KEGG   DISEASE: キャッスルマン病
エントリ  
H01479                                                             
名称    
キャッスルマン病
概要    
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.
カテゴリ  
免疫系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  免疫系が関与する疾患
   4B2Y  その他の明示された免疫系が関与する疾患
    H01479  キャッスルマン病
指定難病 [jp08407.html]
 H01479
パスウェイ 
hsa04060  Cytokine-cytokine receptor interaction
hsa04630  JAK-STAT signaling pathway
hsa04640  Hematopoietic cell lineage
hsa04659  Th17 cell differentiation
病因遺伝子 
IL6 [HSA:3569] [KO:K05405]
治療薬   
トシリズマブ [DR:D02596]
リンク   
ICD-11: 4B2Y
MeSH: D005871
OMIM: 148000
文献    
  著者
Soumerai JD, Sohani AR, Abramson JS
  タイトル
Diagnosis and management of Castleman disease.
  雑誌
Cancer Control 21:266-78 (2014)
DOI:10.1177/107327481402100403
文献    
  著者
Puram SV, Hasserjian RP, Faquin WC, Lin HW, Rocco JW
  タイトル
Castleman disease presenting in the neck: report of a case and review of the literature.
  雑誌
Am J Otolaryngol 34:239-44 (2013)
DOI:10.1016/j.amjoto.2012.11.007
文献    
  著者
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
  タイトル
Discovery and Characterization of a Potent Interleukin-6 Binding Peptide with Neutralizing Activity In Vivo.
  雑誌
PLoS One 10:e0141330 (2015)
DOI:10.1371/journal.pone.0141330
LinkDB    

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