| H01479 | |
| H番号 | 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 |
| ネットワーク | - |
| 病因遺伝子 | IL6 [HSA:3569] [KO:K05405] |
| 病原体 | - |
| 環境要因 | - |
| 発癌物質 | - |
| 治療薬 | トシリズマブ [DR:D02596] シルツキシマブ [DR:D09669] |
| コメント | - |
| リンク | ICD-11: 4B2Y ICD-10: D36.0 MeSH: D005871 OMIM: 148000 |
| 文献 | PMID:25310208 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 PMID:23375540 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 PMID:26555695 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 |