PEL
MCID: PRM042
MIFTS: 53

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

MalaCards based summary: Primary Effusion Lymphoma, also known as body cavity-based lymphoma, is related to multiple myeloma and b-cell lymphomas, and has symptoms including acute abdominal pain/colic, peritonitis/peritoneal abscess and abnormal pleura/hydrothorax/pleuresia/pleural effusion/chylothorax. 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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Sources:
42NIH Rare Diseases, 48Orphanet, 62UMLS, 44Novoseek, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Primary Effusion Lymphoma, Aliases & Descriptions:

Name: Primary Effusion Lymphoma 42 48 62
Body Cavity-Based Lymphoma 48 62
Pel 42 48
 
Lymphoma, Aids-Related 62
Lymphoma Aids-Related 44
Aids-Related Lymphoma 42


Classifications:



Characteristics (Orphanet epidemiological data):

48
primary effusion lymphoma:
Age of onset: Adulthood; Age of death: Adult


External Ids:

MESH via Orphanet35 D054685
ICD10 via Orphanet26 C83.8
UMLS via Orphanet63 C1292753

Related Diseases for Primary Effusion Lymphoma

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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
1multiple myeloma30.3XBP1, IRF4
2b-cell lymphomas30.1CD58, IRF4, FCER2, SPI1, XBP1
3myeloma29.9FCER2, CD58, IRF4, XBP1
4burkitt's lymphoma29.0SPI1, CD58, CD2, CASP2, IRF4, FCER2
5sarcoma11.0
6kaposi's sarcoma11.0
7hepatitis10.7
8castleman's disease10.7
9hepatitis c10.6
10hepatitis c virus10.6
11liver cirrhosis10.5
12peritonitis10.5
13endotheliitis10.4
14hepatitis b10.4
15hiv/aids10.4
16diffuse large b-cell lymphoma10.3
17acquired immunodeficiency syndrome10.3
18anaplastic large cell lymphoma10.1
19common variable immunodeficiency10.1
20hemolytic anemia10.1
21hiv-110.1
22hypertension10.1
23pulmonary hypertension10.1
24severe combined immunodeficiency10.1
25cardiac tamponade10.1
26richter's syndrome10.1
27autoimmune hemolytic anemia10.1
28polycystic kidney disease10.1
29constrictive pericarditis10.1
30kidney disease10.1
31mediastinitis10.1
32pericardial effusion10.1
33pericarditis10.1
34chylous ascites10.1
35plasmablastic lymphoma10.1
36t-lymphocytopenia10.1
37hodgkin's lymphoma10.1
38central nervous system lymphoma10.1
39lymphoma aidsrelated10.1
40pancytopenia10.1
41follicular lymphoma10.1IRF4, FCER2
42plasmacytoma10.0SPI1, XBP1, IRF4
43myocardial infarction10.0SELPLG, CD2
44eosinophilia10.0CD2, FCER2
45non-hodgkin lymphoma9.9IRF4, FCER2, SPI1
46ipex syndrome9.8CD58, FCER2, CD2
47melanoma9.8CD58, CD2, IRF4, CASP2
48t-cell leukemia9.8FCER2, SPI1, XBP1, IRF4
49multiple sclerosis9.7SELPLG, CD58, CD2
50leukemia9.0CD58, CD2, CASP2, IRF4, SELPLG, SPI1

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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Symptoms:

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

HPO human phenotypes related to Primary Effusion Lymphoma:

(show all 7)
id Description Frequency HPO Source Accession
1 abnormality of the pericardium hallmark (90%) HP:0001697
2 abdominal pain hallmark (90%) HP:0002027
3 respiratory insufficiency hallmark (90%) HP:0002093
4 abnormality of the pleura hallmark (90%) HP:0002103
5 peritonitis hallmark (90%) HP:0002586
6 lymphoma hallmark (90%) HP:0002665
7 abnormality of immune system physiology typical (50%) HP:0010978

Drugs & Therapeutics for Primary Effusion Lymphoma

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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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MalaCards organs/tissues related to Primary Effusion Lymphoma:

32
B cells, T cells, Liver, Lung, Endothelial, Heart, Colon, Kidney, Skin, Testis

Animal Models for Primary Effusion Lymphoma or affiliated genes

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MGI Mouse Phenotypes related to Primary Effusion Lymphoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053847.3CORO1A, SELPLG, FCER2, XBP1, SPI1, CASP2
2MP:00053976.9SPI1, CASP2, CORO1A, IRF4, SELPLG, FCER2
3MP:00053876.7SPI1, XBP1, FCER2, SELPLG, IRF4, CORO1A

Publications for Primary Effusion Lymphoma

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Articles related to Primary Effusion Lymphoma:

(show top 50)    (show all 325)
idTitleAuthorsYear
1
HIV protease inhibitor Lopinavir induces apoptosis of primary effusion lymphoma cells via suppression of NF-I_B pathway. (24012878)
2014
2
Current status of treatment for primary effusion lymphoma. (25364646)
2014
3
HIV-negative, HHV-8-unrelated primary effusion lymphoma-like lymphoma with genotypic infidelity and c-MYC expression. (24408158)
2014
4
Extracavitary primary effusion lymphoma: clinical, morphological, phenotypic and cytogenetic characterization using nuclei enrichment technique. (25139766)
2014
5
Primary effusion lymphoma with aberrant T-cell phenotype in an iatrogenically immunosuppressed renal transplant male: Cytologic diagnosis in peritoneal fluid. (24644156)
2014
6
A case of primary effusion lymphoma diagnosed by open cardiocentesis. (24585314)
2014
7
Role of IRF4 in IFN-stimulated gene induction and maintenance of Kaposi sarcoma-associated herpesvirus latency in primary effusion lymphoma cells. (23804715)
2013
8
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
9
Diagnosis and management of primary effusion lymphoma in the immunocompetent and immunocompromised hosts. (23424065)
2013
10
Primary effusion lymphoma. (23899073)
2013
11
HSP70 inhibition by 2-phenylethynesulfonamide induces lysosomal cathepsin D release and immunogenic cell death in primary effusion lymphoma. (23868063)
2013
12
Primary effusion lymphoma cell death induced by bortezomib and AG 490 activates dendritic cells through CD91. (22412839)
2012
13
A case of human herpes virus-8 unrelated primary effusion lymphoma-like lymphoma presented as pleural effusion. (23319997)
2012
14
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
15
Primary effusion lymphoma-like lymphoma with polycystic kidney disease. (22802562)
2012
16
Diethyldithiocarbamate induces apoptosis in HHV-8-infected primary effusion lymphoma cells via inhibition of the NF-I_B pathway. (22200846)
2012
17
Primary effusion lymphoma in an HIV-negative man. (21762120)
2011
18
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
19
Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of the literature. (19245595)
2009
20
A case of HIV-negative primary effusion lymphoma treated with bortezomib, pegylated liposomal doxorubicin, and rituximab. (18854285)
2008
21
Solid variant of primary effusion lymphoma in successfully treated HIV infection: a case report. (18663051)
2008
22
Primary effusion lymphoma cells undergoing human herpesvirus type 8 productive infection produce C-type retroviral particles. (19144286)
2008
23
Primary effusion lymphoma. (17522245)
2007
24
Primary effusion lymphoma: a series of 4 cases and review of the literature with emphasis on cytomorphologic and immunocytochemical differential diagnosis. (17554754)
2007
25
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
26
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
27
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
28
Apoptotic effect of ganciclovir on primary effusion lymphoma cells infected with Kaposi's sarcoma-associated herpesvirus. (16838851)
2006
29
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
30
Effusion and solid lymphomas have distinctive gene and protein expression profiles in an animal model of primary effusion lymphoma. (16741895)
2006
31
Successful secondary prophylaxis for primary effusion lymphoma with human herpesvirus 8 therapy. (16847420)
2006
32
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
33
Inhibition of HHV-8/KSHV infected primary effusion lymphomas in NOD/SCID mice by azidothymidine and interferon-alpha. (15755507)
2005
34
Curcumin suppresses growth and induces apoptosis in primary effusion lymphoma. (16044161)
2005
35
Velcade displays promising activity in primary effusion lymphoma cells. (15908793)
2005
36
Distinct subsets of primary effusion lymphoma can be identified based on their cellular gene expression profile and viral association. (15613351)
2005
37
Mono/oligoclonal pattern of Kaposi Sarcoma-associated herpesvirus (KSHV/HHV-8) episomes in primary effusion lymphoma cells. (15700304)
2005
38
Human-immunodeficiency-virus-negative, human-herpes-virus-8-negative abdominal cavity primary effusion lymphoma. (16372490)
2005
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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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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Compounds for genes affiliated with Primary Effusion Lymphoma

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Compounds related to Primary Effusion Lymphoma according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1alefacept44 1110.8CD2, CD58
2lithium44 50 24 1112.6CASP2, XBP1
3tunicamycin44 6110.5FCER2, XBP1
4slex449.3CD58, SELPLG
5leucine449.3SPI1, XBP1, CORO1A
6cytochalasin b449.1SELPLG, FCER2
7latex449.0SELPLG, CD2
8lipid448.2CD58, CD2, CASP2, SELPLG, XBP1
9serine448.1CASP2, SELPLG, FCER2, SPI1

GO Terms for genes affiliated with Primary Effusion Lymphoma

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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

Sources for Primary Effusion Lymphoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet