KEGG   DISEASE: 自己免疫性血小板減少性紫斑病
エントリ  
H01240                                                             

名称    
自己免疫性血小板減少性紫斑病
概要    
Immune thrombocytopenic purpura (ITP) is an acquired autoimmune disorder, and the most common cause of isolated thrombocytopenia in children. Destruction of autoantibody-sensitized platelets by Fc[gamma]R-bearing phagocytic cells in the reticuloendothelial system plays an important role. ITP is diagnosed clinically, based upon onset of thrombocytopenia in the absence of other hematologic abnormalities, or other causes of low platelets, and with a characteristic blood smear. While around 50% of typical childhood acute ITP is preceded by a viral or bacterial infection and commonly resolves within weeks to months without treatment, fewer adult cases are acute and self-resolving. Typical adult primary ITP is very similar clinically to chronic pediatric ITP. Intravenous immunoglobulin (IVIg) treatment and splenectomy (removal of the platelet-destructing organ) are effective treatment options. It has been reported that the activating FCGR2C-ORF genotype predisposes to ITP by altering the balance of activating and inhibitory Fc[gamma]R on immune cells.
カテゴリ  
免疫系疾患
階層分類  
ヒト疾患 [BR:jp08402]
 免疫系疾患
  原発性免疫不全症候群
   H01240  自己免疫性血小板減少性紫斑病
ICD-11 による疾患分類 [BR:jp08403]
 03 血液・造血器の疾患
  凝固障害, 紫斑病またはその他の出血性または関連病状
   3B64  血小板減少症
    H01240  自己免疫性血小板減少性紫斑病
特定疾患 (難病) [jp08407.html]
 H01240
関連パスウェイ
hsa04145  Phagosome
hsa04380  Osteoclast differentiation
病因遺伝子 
FCGR2C [HSA:9103] [KO:K16824]
環境要因  
Quinine [CPD:C06526]
Trimethoprim-sulfamethoxazole
病原体   
Hepatitis C virus
Human immunodeficiency virus
Helicobacter pylori
治療薬   
ロミプロスチム [DR:D08990]
エルトロンボパグ オラミン [DR:D03978]
リツキシマブ [DR:D02994]
pH4処理酸性人免疫グロブリン [DR:D08779]
ポリエチレングリコール処理人免疫グロブリン [DR:D08783]
乾燥pH4処理人免疫グロブリン [DR:D08784]
乾燥スルホ化人免疫グロブリン [DR:D08786]
乾燥ポリエチレングリコール処理人免疫グロブリン [DR:D08788]
リンク   
ICD-11: 3B64.10
ICD-10: D69.3
MeSH: D016553
OMIM: 188030
文献    
PMID:21632906 (description, environmental factor, drug)
  著者
McCrae K
  タイトル
Immune thrombocytopenia: no longer 'idiopathic'.
  雑誌
Cleve Clin J Med 78:358-73 (2011)
DOI:10.3949/ccjm.78gr.10005
文献    
  著者
Cines DB, McMillan R
  タイトル
Pathogenesis of chronic immune thrombocytopenic purpura.
  雑誌
Curr Opin Hematol 14:511-4 (2007)
DOI:10.1097/MOH.0b013e3282ba5552
文献    
  著者
Bergmann AK, Grace RF, Neufeld EJ
  タイトル
Genetic studies in pediatric ITP: outlook, feasibility, and requirements.
  雑誌
Ann Hematol 89:S95-103 (2010)
DOI:10.1007/s00277-009-0865-9
文献    
  著者
Breunis WB, van Mirre E, Bruin M, Geissler J, de Boer M, Peters M, Roos D, de Haas M, Koene HR, Kuijpers TW
  タイトル
Copy number variation of the activating FCGR2C gene predisposes to idiopathic thrombocytopenic purpura.
  雑誌
Blood 111:1029-38 (2008)
DOI:10.1182/blood-2007-03-079913
LinkDB    

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