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KSHV inflammatory cytokine syndrome

MedGen UID:
901443
•Concept ID:
C4086533
•
Disease or Syndrome
Synonyms: Kaposi-sarcoma Associated Herpesvirus (KSHV) Inflammatory Cytokine Syndrome; Kaposi-sarcoma associated Herpesvirus (KSHV) inflammatory cytokine syndrome; KICS; KSHV Inflammatory Cytokine Syndrome
 
Monarch Initiative: MONDO:0023528

Definition

A syndrome caused by Kaposi sarcoma-associated herpesvirus (KSHV) infection. It manifests with fever, weight loss, and fluid retention in the legs or abdomen. Patients are at risk of developing KSHV-related cancers including Kaposi sarcoma and lymphoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKSHV inflammatory cytokine syndrome

Professional guidelines

PubMed

Clinical management of Kaposi sarcoma herpesvirus-associated diseases: an update on disease manifestations and treatment strategies.
Patel R, Lurain K, Yarchoan R, Ramaswami R
Expert Rev Anti Infect Ther 2023 Jul-Dec;21(9):929-941. Epub 2023 Aug 15 doi: 10.1080/14787210.2023.2247161. PMID: 37578202Free PMC Article

Recent clinical studies

Etiology

KSHV vIL-6 enhances inflammatory responses by epigenetic reprogramming.
Inagaki T, Wang KH, Kumar A, Izumiya C, Miura H, Komaki S, Davis RR, Tepper CG, Katano H, Shimoda M, Izumiya Y
PLoS Pathog 2023 Nov;19(11):e1011771. Epub 2023 Nov 7 doi: 10.1371/journal.ppat.1011771. PMID: 37934757Free PMC Article
Characteristics of patients admitted to the ICU with Kaposi sarcoma herpesvirus-associated diseases.
Hansen ME, Mangusan R, Lurain K, Odeny T, George J, Lu C, Manion M, Widell A, Ekwede I, Whitby D, Gulley JL, Kadri SS, Elinoff JM, Barochia A, Torabi-Parizi P, Uldrick TS, Yarchoan R, Ramaswami R
AIDS 2022 Nov 15;36(14):1969-1978. Epub 2022 Jul 16 doi: 10.1097/QAD.0000000000003333. PMID: 35848586Free PMC Article
Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
Kaposi Sarcoma Herpesvirus Inflammatory Cytokine Syndrome-like Clinical Presentation in Human Immunodeficiency Virus-infected Children in Malawi.
El-Mallawany NK, Kamiyango W, Villiera J, Peckham-Gregory EC, Scheurer ME, McAtee CL, Allen CE, Kovarik CL, Frank D, Eason AB, Caro-Vegas C, Chiao EY, Schutze GE, Ozuah NW, Mehta PS, Kazembe PN, Dittmer DP
Clin Infect Dis 2019 Nov 13;69(11):2022-2025. doi: 10.1093/cid/ciz250. PMID: 31102440Free PMC Article
The Contribution of Kaposi's Sarcoma-Associated Herpesvirus to Mortality in Hospitalized Human Immunodeficiency Virus-Infected Patients Being Investigated for Tuberculosis in South Africa.
Blumenthal MJ, Schutz C, Barr D, Locketz M, Marshall V, Whitby D, Katz AA, Uldrick T, Meintjes G, Schäfer G
J Infect Dis 2019 Jul 31;220(5):841-851. doi: 10.1093/infdis/jiz180. PMID: 31004430Free PMC Article

Diagnosis

Clinical management of Kaposi sarcoma herpesvirus-associated diseases: an update on disease manifestations and treatment strategies.
Patel R, Lurain K, Yarchoan R, Ramaswami R
Expert Rev Anti Infect Ther 2023 Jul-Dec;21(9):929-941. Epub 2023 Aug 15 doi: 10.1080/14787210.2023.2247161. PMID: 37578202Free PMC Article
A novel approach for characterization of KSHV-associated multicentric Castleman disease from effusions.
Zhou T, Yuan CM, Lurain K, Rous C, Weaver L, Raffeld M, Stetler-Stevenson M, Uldrick TS, Filie AC, Pittaluga S, Jaffe ES, Marshall V, Moore K, Whitby D, Ramaswami R, Yarchoan R, Wang HW
Br J Haematol 2023 Feb;200(4):462-475. Epub 2022 Oct 20 doi: 10.1111/bjh.18518. PMID: 36264007Free PMC Article
Characteristics of patients admitted to the ICU with Kaposi sarcoma herpesvirus-associated diseases.
Hansen ME, Mangusan R, Lurain K, Odeny T, George J, Lu C, Manion M, Widell A, Ekwede I, Whitby D, Gulley JL, Kadri SS, Elinoff JM, Barochia A, Torabi-Parizi P, Uldrick TS, Yarchoan R, Ramaswami R
AIDS 2022 Nov 15;36(14):1969-1978. Epub 2022 Jul 16 doi: 10.1097/QAD.0000000000003333. PMID: 35848586Free PMC Article
Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
Pathological Features of Kaposi's Sarcoma-Associated Herpesvirus Infection.
Katano H
Adv Exp Med Biol 2018;1045:357-376. doi: 10.1007/978-981-10-7230-7_16. PMID: 29896675

Therapy

Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
Recent Advances in Developing Treatments of Kaposi's Sarcoma Herpesvirus-Related Diseases.
Naimo E, Zischke J, Schulz TF
Viruses 2021 Sep 9;13(9) doi: 10.3390/v13091797. PMID: 34578378Free PMC Article
The Contribution of Kaposi's Sarcoma-Associated Herpesvirus to Mortality in Hospitalized Human Immunodeficiency Virus-Infected Patients Being Investigated for Tuberculosis in South Africa.
Blumenthal MJ, Schutz C, Barr D, Locketz M, Marshall V, Whitby D, Katz AA, Uldrick T, Meintjes G, Schäfer G
J Infect Dis 2019 Jul 31;220(5):841-851. doi: 10.1093/infdis/jiz180. PMID: 31004430Free PMC Article
Kaposi sarcoma herpesvirus-associated cancers and related diseases.
Goncalves PH, Ziegelbauer J, Uldrick TS, Yarchoan R
Curr Opin HIV AIDS 2017 Jan;12(1):47-56. doi: 10.1097/COH.0000000000000330. PMID: 27662501Free PMC Article
Clinical Features and Outcomes of Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV)-associated Inflammation: Prospective Characterization of KSHV Inflammatory Cytokine Syndrome (KICS).
Polizzotto MN, Uldrick TS, Wyvill KM, Aleman K, Marshall V, Wang V, Whitby D, Pittaluga S, Jaffe ES, Millo C, Tosato G, Little RF, Steinberg SM, Sereti I, Yarchoan R
Clin Infect Dis 2016 Mar 15;62(6):730-738. Epub 2015 Dec 12 doi: 10.1093/cid/civ996. PMID: 26658701Free PMC Article

Prognosis

Castleman disease.
Carbone A, Borok M, Damania B, Gloghini A, Polizzotto MN, Jayanthan RK, Fajgenbaum DC, Bower M
Nat Rev Dis Primers 2021 Nov 25;7(1):84. doi: 10.1038/s41572-021-00317-7. PMID: 34824298Free PMC Article
Severe Kaposi Sarcoma in an Urban Public Hospital.
Kasturia SE, Gunthel C, Zeng C, Nguyen ML
AIDS Res Hum Retroviruses 2017 Jun;33(6):583-589. Epub 2017 Jan 31 doi: 10.1089/AID.2016.0141. PMID: 28019110Free PMC Article

Clinical prediction guides

A novel approach for characterization of KSHV-associated multicentric Castleman disease from effusions.
Zhou T, Yuan CM, Lurain K, Rous C, Weaver L, Raffeld M, Stetler-Stevenson M, Uldrick TS, Filie AC, Pittaluga S, Jaffe ES, Marshall V, Moore K, Whitby D, Ramaswami R, Yarchoan R, Wang HW
Br J Haematol 2023 Feb;200(4):462-475. Epub 2022 Oct 20 doi: 10.1111/bjh.18518. PMID: 36264007Free PMC Article
Pathological Features of Kaposi's Sarcoma-Associated Herpesvirus Infection.
Katano H
Adv Exp Med Biol 2018;1045:357-376. doi: 10.1007/978-981-10-7230-7_16. PMID: 29896675

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