PEL
MCID: PRM042
MIFTS: 52

Primary Effusion Lymphoma (PEL) malady

Rare diseases, Blood diseases, Cancer diseases, Infectious diseases, Immune diseases categories
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Summaries for Primary Effusion Lymphoma

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65Wikipedia, 33MalaCards
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Wikipedia:65 Primary effusion lymphoma (PEL) is a B-cell lymphoma. more...

MalaCards: Primary Effusion Lymphoma, also known as PEL, is related to b-cell lymphomas and multiple myeloma, and has symptoms including early death/lethality, immunodeficiency/increased susceptibility to infections/recurrent infections and lymphoma. An important gene associated with Primary Effusion Lymphoma is IRF4 (interferon regulatory factor 4), and among its related pathways are IL-4 Signaling and its Primary Biological Effects in Different Immune Cell Types and Type II interferon signaling (IFNG). The compounds alefacept and lithium have been mentioned in the context of this disorder. Affiliated tissues include b cells, t cells and liver, and related mouse phenotypes are cellular and hematopoietic system.

Aliases & Classifications for Primary Effusion Lymphoma

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43NIH Rare Diseases, 49Orphanet, 62UMLS, 45Novoseek, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
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Classifications:



Characteristics (Orphanet epidemiological data):

49
primary effusion lymphoma:
Age of onset: Adulthood; Age of death: Adult


Aliases & Descriptions:

primary effusion lymphoma 43 49 62
pel 43 49
body cavity-based lymphoma 49
lymphoma, aids-related 62
aids-related lymphoma 43
lymphoma aids-related 45


External Ids:

MESH via Orphanet36 D054685
ICD10 via Orphanet26 C83.8
SNOMED-CT via Orphanet59 128800006
UMLS via Orphanet63 C1292753

Related Diseases for Primary Effusion Lymphoma

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17GeneCards, 18GeneDecks
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Diseases related to Primary Effusion Lymphoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 50)
idRelated DiseaseScoreTop Affiliating Genes
1b-cell lymphomas30.6SPI1, XBP1, FCER2, IRF4, CD58
2multiple myeloma30.2IRF4, XBP1
3myeloma30.1CD58, IRF4, FCER2, XBP1
4burkitt's lymphoma30.1CD58, CD2, CASP2, IRF4, FCER2, XBP1
5multiple sclerosis30.1CD58, CD2, SELPLG
6sarcoma11.0
7kaposi's sarcoma11.0
8castleman's disease10.6
9hepatitis10.6
10hepatitis c10.5
11hepatitis c virus10.5
12liver cirrhosis10.5
13peritonitis10.4
14hiv/aids10.4
15endotheliitis10.4
16hepatitis b10.3
17diffuse large b-cell lymphoma10.3
18acquired immunodeficiency syndrome10.3
19cardiac tamponade10.1
20richter's syndrome10.1
21autoimmune hemolytic anemia10.1
22polycystic kidney disease10.1
23constrictive pericarditis10.1
24common variable immunodeficiency10.1
25anaplastic large cell lymphoma10.1
26hemolytic anemia10.1
27hepatocellular carcinoma10.1
28hypertension10.1
29kidney disease10.1
30mediastinitis10.1
31pericardial effusion10.1
32pericarditis10.1
33severe combined immunodeficiency10.1
34chylous ascites10.1
35plasmablastic lymphoma10.1
36t-lymphocytopenia10.1
37hiv-110.1
38central nervous system lymphoma10.1
39hodgkin's lymphoma10.1
40lymphoma aidsrelated10.1
41pancytopenia10.0
42follicular lymphoma10.0IRF4, FCER2
43plasmacytoma10.0SPI1, XBP1, IRF4
44myocardial infarction10.0CD2, SELPLG
45eosinophilia10.0FCER2, CD2
46non-hodgkin lymphoma10.0IRF4, FCER2, SPI1
47ipex syndrome10.0FCER2, CD2, CD58
48melanoma9.9IRF4, CASP2, CD2, CD58
49t-cell leukemia9.9IRF4, FCER2, XBP1, SPI1
50leukemia9.8SPI1, FCER2, SELPLG, IRF4, CASP2, CD2

