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Idiopathic multicentric Castleman disease

MedGen UID:
1680858
•Concept ID:
C5197665
•
Disease or Syndrome
Synonyms: HHV-8-negative idiopathic multicentric Castleman disease; HHV-8-negative MCD; Idiopathic multicentric Castleman's disease
 
Monarch Initiative: MONDO:0035838
Orphanet: ORPHA570431

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic multicentric Castleman disease

Professional guidelines

PubMed

Idiopathic multicentric Castleman disease: An update in diagnosis and treatment advances.
Lang E, van Rhee F
Blood Rev 2024 Mar;64:101161. Epub 2023 Dec 5 doi: 10.1016/j.blre.2023.101161. PMID: 38087716
International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease.
van Rhee F, Voorhees P, Dispenzieri A, Fosså A, Srkalovic G, Ide M, Munshi N, Schey S, Streetly M, Pierson SK, Partridge HL, Mukherjee S, Shilling D, Stone K, Greenway A, Ruth J, Lechowicz MJ, Chandrakasan S, Jayanthan R, Jaffe ES, Leitch H, Pemmaraju N, Chadburn A, Lim MS, Elenitoba-Johnson KS, Krymskaya V, Goodman A, Hoffmann C, Zinzani PL, Ferrero S, Terriou L, Sato Y, Simpson D, Wong R, Rossi JF, Nasta S, Yoshizaki K, Kurzrock R, Uldrick TS, Casper C, Oksenhendler E, Fajgenbaum DC
Blood 2018 Nov 15;132(20):2115-2124. Epub 2018 Sep 4 doi: 10.1182/blood-2018-07-862334. PMID: 30181172Free PMC Article
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Castleman Disease, 2024

Recent clinical studies

Etiology

Asymptomatic multicentric Castleman disease: a potential early stage of idiopathic MCD.
Zhang L, Liu QH, Zhou H, Zhang HL, Dong YJ, Wang XB, Wang LQ, Su LP, Yan XJ, Li Y, Zhang MZ, Ding KY, Wang HH, Peng HL, Zhong LY, Yang L, Bi LT, Gao D, Gao GX, Huang L, Sun CY, Song J, Qian WB, Huang WR, Li ZL, Liu Y, Li J
Blood Adv 2024 Nov 12;8(21):5598-5602. doi: 10.1182/bloodadvances.2024013728. PMID: 39293084Free PMC Article
Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.
Fajgenbaum DC
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):318-325. doi: 10.1182/asheducation-2018.1.318. PMID: 30504327Free PMC Article
Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.
Fajgenbaum DC
Blood 2018 Nov 29;132(22):2323-2330. doi: 10.1182/blood-2018-05-848671. PMID: 30487129Free PMC Article
International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease.
van Rhee F, Voorhees P, Dispenzieri A, Fosså A, Srkalovic G, Ide M, Munshi N, Schey S, Streetly M, Pierson SK, Partridge HL, Mukherjee S, Shilling D, Stone K, Greenway A, Ruth J, Lechowicz MJ, Chandrakasan S, Jayanthan R, Jaffe ES, Leitch H, Pemmaraju N, Chadburn A, Lim MS, Elenitoba-Johnson KS, Krymskaya V, Goodman A, Hoffmann C, Zinzani PL, Ferrero S, Terriou L, Sato Y, Simpson D, Wong R, Rossi JF, Nasta S, Yoshizaki K, Kurzrock R, Uldrick TS, Casper C, Oksenhendler E, Fajgenbaum DC
Blood 2018 Nov 15;132(20):2115-2124. Epub 2018 Sep 4 doi: 10.1182/blood-2018-07-862334. PMID: 30181172Free PMC Article
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article

Diagnosis

Idiopathic multicentric Castleman disease: An update in diagnosis and treatment advances.
Lang E, van Rhee F
Blood Rev 2024 Mar;64:101161. Epub 2023 Dec 5 doi: 10.1016/j.blre.2023.101161. PMID: 38087716
Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.
González García A, Fernández-Martín J, Robles Marhuenda Á
Rheumatology (Oxford) 2023 Apr 3;62(4):1426-1435. doi: 10.1093/rheumatology/keac481. PMID: 35997567Free PMC Article
Castleman disease and TAFRO syndrome.
Masaki Y, Arita K, Sakai T, Takai K, Aoki S, Kawabata H
Ann Hematol 2022 Mar;101(3):485-490. Epub 2022 Jan 19 doi: 10.1007/s00277-022-04762-6. PMID: 35044513Free PMC Article
TAFRO Syndrome.
Igawa T, Sato Y
Hematol Oncol Clin North Am 2018 Feb;32(1):107-118. doi: 10.1016/j.hoc.2017.09.009. PMID: 29157612
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS
Blood 2017 Mar 23;129(12):1646-1657. Epub 2017 Jan 13 doi: 10.1182/blood-2016-10-746933. PMID: 28087540Free PMC Article

Therapy

Treatment consistent with idiopathic multicentric Castleman disease guidelines is associated with improved outcomes.
Pierson SK, Lim MS, Srkalovic G, Brandstadter JD, Sarmiento Bustamante M, Shyamsundar S, Mango N, Lavery C, Austin B, Alapat D, Lechowicz MJ, Bagg A, Li H, Casper C, van Rhee F, Fajgenbaum DC
Blood Adv 2023 Nov 14;7(21):6652-6664. doi: 10.1182/bloodadvances.2023010745. PMID: 37656441Free PMC Article
Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.
Fajgenbaum DC
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):318-325. doi: 10.1182/asheducation-2018.1.318. PMID: 30504327Free PMC Article
International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease.
van Rhee F, Voorhees P, Dispenzieri A, Fosså A, Srkalovic G, Ide M, Munshi N, Schey S, Streetly M, Pierson SK, Partridge HL, Mukherjee S, Shilling D, Stone K, Greenway A, Ruth J, Lechowicz MJ, Chandrakasan S, Jayanthan R, Jaffe ES, Leitch H, Pemmaraju N, Chadburn A, Lim MS, Elenitoba-Johnson KS, Krymskaya V, Goodman A, Hoffmann C, Zinzani PL, Ferrero S, Terriou L, Sato Y, Simpson D, Wong R, Rossi JF, Nasta S, Yoshizaki K, Kurzrock R, Uldrick TS, Casper C, Oksenhendler E, Fajgenbaum DC
Blood 2018 Nov 15;132(20):2115-2124. Epub 2018 Sep 4 doi: 10.1182/blood-2018-07-862334. PMID: 30181172Free PMC Article
Treatment of Idiopathic Castleman Disease.
van Rhee F, Greenway A, Stone K
Hematol Oncol Clin North Am 2018 Feb;32(1):89-106. doi: 10.1016/j.hoc.2017.09.008. PMID: 29157622
Siltuximab: a targeted therapy for idiopathic multicentric Castleman disease.
Fajgenbaum DC, Kurzrock R
Immunotherapy 2016;8(1):17-26. Epub 2015 Dec 4 doi: 10.2217/imt.15.95. PMID: 26634298

Prognosis

Asymptomatic multicentric Castleman disease: a potential early stage of idiopathic MCD.
Zhang L, Liu QH, Zhou H, Zhang HL, Dong YJ, Wang XB, Wang LQ, Su LP, Yan XJ, Li Y, Zhang MZ, Ding KY, Wang HH, Peng HL, Zhong LY, Yang L, Bi LT, Gao D, Gao GX, Huang L, Sun CY, Song J, Qian WB, Huang WR, Li ZL, Liu Y, Li J
Blood Adv 2024 Nov 12;8(21):5598-5602. doi: 10.1182/bloodadvances.2024013728. PMID: 39293084Free PMC Article
Peripheral helper-T-cell-derived CXCL13 is a crucial pathogenic factor in idiopathic multicentric Castleman disease.
Harada T, Kikushige Y, Miyamoto T, Uno K, Niiro H, Kawakami A, Koga T, Akashi K, Yoshizaki K
Nat Commun 2023 Oct 31;14(1):6959. doi: 10.1038/s41467-023-42718-0. PMID: 37907518Free PMC Article
TAFRO Syndrome.
Igawa T, Sato Y
Hematol Oncol Clin North Am 2018 Feb;32(1):107-118. doi: 10.1016/j.hoc.2017.09.009. PMID: 29157612
Epidemiology of Castleman Disease.
Simpson D
Hematol Oncol Clin North Am 2018 Feb;32(1):1-10. doi: 10.1016/j.hoc.2017.09.001. PMID: 29157611
TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease.
Srkalovic G, Marijanovic I, Srkalovic MB, Fajgenbaum DC
Bosn J Basic Med Sci 2017 May 20;17(2):81-84. doi: 10.17305/bjbms.2017.1930. PMID: 28135567Free PMC Article

Clinical prediction guides

Asymptomatic multicentric Castleman disease: a potential early stage of idiopathic MCD.
Zhang L, Liu QH, Zhou H, Zhang HL, Dong YJ, Wang XB, Wang LQ, Su LP, Yan XJ, Li Y, Zhang MZ, Ding KY, Wang HH, Peng HL, Zhong LY, Yang L, Bi LT, Gao D, Gao GX, Huang L, Sun CY, Song J, Qian WB, Huang WR, Li ZL, Liu Y, Li J
Blood Adv 2024 Nov 12;8(21):5598-5602. doi: 10.1182/bloodadvances.2024013728. PMID: 39293084Free PMC Article
Single-cell landscape of idiopathic multicentric Castleman disease in identical twins.
Chan JY, Loh JW, Lim JQ, Liany H, Lee ECY, Lee JY, Kannan B, Lim BY, Guo Z, Lim K, Ha JCH, Ng CC, Ko TK, Huang D, Seow DYB, Cheng CL, Chan SH, Ngeow J, Teh BT, Lim ST, Ong CK
Blood 2024 May 2;143(18):1837-1844. doi: 10.1182/blood.2023021992. PMID: 38170173
Treatment consistent with idiopathic multicentric Castleman disease guidelines is associated with improved outcomes.
Pierson SK, Lim MS, Srkalovic G, Brandstadter JD, Sarmiento Bustamante M, Shyamsundar S, Mango N, Lavery C, Austin B, Alapat D, Lechowicz MJ, Bagg A, Li H, Casper C, van Rhee F, Fajgenbaum DC
Blood Adv 2023 Nov 14;7(21):6652-6664. doi: 10.1182/bloodadvances.2023010745. PMID: 37656441Free PMC Article
Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.
Fajgenbaum DC
Hematology Am Soc Hematol Educ Program 2018 Nov 30;2018(1):318-325. doi: 10.1182/asheducation-2018.1.318. PMID: 30504327Free PMC Article
Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.
Fajgenbaum DC
Blood 2018 Nov 29;132(22):2323-2330. doi: 10.1182/blood-2018-05-848671. PMID: 30487129Free PMC Article

Recent systematic reviews

Bone marrow findings of idiopathic Multicentric Castleman disease: A histopathologic analysis and systematic literature review.
Belyaeva E, Rubenstein A, Pierson SK, Dalldorf D, Frank D, Lim MS, Fajgenbaum DC
Hematol Oncol 2022 Apr;40(2):191-201. Epub 2022 Feb 18 doi: 10.1002/hon.2969. PMID: 35104370Free PMC Article

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    Clinical resources

    Practice guidelines

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      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Castleman Disease, 2024

    Reviews

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