KEGG   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
Pathway
hsa04060  Cytokine-cytokine receptor interaction
hsa04630  JAK-STAT signaling pathway
hsa04640  Hematopoietic cell lineage
Gene
IL6 [HSA:3569] [KO:K05405]
Drug
Siltuximab [DR:D09669]
Other DBs
ICD-11: 4B2Y
ICD-10: D36.0
MeSH: D005871
OMIM: 148000
Reference
  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
  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
  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
Reference
PMID:26749192 (drug)
  Authors
Gonzalez Garcia A, Moreno Cobo MA, Patier de la Pena JL
  Title
Current diagnosis and treatment of Castleman's disease.
  Journal
Rev Clin Esp 216:146-56 (2016)
DOI:10.1016/j.rce.2015.11.008
Reference
PMID:20625121 (drug)
  Authors
van Rhee F, Fayad L, Voorhees P, Furman R, Lonial S, Borghaei H, Sokol L, Crawford J, Cornfeld M, Qi M, Qin X, Herring J, Casper C, Kurzrock R
  Title
Siltuximab, a novel anti-interleukin-6 monoclonal antibody, for Castleman's disease.
  Journal
J Clin Oncol 28:3701-8 (2010)
DOI:10.1200/JCO.2009.27.2377
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