Graphical network of the top 20 diseases related to Primary Effusion Lymphoma:



Diseases related to primary effusion lymphoma

Symptoms for Primary Effusion Lymphoma

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49Orphanet
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Symptoms:

49 (show all 8)
  • early death/lethality
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • lymphoma
  • pericardium anomalies/pericarditis/absence/cysts/pericardial effusion
  • respiratory distress/dyspnea/respiratory failure/lung volume reduction
  • abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax
  • peritonitis/peritoneal abscess
  • acute abdominal pain/colic

Drugs & Therapeutics for Primary Effusion Lymphoma

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Primary Effusion Lymphoma

Search NIH Clinical Center for Primary Effusion Lymphoma

Genetic Tests for Primary Effusion Lymphoma

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Anatomical Context for Primary Effusion Lymphoma

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33MalaCards
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MalaCards organs/tissues related to Primary Effusion Lymphoma:

33
B cells, T cells, Liver, Lung, Endothelial, Heart, Testis, Skin, Kidney, Colon

Animal Models for Primary Effusion Lymphoma or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Primary Effusion Lymphoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053847.3CASP2, CORO1A, SELPLG, FCER2, XBP1, SPI1
2MP:00053976.9CASP2, SPI1, XBP1, FCER2, SELPLG, IRF4
3MP:00053876.7SPI1, XBP1, FCER2, SELPLG, IRF4, CORO1A

Publications for Primary Effusion Lymphoma

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52PubMed
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Articles related to Primary Effusion Lymphoma:

(show top 50)    (show all 306)
idTitleAuthorsYear
1
HIV protease inhibitor Lopinavir induces apoptosis of primary effusion lymphoma cells via suppression of NF-I_B pathway. (24012878)
2014
2
HIV-negative, HHV-8-unrelated primary effusion lymphoma-like lymphoma with genotypic infidelity and c-MYC expression. (24408158)
2014
3
Targeting Sphingosine Kinase Induces Apoptosis and Tumor Regression for KSHV-Associated Primary Effusion Lymphoma. (24140934)
2014
4
Role of IRF4 in IFN-stimulated gene induction and maintenance of Kaposi sarcoma-associated herpesvirus latency in primary effusion lymphoma cells. (23804715)
2013
5
Recruitment of the tumour suppressor protein p73 by Kaposi's Sarcoma Herpesvirus latent nuclear antigen contributes to the survival of primary effusion lymphoma cells. (22964633)
2013
6
Diagnosis and management of primary effusion lymphoma in the immunocompetent and immunocompromised hosts. (23424065)
2013
7
Primary effusion lymphoma. (23899073)
2013
8
HSP70 inhibition by 2-phenylethynesulfonamide induces lysosomal cathepsin D release and immunogenic cell death in primary effusion lymphoma. (23868063)
2013
9
Primary effusion lymphoma cell death induced by bortezomib and AG 490 activates dendritic cells through CD91. (22412839)
2012
10
A case of human herpes virus-8 unrelated primary effusion lymphoma-like lymphoma presented as pleural effusion. (23319997)
2012
11
Extracavitary KSHV-associated large B-Cell lymphoma: a distinct entity or a subtype of primary effusion lymphoma? Study of 9 cases and review of an additional 43 cases. (22790853)
2012
12
Primary effusion lymphoma-like lymphoma with polycystic kidney disease. (22802562)
2012
13
Diethyldithiocarbamate induces apoptosis in HHV-8-infected primary effusion lymphoma cells via inhibition of the NF-I_B pathway. (22200846)
2012
14
Primary effusion lymphoma in an HIV-negative man. (21762120)
2011
15
A case of Kaposi sarcoma-associated herpesvirus/human herpesvirus 8-unrelated but Epstein-Barr virus-positive primary effusion lymphoma-like lymphoma in the setting of human immunodeficiency virus and hepatitis C virus infection. (20929325)
2010
16
Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of the literature. (19245595)
2009
17
A case of HIV-negative primary effusion lymphoma treated with bortezomib, pegylated liposomal doxorubicin, and rituximab. (18854285)
2008
18
Solid variant of primary effusion lymphoma in successfully treated HIV infection: a case report. (18663051)
2008
19
Primary effusion lymphoma cells undergoing human herpesvirus type 8 productive infection produce C-type retroviral particles. (19144286)
2008
20
KSHV-transformed primary effusion lymphoma cells induce a VEGF-dependent angiogenesis and establish functional gap junctions with endothelial cells. (18094712)
2008
21
Lyme borreliosis and multiple sclerosis are associated with primary effusion lymphoma. (17197115)
2007
22
Primary effusion lymphoma. (17522245)
2007
23
Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis. (17554754)
2007
24
In primary effusion lymphoma cells, MYB transcriptional repression is associated with v-FLIP expression during latent KSHV infection while both v-FLIP and v-GPCR become involved during the lytic cycle. (17659053)
2007
25
Conservation of virally encoded microRNAs in Kaposi sarcoma--associated herpesvirus in primary effusion lymphoma cell lines and in patients with Kaposi sarcoma or multicentric Castleman disease. (17262705)
2007
26
PS-341 or a combination of arsenic trioxide and interferon-alpha inhibit growth and induce caspase-dependent apoptosis in KSHV/HHV-8-infected primary effusion lymphoma cells. (17568816)
2007
27
Apoptotic effect of ganciclovir on primary effusion lymphoma cells infected with Kaposi's sarcoma-associated herpesvirus. (16838851)
2006
28
Successful eradication of relapsed primary effusion lymphoma with high-dose chemotherapy and autologous stem cell transplantation in a patient seronegative for human immunodeficiency virus. (16757433)
2006
29
Effusion and solid lymphomas have distinctive gene and protein expression profiles in an animal model of primary effusion lymphoma. (16741895)
2006
30
Successful secondary prophylaxis for primary effusion lymphoma with human herpesvirus 8 therapy. (16847420)
2006
31
Downregulation of the major histocompatibility complex class I molecules by human herpesvirus type 8 and impaired natural killer cell activity in primary effusion lymphoma development. (16044583)
2005
32
Inhibition of HHV-8/KSHV infected primary effusion lymphomas in NOD/SCID mice by azidothymidine and interferon-alpha. (15755507)
2005
33
Curcumin suppresses growth and induces apoptosis in primary effusion lymphoma. (16044161)
2005
34
Velcade displays promising activity in primary effusion lymphoma cells. (15908793)
2005
35
Distinct subsets of primary effusion lymphoma can be identified based on their cellular gene expression profile and viral association. (15613351)
2005
36
Mono/oligoclonal pattern of Kaposi Sarcoma-associated herpesvirus (KSHV/HHV-8) episomes in primary effusion lymphoma cells. (15700304)
2005
37
Human-immunodeficiency-virus-negative, human-herpes-virus-8-negative abdominal cavity primary effusion lymphoma. (16372490)
2005
38
Durable remission by sobuzoxane in an HIV-seronegative patient with human herpesvirus 8-negative primary effusion lymphoma. (15168597)
2004
39
Co-expression of vascular endothelial growth factor and its receptor, neuropilin-1, in a B cell lymphoma cell line (OHK) with primary effusion lymphoma immunophenotype. (14507325)
2003
40
Primary effusion lymphoma: cytopathologic diagnosis using in situ molecular genetic analysis for human herpesvirus 8. (12218212)
2002
41
Human herpesvirus 8-associated primary effusion lymphoma in HIV--patients: a clinicopidemiologic variant resembling classic Kaposi's sarcoma. (11940475)
2002
42
p16INK4a loss and sensitivity in KSHV associated primary effusion lymphoma. (11896614)
2002
43
CD3+CD4-CD8-TCR-alphabeta+ T-cell lymphoma with clinical features of primary effusion lymphoma: an autopsy case. (11794701)
2001
44
HHV8-negative primary effusion lymphoma of the peritoneal cavity presenting with a distinct immunohistochemical phenotype. (11350613)
2001
45
Primary effusion lymphoma (PEL) in HIV-negative patients--a distinct clinical entity. (11378560)
2001
46
Kaposi's sarcoma-associated herpesvirus open reading frame 50/Rta protein activates the entire viral lytic cycle in the HH-B2 primary effusion lymphoma cell line. (10846108)
2000
47
Novel association of haemophagocytic syndrome with Kaposi's sarcoma-associated herpesvirus-related primary effusion lymphoma. (11167749)
2000
48
Differential viral protein expression in Kaposi's sarcoma-associated herpesvirus-infected diseases: Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. (10702388)
2000
49
Human herpesvirus 8 open reading frame 26 and open reading frame 65 sequences from multiple myeloma patients: a shared pattern not found in Kaposi's sarcoma or primary effusion lymphoma. (11106236)
2000
50
Cellular tropism and viral interleukin-6 expression distinguish human herpesvirus 8 involvement in Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. (10196314)
1999

