KEGG   DISEASE: Castleman disease
H01479                      Disease                                
Castleman disease
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.
Immune system disease
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
hsa04060  Cytokine-cytokine receptor interaction
hsa04630  JAK-STAT signaling pathway
hsa04640  Hematopoietic cell lineage
hsa04659  Th17 cell differentiation
IL6 [HSA:3569] [KO:K05405]
Siltuximab [DR:D09669]
Other DBs
ICD-11: 4B2Y
ICD-10: D36.0
MeSH: D005871
OMIM: 148000
Soumerai JD, Sohani AR, Abramson JS
Diagnosis and management of Castleman disease.
Cancer Control 21:266-78 (2014)
Puram SV, Hasserjian RP, Faquin WC, Lin HW, Rocco JW
Castleman disease presenting in the neck: report of a case and review of the literature.
Am J Otolaryngol 34:239-44 (2013)
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
Discovery and Characterization of a Potent Interleukin-6 Binding Peptide with Neutralizing Activity In Vivo.
PLoS One 10:e0141330 (2015)

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