KEGG   DISEASE: クロウ・深瀬症候群
エントリ  
H01461                                                             

名称    
クロウ・深瀬症候群;
POEMS 症候群
概要    
Crow-Fukase syndrome, also called POEMS syndrome, is a rare paraneoplastic syndrome due to an underlying plasma cell neoplasm, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes as its salient features. Major diagnostic criteria include sclerotic bone lesions, elevated serum or plasma levels of vascular endothelial growth factor (VEGF), and Castleman disease. The misdiagnosis is very common due to its rarity and complicated clinical manifestations. Recognition of the complex of clinical complications is the first step in effectively managing the disease. The pathogenesis is not well understood. VEGF appears to play an important role in the disease and is especially useful for monitoring therapy, but it is not likely the sole factor driving the disease. The most commonly used therapies include alkylators and steroids, high-dose chemotherapy with peripheral blood stem cell transplantation, lenalidomide, and bortezomib.
カテゴリ  
その他の疾患
階層分類  
ヒト疾患 [BR:jp08402]
 その他の疾患
  その他
   H01461  クロウ・深瀬症候群
ICD-11 による疾患分類 [BR:jp08403]
 02 腫瘍
  造血またはリンパ組織の腫瘍
   成熟B細胞腫瘍
    2A83  形質細胞腫瘍
     H01461  クロウ・深瀬症候群
特定疾患 (難病) [jp08407.html]
 H01461
治療薬   
サリドマイド [DR:D00754]
リンク   
ICD-11: 2A83.Y
ICD-10: D47.7
MeSH: D016878
文献    
  著者
Dispenzieri A, Buadi FK
  タイトル
A review of POEMS syndrome.
  雑誌
Oncology (Williston Park) 27:1242-50 (2013)
文献    
  著者
Cui RT, Yu SY, Huang XS, Zhang JT, Tian CL, Dou LP, Pu CQ
  タイトル
Incidence and risk factors of pleural effusions in patients with POEMS syndrome.
  雑誌
Hematol Oncol 33:80-4 (2015)
DOI:10.1002/hon.2135
文献    
  著者
Raheja D, Specht C, Simmons Z
  タイトル
Paraproteinemic neuropathies.
  雑誌
Muscle Nerve 51:1-13 (2015)
DOI:10.1002/mus.24471
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