proposed Scoring System. 61
26447896 2016
50
Mediastinal Gray Zone Lymphoma: A Wider Category Than We Think? 61
26957476 2016

Variations for Gray Zone Lymphoma

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 11.68 TNFRSF8 PDCD1LG2 PAX5 CIITA CD79A CD274
2 11.08 PDCD1LG2 PAX5 CD274 BCL6
3 10.74 CIITA CD79A

GO Terms for Gray Zone Lymphoma

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

# Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 8.92 MME FUT4 CIITA CD274

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of T cell proliferation GO:0042130 9.37 PDCD1LG2 CD274
2 aging GO:0007568 9.33 PAX5 MME CIITA
3 negative regulation of interferon-gamma production GO:0032689 9.32 PDCD1LG2 CD274
4 toxin transport GO:1901998 9.26 SLC22A2 CD274
5 negative regulation of interleukin-10 production GO:0032693 8.96 PDCD1LG2 CD274
6 negative regulation of activated T cell proliferation GO:0046007 8.62 PDCD1LG2 CD274

Sources for Gray Zone Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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