PEL
MCID: PRM042
MIFTS: 51

Primary Effusion Lymphoma (PEL) malady

Blood, Cancer, Infectious, Immune categories

Summaries for Primary Effusion Lymphoma

Sources:
64Wikipedia, 33MalaCards
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Wikipedia:64 Primary effusion lymphoma (PEL) is a B-cell lymphoma. more...

MalaCards: Primary Effusion Lymphoma, also known as PEL, is related to hepatitis c and hepatitis b, and has symptoms including respiratory distress/dyspnea/respiratory failure/lung volume reduction, early death/lethality and immunodeficiency/increased susceptibility to infections/recurrent infections. An important gene associated with Primary Effusion Lymphoma is CD2 (CD2 molecule), and among its related pathways are Cell surface interactions at the vascular wall and TRAF Pathway. The compounds doxorubicin and serine have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and kidney, and related mouse phenotypes are reproductive system and hematopoietic system.

Aliases & Classifications for Primary Effusion Lymphoma

Sources:
43NIH Rare Diseases, 45Novoseek, 49Orphanet, 61UMLS, 36MESH via Orphanet, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer, Infectious
Anatomical: Blood, Immune


Characteristics (Orphanet epidemiological data):

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


Aliases & Descriptions:

primary effusion lymphoma 43 49 61
pel 43 49
body cavity-based lymphoma 49
lymphoma, aids-related 61
aids-related lymphoma 43
lymphoma aids-related 45


External Ids:

MESH via Orphanet36 D054685
ICD10 via Orphanet26 C83.8
SNOMED-CT via Orphanet58 128800006
UMLS via Orphanet62 C1292753

Related Diseases for Primary Effusion Lymphoma

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 77)
idRelated DiseaseScoreTop Affiliating Genes
1hepatitis c30.8CD58, MYB, IRF3
2hepatitis b30.1CD58, MYB
3central nervous system lymphoma30.1BCL6
4lymphoma aidsrelated30.1TCL1A, BCL6
5kaposi's sarcoma11.0
6castleman's disease10.7
7multicentric castleman disease10.7
8multicentric castleman’s disease10.7
9hepatitis e10.6
10hepatitis c virus10.5
11liver lymphoma10.5
12plasmablastic lymphoma10.4
13heart lymphoma10.3
14primary malignant lymphoma10.3
15lymphoma, diffuse large cell10.3
16acquired immunodeficiency syndrome10.2
17primary pulmonary hypertension10.1
18cardiac tamponade10.1
19colon lymphoma10.1
20richter's syndrome10.1
21epstein-barr virus hepatitis10.1
22common variable immunodeficiency10.1
23autoimmune hemolytic anemia10.1
24polycystic kidney disease10.1
25anaplastic large cell lymphoma10.1
26hepatitis a10.1
27constrictive pericarditis10.1
28central nervous system lymphoma, primary10.1
29chylous ascites10.1
30classic kaposi sarcoma10.1
31heart tumor10.1
32t cell immunodeficiency primary10.1
33hepatitis b virus infection10.1
34primary pulmonary lymphoma10.1
35pancytopenia10.0
36pelger-huet anomaly10.0
37sarcoma10.0TNFRSF10B
38multiple myeloma10.0SDC1, XBP1
39myeloid leukemia10.0MYB, TNFRSF10B
40acute leukemia10.0MYB, CD58, CD7
41multiple sclerosis10.0SELPLG, CD2, CD58
42cervical cancer10.0SKP2, CDKN1B, TNFRSF10B
43adenoma10.0TNFRSF10B, VEGFB, CDKN1B, SKP2
44arthritis10.0CD58, TNFRSF10B
45b-cell chronic lymphocytic leukemia10.0TNFRSF10B, CDKN1B, TCL1A, BCL6
46hodgkin's lymphoma10.0POU2AF1, POU2F2, TCL1A, BCL6
47lymphedema10.0LYVE1
48burkitt's lymphoma10.0TNFRSF10B, MYB, TCL1A, BCL6
49adenocarcinoma10.0SKP2, CDKN1B, MYB, TNFRSF10B
50follicular lymphoma10.0MYB, CDKN1B, SKP2, TCL1A, BCL6

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



Diseases related to primary effusion lymphoma

Clinical Features for Primary Effusion Lymphoma

Sources:
49Orphanet
See all sources


Symptoms:

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

Drugs & Therapeutics for Primary Effusion Lymphoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Primary Effusion Lymphoma

Drug clinical trials:

Search ClinicalTrials for Primary Effusion Lymphoma

Search NIH Clinical Center for Primary Effusion Lymphoma

Search CenterWatch for Primary Effusion Lymphoma

Genetic Tests for Primary Effusion Lymphoma

Anatomical Context for Primary Effusion Lymphoma

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

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

Animal Models for Primary Effusion Lymphoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

Publications for Primary Effusion Lymphoma

Sources:
51PubMed
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Articles related to Primary Effusion Lymphoma:

(show top 50)    (show all 317)
idTitleAuthorsYear
1
HIV-negative, HHV-8-unrelated primary effusion lymphoma-like lymphoma with genotypic infidelity and c-MYC expression. (24408158)
2014
2
Targeting Sphingosine Kinase Induces Apoptosis and Tumor Regression for KSHV-Associated Primary Effusion Lymphoma. (24140934)
2014
3
Primary effusion anaplastic large cell lymphoma in a patient with long-standing dermatitis herpetiformis and celiac disease. (22915338)
2013
4
Diagnosis and management of primary effusion lymphoma in the immunocompetent and immunocompromised hosts. (23424065)
2013
5
Hippuristanol reduces the viability of primary effusion lymphoma cells both in vitro and in vivo. (24018901)
2013
6
Human Immunodeficiency Virus (HIV)-Negative and Human Herpes Virus-8 (HHV-8)-Positive Primary Effusion Lymphoma: A Case Report and Review of the Literature. (24385757)
2013
7
Castleman's disease and primary effusion lymphoma in a HIV-positive patient. (24287026)
2013
8
Extracavitary KSHV-positive solid lymphoma: a large B-cell lymphoma within the spectrum of primary effusion lymphoma. (24076781)
2013
9
HHV-8-positive but EBV-negative primary effusion lymphoma. (24427809)
2013
10
Primary effusion lymphoma (PEL) without effusion: a patient case report of a PEL solid variant. (22011447)
2012
11
Constrictive pericarditis and primary effusion lymphoma following allogeneic BMT for CML. (22246087)
2012
12
T-cell immunity to Kaposi sarcoma-associated herpesvirus: recognition of primary effusion lymphoma by LANA-specific CD4+ T cells. (22234686)
2012
13
X-box binding protein 1 induces the expression of the lytic cycle transactivator of Kaposi's sarcoma-associated herpesvirus but not Epstein-Barr virus in co-infected primary effusion lymphoma. (20980528)
2011
14
Primary effusion lymphoma associated with Human Herpes Virus-8 and Epstein Barr virus in an HIV-infected woman from Kampala, Uganda: a case report. (21320326)
2011
15
Primary effusion lymphoma of T-cell origin with t(7;8)(q32;q13) in an HIV-negative patient with HCV-related liver cirrhosis and hepatocellular carcinoma positive for HHV6 and HHV8. (21302114)
2011
16
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
17
Primary effusion lymphoma in two HIV-negative patients successfully treated with pleurodesis as first-line therapy. (20150647)
2010
18
Human herpes virus 8-unrelated primary effusion lymphoma-like lymphoma: report of a rare case and review of the literature. (19245595)
2009
19
Elevation of clonal serum free light chains in patients with HIV-negative primary effusion lymphoma (PEL) and PEL-like lymphoma. (19681885)
2009
20
Antineoplastic activity of lentiviral vectors expressing interferon-alpha in a preclinical model of primary effusion lymphoma. (19196659)
2009
21
Human herpes virus 8-negative primary effusion lymphoma with BCL6 rearrangement in a patient with idiopathic CD4 positive T-lymphocytopenia. (18166773)
2008
22
Rare B-cell lymphomas: primary mediastinal, intravascular, and primary effusion lymphoma. (18283789)
2008
23
EBV is necessary for proliferation of dually infected primary effusion lymphoma cells. (18757410)
2008
24
Apoptotic effect of ganciclovir on primary effusion lymphoma cells infected with Kaposi's sarcoma-associated herpesvirus. (16838851)
2006
25
Sodium butyrate induces apoptosis and cell cycle arrest in primary effusion lymphoma cells independently of oxidative stress and p21(CIP1/WAF1) induction. (16477379)
2006
26
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
27
Velcade displays promising activity in primary effusion lymphoma cells. (15908793)
2005
28
Treatment of herpesvirus associated primary effusion lymphoma with intracavity cidofovir. (15674353)
2005
29
Patterned CpG methylation of silenced B cell gene promoters in classical Hodgkin lymphoma-derived and primary effusion lymphoma cell lines. (15967459)
2005
30
Inhibiting primary effusion lymphoma by lentiviral vectors encoding short hairpin RNA. (15572586)
2005
31
Kaposi's sarcoma-associated herpesvirus/human herpesvirus type 8-positive solid lymphomas: a tissue-based variant of primary effusion lymphoma. (15681470)
2005
32
The proteasome inhibitor bortezomib (PS-341) inhibits growth and induces apoptosis in primary effusion lymphoma cells. (15662128)
2005
33
Durable remission by sobuzoxane in an HIV-seronegative patient with human herpesvirus 8-negative primary effusion lymphoma. (15168597)
2004
34
The tumor microenvironment controls primary effusion lymphoma growth in vivo. (15256448)
2004
35
Failure to eradicate AIDS-associated primary effusion lymphoma with high-dose chemotherapy and autologous stem cell reinfusion: case report and literature review. (15006181)
2004
36
Defective immune function of primary effusion lymphoma cells is associated with distinct KSHV gene expression profiles. (15360006)
2004
37
Rituximab is effective for human herpesvirus-8-negative primary effusion lymphoma with CD20 phenotype associated hepatitis C virus-related liver cirrhosis. (15551361)
2004
38
HIV-1 infection of primary effusion lymphoma cell line triggers Kaposi's sarcoma-associated herpesvirus (KSHV) reactivation. (11857356)
2002
39
Primary effusion lymphoma with subsequent development of a small bowel mass in an HIV-seropositive patient: a case report and literature review. (12360052)
2002
40
Establishment of a primary effusion lymphoma cell line (RM-P1) and in vivo growth system using SCID mice. (12215016)
2002
41
Primary effusion lymphoma in an HIV-negative patient with no serologic evidence of Kaposi's sarcoma virus. (11342372)
2001
42
Acute pulmonary hypertension following paclitaxel in a patient with AIDS-related primary effusion lymphoma. (11422257)
2001
43
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
44
Herpesvirus 8-negative primary effusion lymphoma associated with hepatitis C virus. (10632503)
2000
45
Characterization of a novel HHV-8-positive cell line reveals implications for the pathogenesis and cell cycle control of primary effusion lymphoma. (10914556)
2000
46
Unusual presentation of "extracavitary" primary effusion lymphoma in previously unknown HIV disease. (11100630)
2000
47
HIV/AIDS case histories: diagnostic problems. Primary effusion lymphoma. (11361976)
1998
48
Establishment and characterization of EBV-positive and EBV-negative primary effusion lymphoma cell lines harbouring human herpesvirus type-8. (9734661)
1998
49
Kaposi's sarcoma-associated herpesvirus: a lymphotropic human herpesvirus associated with Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. (9044510)
1997
50
Kaposi's sarcoma-associated herpesvirus (KSHV): a new viral pathogen associated with Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. (9265864)
1997

