Details of Disease

General Information of Disease (ID: DISSLRAU)

• Disease Name: AIDS-related lymphoma
• Synonyms: AIDS-related lymphoma; PEL; primary Effusion Lymphoma; body cavity-based lymphoma
• Disease Class: 2B33: Malignant haematopoietic neoplasm
• Definition: Primary effusion lymphoma (PEL) is a large B-cell lymphoma located in the body cavities, characterized by pleural, peritoneal, and pericardial fluid lymphomatous effusions and that is always associated with human herpes virus-8 (HHV-8).
• Disease Hierarchy:
  DISUUNPX: Epstein-Barr virus-associated malignant lymphoproliferative disorder
  DISIH1YQ: B-cell lymphoma
  DISSU8N1: Human herpesvirus 8-related tumor
  DISSLRAU: AIDS-related lymphoma
• ICD Code:
  ICD-11: ICD-11: 2B33.5
  ICD-10: ICD-10: C85.9
• Disease Identifiers:
  MONDO ID: MONDO_0018842
  MESH ID: D054685
  UMLS CUI: C1292753
  MedGen ID: 220953
  Orphanet ID: 48686
  SNOMED CT ID: 128800006

Drug-Interaction Atlas (DIA) of This Disease

This Disease is Treated as An Indication in 1 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
MultiStem	DMOT705	Phase 3	NA	[1]

Molecular Interaction Atlas (MIA) of This Disease

This Disease Is Related to 14 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
IKZF3	TTCZVFZ	Limited	Biomarker	[2]
LTA4H	TTXZEAJ	Limited	Altered Expression	[3]
PABPC1	TTHC8EF	Limited	Biomarker	[4]
EPHA7	TTAHTVG	Disputed	Biomarker	[5]
BCL6	TTC9YX5	Strong	Altered Expression	[6]
CRBN	TTDKGTC	Strong	Biomarker	[7]
F10	TTCIHJA	Strong	Altered Expression	[8]
SLC22A2	TT0XOJN	Strong	Altered Expression	[9]
SPHK2	TTCN0M9	Strong	Biomarker	[10]
TCL1A	TTUKRDV	Strong	Biomarker	[11]
TNFRSF8	TT2GM5R	Strong	Altered Expression	[12]
TRIM28	TTQ2BKV	Strong	Altered Expression	[13]
USP7	TTXU3EQ	Strong	Biomarker	[14]
VKORC1	TTEUC8H	Strong	Biomarker	[15]

This Disease Is Related to 21 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
CDC42EP1	OTT525WA	Limited	Biomarker	[16]
CNTRL	OTQI3VBA	Limited	Biomarker	[16]
CORO1C	OTXDF9T3	Limited	Altered Expression	[17]
IRF4	OT1DHQ1P	Limited	Altered Expression	[6]
MNDA	OTCTKR47	Limited	Altered Expression	[18]
OSGIN1	OT9KIVZW	Limited	Biomarker	[19]
PLXNB1	OTCA7JIT	Limited	Altered Expression	[20]
POU2AF1	OTOO6WHL	Limited	Altered Expression	[9]
PPP1R11	OTSHYPPW	Limited	Biomarker	[21]
PWWP3A	OTXQVL4U	Limited	Altered Expression	[22]
SUMO2	OT1Y5IKN	Limited	Biomarker	[23]
CDKN3	OTBE3H07	Strong	Altered Expression	[13]
CLIP1	OTTGAEJE	Strong	Biomarker	[24]
CLIP2	OTSCIQIY	Strong	Biomarker	[24]
FAM107B	OT5RG4J0	Strong	Biomarker	[24]
IKZF1	OTCW1FKL	Strong	Biomarker	[2]
INAFM2	OT2H4HAP	Strong	Biomarker	[25]
PDLIM7	OTAZVODU	Strong	Biomarker	[26]
POU2F2	OTPV0J0C	Strong	Altered Expression	[9]
PRDM1	OTQLSVBS	Strong	Posttranslational Modification	[27]
TCL1B	OT4CSO39	Strong	Biomarker	[11]

References

• [1] ClinicalTrials.gov (NCT02277613) A Phase 2 Trial of AMI MultiStem Therapy in Subjects With Non-ST Elevation Acute Myocardial Infarction. U.S. National Institutes of Health.
• [2] CK1 and IRF4 are essential and independent effectors of immunomodulatory drugs in primary effusion lymphoma.Blood. 2018 Aug 9;132(6):577-586. doi: 10.1182/blood-2018-01-828418. Epub 2018 Jun 28.
• [3] Leukotriene A4 hydrolase expression in PEL cells is regulated at the transcriptional level and leads to increased leukotriene B4 production.J Immunol. 2006 Jun 1;176(11):7051-61. doi: 10.4049/jimmunol.176.11.7051.
• [4] Expression of Kaposi's sarcoma-associated herpesvirus-encoded K10/10.1 protein in tissues and its interaction with poly(A)-binding protein.Virology. 2006 Aug 15;352(1):100-9. doi: 10.1016/j.virol.2006.04.009. Epub 2006 May 22.
• [5] EphA7 Functions as Receptor on BJAB Cells for Cell-to-Cell Transmission of the Kaposi's Sarcoma-Associated Herpesvirus and for Cell-Free Infection by the Related Rhesus Monkey Rhadinovirus.J Virol. 2019 Jul 17;93(15):e00064-19. doi: 10.1128/JVI.00064-19. Print 2019 Aug 1.
• [6] KSHV vIRF4 enhances BCL6 transcription via downregulation of IRF4 expression.Biochem Biophys Res Commun. 2018 Feb 19;496(4):1128-1133. doi: 10.1016/j.bbrc.2018.01.154.
• [7] Genome-wide CRISPR screens reveal genetic mediators of cereblon modulator toxicity in primary effusion lymphoma.Blood Adv. 2019 Jul 23;3(14):2105-2117. doi: 10.1182/bloodadvances.2019031732.
• [8] 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.J Gen Virol. 2011 Feb;92(Pt 2):421-31. doi: 10.1099/vir.0.025494-0. Epub 2010 Oct 27.
• [9] Role of defective Oct-2 and OCA-B expression in immunoglobulin production and Kaposi's sarcoma-associated herpesvirus lytic reactivation in primary effusion lymphoma.J Virol. 2009 May;83(9):4308-15. doi: 10.1128/JVI.02196-08. Epub 2009 Feb 18.
• [10] ABC294640, A Novel Sphingosine Kinase 2 Inhibitor, Induces Oncogenic Virus-Infected Cell Autophagic Death and Represses Tumor Growth.Mol Cancer Ther. 2017 Dec;16(12):2724-2734. doi: 10.1158/1535-7163.MCT-17-0485. Epub 2017 Sep 22.
• [11] TCL1 oncogene expression in B cell subsets from lymphoid hyperplasia and distinct classes of B cell lymphoma.Lab Invest. 2001 Apr;81(4):555-64. doi: 10.1038/labinvest.3780264.
• [12] Body Cavity-Based Lymphoma in a Country with Low Human Immunodeficiency Virus Prevalence: A Series of 17 Cases from the Consortium for Improving Survival of Lymphoma.Cancer Res Treat. 2019 Oct;51(4):1302-1312. doi: 10.4143/crt.2018.555. Epub 2019 Feb 14.
• [13] Activated Nrf2 Interacts with Kaposi's Sarcoma-Associated Herpesvirus Latency Protein LANA-1 and Host Protein KAP1 To Mediate Global Lytic Gene Repression.J Virol. 2015 Aug;89(15):7874-92. doi: 10.1128/JVI.00895-15. Epub 2015 May 20.
• [14] USP7-Dependent Regulation of TRAF Activation and Signaling by a Viral Interferon Regulatory Factor Homologue.J Virol. 2020 Jan 6;94(2):e01553-19. doi: 10.1128/JVI.01553-19. Print 2020 Jan 6.
• [15] Promotion of Endoplasmic Reticulum-Associated Degradation of Procathepsin D by Human Herpesvirus 8-Encoded Viral Interleukin-6.J Virol. 2015 Aug;89(15):7979-90. doi: 10.1128/JVI.00375-15. Epub 2015 May 27.
• [16] Biscoclaurine alkaloid cepharanthine inhibits the growth of primary effusion lymphoma in vitro and in vivo and induces apoptosis via suppression of the NF-kappaB pathway.Int J Cancer. 2009 Sep 15;125(6):1464-72. doi: 10.1002/ijc.24521.
• [17] Primary effusion lymphoma: genomic profiling revealed amplification of SELPLG and CORO1C encoding for proteins important for cell migration.J Pathol. 2010 Oct;222(2):166-79. doi: 10.1002/path.2752.
• [18] Latency-associated nuclear antigen of Kaposi's sarcoma-associated herpesvirus interacts with human myeloid cell nuclear differentiation antigen induced by interferon alpha.Virus Genes. 2003 Dec;27(3):237-47. doi: 10.1023/a:1026391715071.
• [19] Genomic analysis of xCT-mediated regulatory network: Identification of novel targets against AIDS-associated lymphoma.Oncotarget. 2015 May 20;6(14):12710-22. doi: 10.18632/oncotarget.3710.
• [20] Molecular mechanisms of activating c-MET in KSHV+ primary effusion lymphoma.Oncotarget. 2017 Mar 14;8(11):18373-18380. doi: 10.18632/oncotarget.15444.
• [21] Ribonucleotide reductase represents a novel therapeutic target in primary effusion lymphoma.Oncogene. 2017 Aug 31;36(35):5068-5074. doi: 10.1038/onc.2017.122. Epub 2017 May 1.
• [22] Expression of MUM1/IRF4 selectively clusters with primary effusion lymphoma among lymphomatous effusions: implications for disease histogenesis and pathogenesis.Br J Haematol. 2000 Oct;111(1):247-57. doi: 10.1046/j.1365-2141.2000.02329.x.
• [23] Identification of viral SIM-SUMO2-interaction inhibitors for treating primary effusion lymphoma.PLoS Pathog. 2019 Dec 12;15(12):e1008174. doi: 10.1371/journal.ppat.1008174. eCollection 2019 Dec.
• [24] Ago HITS-CLIP expands understanding of Kaposi's sarcoma-associated herpesvirus miRNA function in primary effusion lymphomas. PLoS Pathog. 2012;8(8):e1002884.
• [25] Establishment and characterization of a novel VEGF-producing HHV-8-unrelated PEL-like lymphoma cell line, OGU1.Eur J Haematol. 2016 Feb;96(2):144-51. doi: 10.1111/ejh.12559. Epub 2015 Apr 21.
• [26] Molecular profile of Epstein-Barr virus infection in HHV-8-positive primary effusion lymphoma.Leukemia. 2000 Feb;14(2):271-7. doi: 10.1038/sj.leu.2401651.
• [27] PEGylated siRNA lipoplexes for silencing of BLIMP-1 in Primary Effusion Lymphoma: In vitro evidences of antitumoral activity.Eur J Pharm Biopharm. 2016 Feb;99:7-17. doi: 10.1016/j.ejpb.2015.11.007. Epub 2015 Nov 25.