DISEASE: Castleman disease

Entry

H01479                      Disease

Name

Castleman disease

Description

Castleman disease is a rare lymphoproliferative disorder with two primary subtypes that vary in presentation and course. Unicentric Castleman disease is localized and carries an excellent prognosis. Multicentric Castleman disease can be associated with HIV and human herpesvirus-8 and is characterized by generalized lymphadenopathy and systemic symptoms, such as fever, fatigue, anorexia, anemia, and cachexia. Castleman disease is unique in that dysregulated secretion of interleukin-6 (IL-6) plays a central pathogenetic role, although the exact events precipitating the oversecretion of IL-6 are unknown. Recently, two antibody-based therapeutics targeting components of the IL-6/IL-6R complex have been approved for the treatment of Castleman disease. Tocilizumab is a monoclonal antibody directed against the IL-6 receptor, and siltuximab is a monoclonal antibody specific for IL-6.

Category

Immune system disease

Brite

Human diseases [BR:br08402]
Immune system diseases
  Allergies and autoimmune diseases
   H01479  Castleman disease
Human diseases in ICD-11 classification [BR:br08403]
04 Diseases of the immune system
  Certain disorders involving the immune system
   4B2Y  Other specified disorders involving the immune system
    H01479  Castleman disease

Related
pathway

hsa04060

Cytokine-cytokine receptor interaction

hsa04630

JAK-STAT signaling pathway

hsa04640

Hematopoietic cell lineage

hsa04659

Th17 cell differentiation

Gene

IL6 [HSA:3569] [KO:K05405]

Drug

Siltuximab [DR:D09669]

Other DBs

ICD-11:

4B2Y

ICD-10:

D36.0

MeSH:

D005871

OMIM:

148000

Reference

PMID:25310208

Authors

Soumerai JD, Sohani AR, Abramson JS

Title

Diagnosis and management of Castleman disease.

Journal

Cancer Control 21:266-78 (2014)
DOI:10.1177/107327481402100403

Reference

PMID:23375540

Authors

Puram SV, Hasserjian RP, Faquin WC, Lin HW, Rocco JW

Title

Castleman disease presenting in the neck: report of a case and review of the literature.

Journal

Am J Otolaryngol 34:239-44 (2013)
DOI:10.1016/j.amjoto.2012.11.007

Reference

PMID:26555695

Authors

Ranganath S, Bhandari A, Avitahl-Curtis N, McMahon J, Wachtel D, Zhang J, Leitheiser C, Bernier SG, Liu G, Tran TT, Celino H, Tobin J, Jung J, Zhao H, Glen KE, Graul C, Griffin A, Schairer WC, Higgins C, Reza TL, Mowe E, Rivers S, Scott S, Monreal A, Shea C, Bourne G, Coons C, Smith A, Tang K, Mandyam RA, Masferrer J, Liu D, Patel DV, Fretzen A, Murphy CA, Milne GT, Smythe ML, Carlson KE

Title

Discovery and Characterization of a Potent Interleukin-6 Binding Peptide with Neutralizing Activity In Vivo.

Journal

PLoS One 10:e0141330 (2015)
DOI:10.1371/journal.pone.0141330

» Japanese version

All links

Disease (1)   
   OMIM (1)   
Drug (2)   
   KEGG DRUG (2)   
Gene (2)   
   KEGG ORTHOLOGY (1)   
   KEGG GENES (1)   
Literature (3)   
   PubMed (3)   
All databases (8)   

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