Genetic Variations for Primary Effusion Lymphoma

Expression for genes affiliated with Primary Effusion Lymphoma

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
54Reactome, 52QIAGEN, 38NCBI BioSystems Database, 30KEGG
See all sources

Compounds for genes affiliated with Primary Effusion Lymphoma

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 24HMDB
See all sources

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

(show all 17)
idCompoundScoreTop Affiliating Genes
1doxorubicin45 50 1112.5TNFRSF10B, CASP2, BCL6, CDKN1B, CD58, MYB
2serine4510.5CDKN1B, CASP2, TNFRSF10B, POU2F2, TCL1A, SELPLG
3flavopiridol45 1111.5CDKN1B, TNFRSF10B, CASP2
4estrogen4510.5BCL6
5paraffin4510.5BCL6, LYVE1, CDKN1B, CASP2, SKP2
6thymidine45 2411.5CDKN1B, TNFRSF10B, MYB, CD2, LYVE1
7threonine4510.5MYB, SELPLG, TCL1A, CASP2, CDKN1B, TNFRSF10B
8butyrate4510.4MYB
9vegf4510.4VEGFB, MYB, XBP1, LYVE1, TNFRSF10B, CDKN1B
10oligonucleotide4510.4SKP2, LYVE1, MYB, POU2F2, CASP2, CDKN1B
11lipid4510.4MYB, TNFRSF10B, IRF3, CD58, CD2, XBP1
12retinoic acid45 2411.4BCL6, CASP2, TNFRSF10B, IRF3, MYB, CD58
13oxygen45 2411.4SELPLG, VEGFB, TNFRSF10B, CASP2, CDKN1B, MYB
14wortmannin4510.3CDKN1B, CD2, TNFRSF10B, CASP2
15tyrosine4510.3POU2AF1, BCL6, SELPLG, KRT15, CDKN1B, CD2
16alefacept45 1111.3CD2, CD58
17steroid459.9IRF3, POU2F2, MYB, LYVE1, CASP2

GO Terms for genes affiliated with Primary Effusion Lymphoma

Sources:
16Gene Ontology
See all sources

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

idNameGO IDScoreTop Affiliating Genes
1transcription from RNA polymerase II promoterGO:00636610.3IRF3, POU2AF1, POU2F2, XBP1
2blood coagulationGO:00759610.2CD58, VEGFB, SELPLG, CD2, MYB
3leukocyte migrationGO:05090010.1CD2, SELPLG, CD58

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

idNameGO IDScoreTop Affiliating Genes
1receptor activityGO:00487210.3TNFRSF10B, CD2, CD7, LYVE1
2protein bindingGO:0055159.5CORO1A, CASP2, TNFRSF10B, IRF3, VEGFB, MYB

Products for genes affiliated with Primary Effusion Lymphoma

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Primary Effusion Lymphoma

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet