U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Castleman disease(ALNH)

MedGen UID:
42211
•Concept ID:
C0017531
•
Disease or Syndrome
Synonyms: ALNH; Angiofollicular lymph hyperplasia; Angiofollicular lymph node hyperplasia; Castleman's disease; Giant Lymph Node Hyperplasia
SNOMED CT: Angiomatous lymphoid hamartoma (207036003); Castleman disease (207036003); Angiofollicular lymph node hyperplasia (207036003); Benign angiofollicular hyperplasia (207036003); Castleman's disease (207036003); Angiolymphoid hyperplasia (207036003); Angiofollicular lymph node hyperplasia (781094002); Giant lymph node hyperplasia (207036003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015564
Orphanet: ORPHA160

Definition

A benign lymphoproliferative disorder that may present as a localized or multicentric form. The clinical manifestations are heterogeneous, ranging from asymptomatic discrete lymphadenopathy to recurrent episodes of diffuse lymphadenopathy with severe systemic symptoms. [from ORDO]

Professional guidelines

PubMed

POEMS syndrome: 2021 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2021 Jul 1;96(7):872-888. Epub 2021 May 31 doi: 10.1002/ajh.26240. PMID: 34000085
Diagnosis and Management of Castleman Disease.
Abramson JS
J Natl Compr Canc Netw 2019 Nov;17(11.5):1417-1419. doi: 10.6004/jnccn.2019.5037. PMID: 31766018
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2023

Recent clinical studies

Etiology

Hematologic cancers in individuals infected by HIV.
Carbone A, Vaccher E, Gloghini A
Blood 2022 Feb 17;139(7):995-1012. doi: 10.1182/blood.2020005469. PMID: 34469512
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
Overview of Castleman disease.
Dispenzieri A, Fajgenbaum DC
Blood 2020 Apr 16;135(16):1353-1364. doi: 10.1182/blood.2019000931. PMID: 32106302
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139
Idiopathic multicentric Castleman's disease: a systematic literature review.
Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, Krymskaya VP, Kelleher D, Rubenstein AH, Fajgenbaum DC
Lancet Haematol 2016 Apr;3(4):e163-75. Epub 2016 Mar 17 doi: 10.1016/S2352-3026(16)00006-5. PMID: 27063975

Diagnosis

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
Overview of Castleman disease.
Dispenzieri A, Fajgenbaum DC
Blood 2020 Apr 16;135(16):1353-1364. doi: 10.1182/blood.2019000931. PMID: 32106302
Diagnosis and Management of Castleman Disease.
Abramson JS
J Natl Compr Canc Netw 2019 Nov;17(11.5):1417-1419. doi: 10.6004/jnccn.2019.5037. PMID: 31766018
Castleman Disease.
van Rhee F, Munshi NC
Hematol Oncol Clin North Am 2018 Feb;32(1):xiii-xiv. doi: 10.1016/j.hoc.2017.10.001. PMID: 29157623
Pathology of Castleman Disease.
Wu D, Lim MS, Jaffe ES
Hematol Oncol Clin North Am 2018 Feb;32(1):37-52. doi: 10.1016/j.hoc.2017.09.004. PMID: 29157618

Therapy

Recent Advances in Castleman Disease.
Hoffmann C, Hentrich M, Tiemann M, Rosenwald A, Weber F, Willenbacher W, Hübel K
Oncol Res Treat 2022;45(11):693-704. Epub 2022 Oct 11 doi: 10.1159/000526640. PMID: 36219975
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
Overview of Castleman disease.
Dispenzieri A, Fajgenbaum DC
Blood 2020 Apr 16;135(16):1353-1364. doi: 10.1182/blood.2019000931. PMID: 32106302
POEMS Syndrome: 2019 Update on diagnosis, risk-stratification, and management.
Dispenzieri A
Am J Hematol 2019 Jul;94(7):812-827. Epub 2019 May 23 doi: 10.1002/ajh.25495. PMID: 31012139
Interleukin (IL-6) Immunotherapy.
Tanaka T, Narazaki M, Kishimoto T
Cold Spring Harb Perspect Biol 2018 Aug 1;10(8) doi: 10.1101/cshperspect.a028456. PMID: 28778870Free PMC Article

Prognosis

Multicentric Castleman disease and the evolution of the concept.
Zhou T, Wang HW, Pittaluga S, Jaffe ES
Pathologica 2021 Oct;113(5):339-353. doi: 10.32074/1591-951X-351. PMID: 34837092Free 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
POEMS syndrome: A multisystem clonal disorder.
Ali T, Qazilbash MH
Eur J Haematol 2021 Jan;106(1):14-18. Epub 2020 Oct 8 doi: 10.1111/ejh.13514. PMID: 32889731
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
Lymphoproliferative Disorders of the Lung.
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

International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease.
van Rhee F, Oksenhendler E, Srkalovic G, Voorhees P, Lim M, Dispenzieri A, Ide M, Parente S, Schey S, Streetly M, Wong R, Wu D, Maillard I, Brandstadter J, Munshi N, Bowne W, Elenitoba-Johnson KS, Fössa A, Lechowicz MJ, Chandrakasan S, Pierson SK, Greenway A, Nasta S, Yoshizaki K, Kurzrock R, Uldrick TS, Casper C, Chadburn A, Fajgenbaum DC
Blood Adv 2020 Dec 8;4(23):6039-6050. doi: 10.1182/bloodadvances.2020003334. PMID: 33284946Free PMC Article
Imaging and clinical features of Castleman Disease.
Zhao S, Wan Y, Huang Z, Song B, Yu J
Cancer Imaging 2019 Jul 25;19(1):53. doi: 10.1186/s40644-019-0238-0. PMID: 31345268Free 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
Idiopathic multicentric Castleman's disease: a systematic literature review.
Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, Krymskaya VP, Kelleher D, Rubenstein AH, Fajgenbaum DC
Lancet Haematol 2016 Apr;3(4):e163-75. Epub 2016 Mar 17 doi: 10.1016/S2352-3026(16)00006-5. PMID: 27063975

Recent systematic reviews

TAFRO syndrome: A severe manifestation of Sjogren's syndrome? A systematic review.
Grange L, Chalayer E, Boutboul D, Paul S, Galicier L, Gramont B, Killian M
Autoimmun Rev 2022 Aug;21(8):103137. Epub 2022 Jul 6 doi: 10.1016/j.autrev.2022.103137. PMID: 35803499
Efficacy and safety of regimens used for the treatment of multicentric Castleman disease: A systematic review.
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
The role of surgical resection in Unicentric Castleman's disease: a systematic review.
Mitsos S, Stamatopoulos A, Patrini D, George RS, Lawrence DR, Panagiotopoulos N
Adv Respir Med 2018;86(1):36-43. doi: 10.5603/ARM.2018.0008. PMID: 29490420
Idiopathic multicentric Castleman's disease: a systematic literature review.
Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, Krymskaya VP, Kelleher D, Rubenstein AH, Fajgenbaum DC
Lancet Haematol 2016 Apr;3(4):e163-75. Epub 2016 Mar 17 doi: 10.1016/S2352-3026(16)00006-5. PMID: 27063975
Surgery in Castleman's disease: a systematic review of 404 published cases.
Talat N, Belgaumkar AP, Schulte KM
Ann Surg 2012 Apr;255(4):677-84. doi: 10.1097/SLA.0b013e318249dcdc. PMID: 22367441

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      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.
    • Bookshelf
      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, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) B-Cell Lymphomas, 2023

    Consumer resources

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...