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Multicentric Castleman disease(MCD)

MedGen UID:
233197
Concept ID:
C1334815
Neoplastic Process
Synonyms: Idiopathic multicentric Castleman's disease; MCD; Multicentric plasma cell variant of Castleman's disease; Plasmablastic multicentric Castleman disease
SNOMED CT: Multicentric Castleman disease (1156805003)
 
Monarch Initiative: MONDO:0019754
Orphanet: ORPHA570438

Definition

A form of angiofollicular lymphoid hyperplasia characterized by fever, generalized lymphadenopathy, hypergammaglobulinemia, and dysfunction of multiple organs. Other signs and symptoms include anemia, thrombocytopenia, hepatomegaly, peripheral neuropathy and pleural effusions. Morphologically, in the majority of cases the lymph nodes show features of angiofollicular lymphoid hyperplasia of the plasma cell type. In a minority of cases, changes of angiofollicular lymphoid hyperplasia of the hyaline-vascular type are seen. In contrast to patients with localized disease who are usually cured following resection of the lesion, patients with the multicentric form of the disease may follow a progressive clinical course, complicated by infection, Kaposi sarcoma, or lymphoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMulticentric Castleman disease
Follow this link to review classifications for Multicentric Castleman disease in Orphanet.

Professional guidelines

PubMed

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
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
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

Recent clinical studies

Etiology

Carbone A, Vaccher E, Gloghini A
Blood 2022 Feb 17;139(7):995-1012. doi: 10.1182/blood.2020005469. PMID: 34469512
Wang W, Medeiros LJ
Surg Pathol Clin 2019 Sep;12(3):849-863. Epub 2019 May 23 doi: 10.1016/j.path.2019.03.003. PMID: 31352991
Vangipuram R, Tyring SK
Cancer Treat Res 2019;177:1-21. doi: 10.1007/978-3-030-03502-0_1. PMID: 30523619
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
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

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
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
Chen LYC, Mattman A, Seidman MA, Carruthers MN
Haematologica 2019 Mar;104(3):444-455. Epub 2019 Jan 31 doi: 10.3324/haematol.2018.205526. PMID: 30705099Free PMC Article
Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772
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

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
Rehman MEU, Chattaraj A, Neupane K, Rafae A, Saeed S, Basit J, Ibrahim A, Khouri J, Mukherjee S, Anwer F
Eur J Haematol 2022 Oct;109(4):309-320. Epub 2022 Jul 13 doi: 10.1111/ejh.13823. PMID: 35770616
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
Fajgenbaum DC
Blood 2018 Nov 29;132(22):2323-2330. doi: 10.1182/blood-2018-05-848671. PMID: 30487129Free PMC Article
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

Prognosis

Wang W, Medeiros LJ
Surg Pathol Clin 2019 Sep;12(3):849-863. Epub 2019 May 23 doi: 10.1016/j.path.2019.03.003. PMID: 31352991
Chen LYC, Mattman A, Seidman MA, Carruthers MN
Haematologica 2019 Mar;104(3):444-455. Epub 2019 Jan 31 doi: 10.3324/haematol.2018.205526. PMID: 30705099Free PMC Article
Vangipuram R, Tyring SK
Cancer Treat Res 2019;177:1-21. doi: 10.1007/978-3-030-03502-0_1. PMID: 30523619
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
Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772

Clinical prediction guides

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
Pierson SK, Katz L, Williams R, Mumau M, Gonzalez M, Guzman S, Rubenstein A, Oromendia AB, Beineke P, Fosså A, van Rhee F, Fajgenbaum DC
Nat Commun 2022 Nov 24;13(1):7236. doi: 10.1038/s41467-022-34873-7. PMID: 36433996Free PMC Article
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
Fajgenbaum DC
Blood 2018 Nov 29;132(22):2323-2330. doi: 10.1182/blood-2018-05-848671. PMID: 30487129Free PMC Article
Oksenhendler E
Curr Opin HIV AIDS 2009 Jan;4(1):16-21. doi: 10.1097/coh.0b013e328319bca9. PMID: 19343828

Recent systematic reviews

Rehman MEU, Chattaraj A, Neupane K, Rafae A, Saeed S, Basit J, Ibrahim A, Khouri J, Mukherjee S, Anwer F
Eur J Haematol 2022 Oct;109(4):309-320. Epub 2022 Jul 13 doi: 10.1111/ejh.13823. PMID: 35770616
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
Zanelli M, Zizzo M, Bisagni A, Froio E, De Marco L, Valli R, Filosa A, Luminari S, Martino G, Massaro F, Fratoni S, Ascani S
Ann Hematol 2020 Oct;99(10):2243-2253. Epub 2020 Apr 19 doi: 10.1007/s00277-020-04024-3. PMID: 32307569
Li J, Zhao S, Wang J, Chen J, Wen W, Zhang Q
Tumour Biol 2016 Mar;37(3):3619-37. Epub 2015 Oct 12 doi: 10.1007/s13277-015-4205-5. PMID: 26459310
Rohner E, Wyss N, Trelle S, Mbulaiteye SM, Egger M, Novak U, Zwahlen M, Bohlius J
Syst Rev 2014 Feb 12;3:11. doi: 10.1186/2046-4053-3-11. PMID: 24521144Free PMC Article

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