Variations for Primary Effusion Lymphoma

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Expression for genes affiliated with Primary Effusion Lymphoma

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Primary Effusion Lymphoma

Search GEO for disease gene expression data for Primary Effusion Lymphoma.

Pathways for genes affiliated with Primary Effusion Lymphoma

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50PathCards, 54R&D Systems, 38NCBI BioSystems Database, 30KEGG, 53QIAGEN, 60Thomson Reuters, 55Reactome
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Compounds for genes affiliated with Primary Effusion Lymphoma

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45Novoseek, 11DrugBank, 51PharmGKB, 24HMDB, 61Tocris Bioscience
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Compounds related to Primary Effusion Lymphoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1alefacept45 1110.8CD2, CD58
2lithium45 51 24 1112.6CASP2, XBP1
3tunicamycin45 6110.5FCER2, XBP1
4slex459.3CD58, SELPLG
5leucine459.3SPI1, XBP1, CORO1A
6cytochalasin b459.1SELPLG, FCER2
7latex459.0SELPLG, CD2
8lipid458.2CD58, CD2, CASP2, SELPLG, XBP1
9serine458.1CASP2, SELPLG, FCER2, SPI1

GO Terms for genes affiliated with Primary Effusion Lymphoma

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16Gene Ontology
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Cellular components related to Primary Effusion Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1integral component of plasma membraneGO:0058878.1CD58, CD2, SELPLG, FCER2

Biological processes related to Primary Effusion Lymphoma according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1heterotypic cell-cell adhesionGO:0341139.8CD58, CD2
2positive regulation of interleukin-8 secretionGO:20004849.7CD58, CD2
3myeloid dendritic cell differentiationGO:0430119.7IRF4, SPI1
4T cell activationGO:0421109.7CD2, IRF4
5histone H3 acetylationGO:0439669.6SPI1, IRF4
6cell-cell adhesionGO:0163379.6CD58, CD2
7leukocyte migrationGO:0509008.9SELPLG, CD2, CD58
8blood coagulationGO:0075968.5CD58, CD2, SELPLG

Molecular functions related to Primary Effusion Lymphoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1receptor bindingGO:0051028.5CD58, CD2, SELPLG
2protein bindingGO:0055157.0SELPLG, IRF4, CORO1A, CASP2, CD2, CD58

Products for genes affiliated with Primary Effusion Lymphoma

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Primary Effusion Lymphoma

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet