MCID: PRM163
MIFTS: 45

Primary Mediastinal Large B-Cell Lymphoma

Categories: Blood diseases, Cancer diseases, Immune diseases, Rare diseases

Aliases & Classifications for Primary Mediastinal Large B-Cell Lymphoma

MalaCards integrated aliases for Primary Mediastinal Large B-Cell Lymphoma:

Name: Primary Mediastinal Large B-Cell Lymphoma 59
Mediastinal Diffuse Large-Cell Lymphoma with Sclerosis 59
Primary Mediastinal Clear Cell Lymphoma of B-Cell Type 59
Large Cell Lymphoma of the Mediastinum 59
Mediastinal Large B-Cell Lymphoma 73
Med-Dlbcl 59

Characteristics:

Orphanet epidemiological data:

59
primary mediastinal large b-cell lymphoma
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: Adult;

Classifications:



External Ids:

Orphanet 59 ORPHA98838
UMLS via Orphanet 74 C0855152 C1292754
ICD10 via Orphanet 34 C83.3
ICD10 33 C85.2
UMLS 73 C1292754

Summaries for Primary Mediastinal Large B-Cell Lymphoma

MalaCards based summary : Primary Mediastinal Large B-Cell Lymphoma, also known as mediastinal diffuse large-cell lymphoma with sclerosis, is related to primary mediastinal b-cell lymphoma and lymphoma. An important gene associated with Primary Mediastinal Large B-Cell Lymphoma is BCL6 (BCL6, Transcription Repressor), and among its related pathways/superpathways are Cytokine Signaling in Immune system and Immune response IL-23 signaling pathway. Affiliated tissues include b cells, bone and heart, and related phenotypes are Increased cell death HMECs cells and cellular

Related Diseases for Primary Mediastinal Large B-Cell Lymphoma

Diseases in the Primary Mediastinal B-Cell Lymphoma family:

Primary Mediastinal Large B-Cell Lymphoma

Diseases related to Primary Mediastinal Large B-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 50)
# Related Disease Score Top Affiliating Genes
1 primary mediastinal b-cell lymphoma 32.2 BCL6 XPO1
2 lymphoma 31.3 BCL2 BCL6 MYC
3 b-cell lymphomas 30.8 BCL2 BCL6 FOXP1 IRF4 MYC
4 diffuse large b-cell lymphoma 30.1 BCL6 FOXP1 IRF4 MYC
5 lymphoma, hodgkin, classic 10.4
6 gray zone lymphoma 10.3
7 mediastinal gray zone lymphoma 10.2
8 colon lymphoma 10.1 BCL6 IRF4
9 central nervous system hematologic cancer 10.1 BCL6 IRF4
10 hemopericardium 10.1
11 pericardial effusion 10.1
12 liposarcoma 10.1
13 thyroiditis 10.1
14 bone marrow necrosis 10.1
15 nodular lymphocyte predominant hodgkin lymphoma 10.1
16 testicular lymphoma 10.0 BCL6 FOXP1
17 plasmablastic lymphoma 10.0 BCL6 MYC
18 epstein-barr virus-associated gastric carcinoma 10.0 CD274 JAK2
19 relapsed/refractory diffuse large b-cell lymphoma 10.0 FOXP1 IRF4
20 lymphatic system cancer 10.0 BCL6 MYC
21 richter's syndrome 10.0 IRF4 MYC
22 nodal marginal zone b-cell lymphoma 10.0 BCL2 BCL6
23 follicular lymphoma 1 10.0 BCL2 BCL6
24 intravascular large b-cell lymphoma 10.0 BCL2 BCL6
25 myasthenic syndrome, congenital, 14 10.0 CD274 JAK2 MYC
26 mature b-cell neoplasm 10.0 BCL6 MYC
27 human herpesvirus 8 10.0 BCL2 BCL6
28 autoimmune lymphoproliferative syndrome, type v 9.9 BCL2 BCL6
29 central nervous system lymphoma 9.9 BCL6 CD274 FOXP1
30 primary effusion lymphoma 9.9 BCL6 IRF4 MYC
31 diffuse large b-cell lymphoma of the central nervous system 9.9 BCL2 MYC
32 leukocyte disease 9.9 BCL6 IRF4 MYC
33 chronic venous leg ulcers 9.9 BCL2 MYC
34 gastric lymphoma 9.9 BCL2 BCL6
35 suppressor of tumorigenicity 3 9.9 BCL2 MYC
36 hematologic cancer 9.9 BCL6 JAK2 MYC
37 myeloma, multiple 9.9 IRF4 JAK2 MYC
38 primary central nervous system lymphoma 9.9 BCL2 BCL6 CD274
39 mediastinal malignant lymphoma 9.8 BCL6 CD274 JAK2 XPO1
40 plasmacytoma 9.8 IRF4 MYC
41 high grade b-cell lymphoma with myc and/ or bcl2 and/or bcl6 rearrangement 9.8 BCL2 BCL6 MYC
42 alk-negative anaplastic large cell lymphoma 9.8 BCL2 CD274 MYC
43 mantle cell lymphoma 9.8 BCL2 BCL6 MYC
44 burkitt lymphoma 9.8 BCL2 BCL6 MYC
45 lymphocytic leukemia 9.7 BCL2 MYC XPO1
46 follicular lymphoma 9.6 BCL2 BCL6 IRF4 MYC
47 gastric adenocarcinoma 9.6 BCL2 CD274 MYC
48 marginal zone b-cell lymphoma 9.6 BCL2 BCL6 FOXP1 IRF4
49 lymphoma, mucosa-associated lymphoid type 9.6 BCL2 BCL6 FOXP1 IRF4
50 lymphoma, non-hodgkin, familial 9.5 BCL2 BCL6 IRF4 JAK2 MYC

Graphical network of the top 20 diseases related to Primary Mediastinal Large B-Cell Lymphoma:



Diseases related to Primary Mediastinal Large B-Cell Lymphoma

Symptoms & Phenotypes for Primary Mediastinal Large B-Cell Lymphoma

GenomeRNAi Phenotypes related to Primary Mediastinal Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased cell death HMECs cells GR00103-A-0 8.92 BCL6 JAK2 MYC XPO1

MGI Mouse Phenotypes related to Primary Mediastinal Large B-Cell Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 9.73 BCL2 BCL6 CD274 IRF4 JAK2 MYC
2 hematopoietic system MP:0005397 9.63 BCL2 BCL6 CD274 IRF4 JAK2 MYC
3 immune system MP:0005387 9.43 BCL2 BCL6 CD274 IRF4 JAK2 MYC
4 neoplasm MP:0002006 8.92 BCL2 IRF4 JAK2 MYC

Drugs & Therapeutics for Primary Mediastinal Large B-Cell Lymphoma

Search Clinical Trials , NIH Clinical Center for Primary Mediastinal Large B-Cell Lymphoma

Genetic Tests for Primary Mediastinal Large B-Cell Lymphoma

Anatomical Context for Primary Mediastinal Large B-Cell Lymphoma

MalaCards organs/tissues related to Primary Mediastinal Large B-Cell Lymphoma:

41
B Cells, Bone, Heart, Thyroid, Bone Marrow, Colon

Publications for Primary Mediastinal Large B-Cell Lymphoma

Articles related to Primary Mediastinal Large B-Cell Lymphoma:

(show top 50) (show all 134)
# Title Authors Year
1
Advances in the management of primary mediastinal large B-cell lymphoma. ( 29741502 )
2018
2
Somatic IL4R mutations in primary mediastinal large B-cell lymphoma lead to constitutive JAK-STAT signaling activation. ( 29467182 )
2018
3
Primary mediastinal large B-cell lymphoma: Outcome of a series of pediatric patients treated with high-dose methotrexate and cytarabine plus anti-CD20. ( 29049862 )
2018
4
Expression pattern of PD-L1 and PD-L2 in classical Hodgkin lymphoma, primary mediastinal large B-cell lymphoma, and gray zone lymphoma. ( 29377256 )
2018
5
Molecular classification of primary mediastinal large B-cell lymphoma using routinely available tissue specimens. ( 30257882 )
2018
6
Brentuximab vedotin in relapsed primary mediastinal large B-cell lymphoma: results from a phase 2 clinical trial. ( 28264798 )
2017
7
Performance of a Commercially Available MAL Antibody in the Diagnosis of Primary Mediastinal Large B-Cell Lymphoma. ( 27879516 )
2017
8
Repeated loss of target surface antigen after immunotherapy in primary mediastinal large B cell lymphoma. ( 27779774 )
2017
9
Primary mediastinal large B-cell lymphoma. ( 28318892 )
2017
10
PDCD1LG2 (PD-L2) RNA inA situ hybridization is a sensitive, specific, and practical marker of primary mediastinal large B-cell lymphoma. ( 28369778 )
2017
11
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. ( 28851661 )
2017
12
Evaluation of primary mediastinal large B cell lymphoma by flow cytometry. ( 28732145 )
2017
13
Primary mediastinal large B cell lymphoma in a woman who is human immunodeficiency virus positive presenting with superior vena cava syndrome: a case report. ( 28187791 )
2017
14
The treatment of primary mediastinal large B-cell lymphoma: a two decades monocentric experience with 98 patients. ( 28415982 )
2017
15
Poor predictive value of positive interim FDG-PET/CT in primary mediastinal large B-cell lymphoma. ( 28634685 )
2017
16
Safety and tolerability of pembrolizumab in patients with relapsed/refractory primary mediastinal large B-cell lymphoma. ( 28490569 )
2017
17
Primary mediastinal large B-cell lymphoma in Japanese children and adolescents. ( 27858330 )
2016
18
Primary mediastinal large B-cell lymphoma and its mimickers: a rare case report with literature review. ( 27568673 )
2016
19
Primary Mediastinal Large B-Cell Lymphoma With Translocations Involving BCL6 and MYC (Double-Hit Lymphoma). ( 27124935 )
2016
20
Bowel perforation from occult ileal involvement after diagnosis in a case of primary mediastinal large B-cell lymphoma. ( 27417993 )
2016
21
PET/CT in primary mediastinal large B-cell lymphoma responding to rituximab-CHOP. ( 27183879 )
2016
22
PET/CT in primary mediastinal large B-cell lymphoma responding to rituximab-CHOP: An analysis of 106 patients regarding prognostic significance and implications for subsequent radiotherapy. ( 25971363 )
2016
23
Primary Mediastinal large B-Cell Lymphoma as an incidental finding: report of a case. ( 27094863 )
2016
24
Primary mediastinal large B-cell lymphoma: transcriptional regulation by miR-92a through FOXP1 targeting. ( 27806315 )
2016
25
A retrospective analysis of outcomes for primary mediastinal large B-cell lymphoma treated with RCHOP followed by radiotherapy or front-line autologous stem cell transplantation. ( 27884092 )
2016
26
Functional Imaging Using 18-Fluorodeoxyglucose PET in the Management of Primary Mediastinal Large B-Cell Lymphoma: The Contributions of the International Extranodal Lymphoma Study Group. ( 27249743 )
2016
27
Primary Mediastinal Large B-cell Lymphoma Exhibiting Endobronchial Involvement. ( 27803409 )
2016
28
Primary mediastinal large B-cell lymphoma in pregnancy. ( 25956044 )
2016
29
A single-institution retrospective analysis of outcomes for stage I-II primary mediastinal large B-cell lymphoma treated with immunochemotherapy with or without radiotherapy. ( 26159046 )
2016
30
The immunophenotypic spectrum of primary mediastinal large B-cell lymphoma reveals prognostic biomarkers associated with outcome. ( 27419920 )
2016
31
Favorable outcome of primary mediastinal large B-cell lymphoma patients treated with sequential RCHOP-RICE regimen without radiotherapy. ( 27056383 )
2016
32
Racial patterns of patients with primary mediastinal large B-cell lymphoma: SEER analysis. ( 27399089 )
2016
33
Emerging Biological Insights and Novel Treatment Strategies in Primary Mediastinal Large B-Cell Lymphoma. ( 25805591 )
2015
34
Genomic Alterations in CIITA Are Frequent in Primary Mediastinal Large B Cell Lymphoma and Are Associated with Diminished MHC Class II Expression. ( 26549456 )
2015
35
Identification of Primary Mediastinal Large B-cell Lymphoma at Nonmediastinal Sites by Gene Expression Profiling. ( 26135560 )
2015
36
(P109) Standard Immunochemotherapy Plus Radiation vs Dose-Intense Chemotherapy With Rituximab in Stage I/II Primary Mediastinal Large B-Cell Lymphoma: A Single-Institution Experience. ( 25930861 )
2015
37
Rituximab induction therapy, survival benefits, and the increasing selection of radiotherapy as the postinduction treatment in patients with primary mediastinal large B-cell lymphoma. ( 25801492 )
2015
38
Primary mediastinal large B-cell lymphoma arising from thyroid in a renal recipient with Hashimoto's thyroiditis. ( 26191324 )
2015
39
Role of radiation therapy in primary mediastinal large B-cell lymphoma in rituximab era: A US population-based analysis. ( 26270899 )
2015
40
High-dose chemotherapy followed by autologous stem cell transplantation for relapsed/refractory primary mediastinal large B-cell lymphoma. ( 26636287 )
2015
41
Current trends in the treatment of primary mediastinal large B-cell lymphoma - an overview. ( 26843837 )
2015
42
Role of radiotherapy in the treatment of primary mediastinal large B-cell lymphoma. ( 26722265 )
2015
43
Primary mediastinal large B-cell lymphoma. ( 25913715 )
2015
44
Activating mutations affecting the NF-kappa B pathway and EZH2-mediated epigenetic regulation are rare events in primary mediastinal large B-cell lymphoma. ( 25275047 )
2014
45
Primary mediastinal large B-cell lymphoma: a single-center experience in Korea. ( 24724065 )
2014
46
[18F]fluorodeoxyglucose positron emission tomography predicts survival after chemoimmunotherapy for primary mediastinal large B-cell lymphoma: results of the International Extranodal Lymphoma Study Group IELSG-26 Study. ( 24799481 )
2014
47
The impact of R-VACOP-B and interim FDG-PET/CT on outcome in primary mediastinal large B cell lymphoma. ( 24595734 )
2014
48
Primary mediastinal large B-cell lymphoma: Clinical features, prognostic factors and survival with RCHOP in Arab patients in the PET scan era. ( 25125808 )
2014
49
Primary mediastinal large B-cell lymphoma. ( 24952250 )
2014
50
Prognostic significance of pleural or pericardial effusion, and the implication of optimal treatment in primary mediastinal large B-cell lymphoma: a multicenter retrospective study in Japan. ( 25216682 )
2014

Variations for Primary Mediastinal Large B-Cell Lymphoma

Cosmic variations for Primary Mediastinal Large B-Cell Lymphoma:

9
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM34215 JAK3 haematopoietic and lymphoid tissue,mediastinum,lymphoid neoplasm,B cell lymphoma unspecified c.1718C>T p.A573V 19:17837197-17837197 1

Expression for Primary Mediastinal Large B-Cell Lymphoma

Search GEO for disease gene expression data for Primary Mediastinal Large B-Cell Lymphoma.

Pathways for Primary Mediastinal Large B-Cell Lymphoma

Pathways related to Primary Mediastinal Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 29)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.96 BCL2 BCL6 IRF4 JAK2 MYC
2
Show member pathways
12.34 BCL2 JAK2 MYC
3 12.26 BCL2 FOXP1 MYC
4
Show member pathways
12.2 BCL2 JAK2 MYC
5
Show member pathways
12.16 BCL2 BCL6 JAK2 MYC
6
Show member pathways
12.12 BCL2 JAK2 MYC
7
Show member pathways
12.1 BCL2 IRF4 JAK2 MYC
8
Show member pathways
12.09 BCL6 IRF4 MYC
9 11.97 BCL6 CD274 IRF4 JAK2
10
Show member pathways
11.83 BCL2 JAK2 MYC
11
Show member pathways
11.74 BCL2 JAK2 MYC
12 11.64 BCL2 BCL6 MYC
13
Show member pathways
11.53 BCL2 JAK2 MYC
14 11.43 BCL2 MYC
15 11.34 BCL2 XPO1
16 11.3 BCL6 XPO1
17 11.29 JAK2 MYC
18 11.27 BCL2 MYC
19
Show member pathways
11.24 BCL2 MYC
20 11.23 BCL2 MYC
21 11.23 BCL2 JAK2
22 11.13 IRF4 JAK2
23 11.08 BCL6 XPO1
24 11.08 BCL2 BCL6 IRF4 JAK2 MYC
25
Show member pathways
10.99 IRF4 JAK2
26 10.96 BCL2 MYC
27 10.93 BCL6 IRF4 JAK2
29
Show member pathways
10.82 BCL2 MYC

GO Terms for Primary Mediastinal Large B-Cell Lymphoma

Cellular components related to Primary Mediastinal Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleoplasm GO:0005654 9.17 BCL2 BCL6 FOXP1 IRF4 JAK2 MYC

Biological processes related to Primary Mediastinal Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.69 BCL2 MYC XPO1
2 response to cytokine GO:0034097 9.54 BCL2 CD274
3 positive regulation of smooth muscle cell proliferation GO:0048661 9.52 FOXP1 MYC
4 branching involved in ureteric bud morphogenesis GO:0001658 9.49 BCL2 MYC
5 ovarian follicle development GO:0001541 9.48 BCL2 MYC
6 positive regulation of B cell proliferation GO:0030890 9.46 BCL2 BCL6
7 response to gamma radiation GO:0010332 9.43 BCL2 MYC
8 positive regulation of smooth muscle cell migration GO:0014911 9.4 BCL2 MYC
9 axon regeneration GO:0031103 9.37 BCL2 JAK2
10 regulation of inflammatory response GO:0050727 9.33 BCL6 FOXP1 JAK2
11 intrinsic apoptotic signaling pathway in response to oxidative stress GO:0008631 9.32 BCL2 JAK2
12 negative regulation of B cell apoptotic process GO:0002903 9.26 BCL6 FOXP1
13 positive regulation of DNA binding GO:0043388 9.13 IRF4 JAK2 MYC
14 cytokine-mediated signaling pathway GO:0019221 9.02 BCL2 BCL6 IRF4 JAK2 MYC

Molecular functions related to Primary Mediastinal Large B-Cell Lymphoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription factor binding GO:0008134 9.33 BCL2 IRF4 MYC
2 DNA-binding transcription factor activity GO:0003700 9.26 BCL6 FOXP1 IRF4 MYC
3 sequence-specific DNA binding GO:0043565 9.02 BCL2 BCL6 FOXP1 IRF4 MYC

Sources for Primary Mediastinal Large B-Cell 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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