KEGG   DISEASE: 自己免疫性溶血性貧血
エントリ  
H01585                                                             
名称    
自己免疫性溶血性貧血
概要    
Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by the increased destruction of red blood cells (RBCs) by anti-RBC autoantibodies with or without complement activation. AIHAs can be distinguished on the basis of the autoantibody Ig class and thermal characteristics, i.e. the optimal temperature of reaction with autologous erythrocytes, in warm (wAIHA), cold (cold agglutinin disease; CAD), and mixed forms, although atypical cases of difficult diagnostic classification are reported with increasing frequency. Moreover, AIHAs are classified in primary (idiopathic), in which hemolysis dominates the clinical picture in the absence of any other coexisting disorder, and in secondary forms, accompanying and complicating an underlying disease. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The diagnosis is usually simple, based on the presence of hemolytic anemia and serological evidence of anti-erythrocyte antibodies, detectable by the direct antiglobulin test (DAT).
カテゴリ  
循環器疾患; 血液疾患; 免疫系疾患
階層分類  
ヒト疾患 [BR:jp08402]
 循環器系疾患
  血液疾患
   H01585  自己免疫性溶血性貧血
ICD-11 による疾患分類 [BR:jp08403]
 03 血液・造血器の疾患
  貧血または他の赤血球系疾患
   溶血性貧血
    後天性溶血性貧血
     3A20  後天性溶血性貧血, 免疫性
      H01585  自己免疫性溶血性貧血
特定疾患 (難病) [jp08407.html]
 H01585
治療薬   
デキサメタゾン [DR:D00292]
デキサメタゾンリン酸エステルナトリウム [DR:D00975]
ベタメタゾンリン酸エステルナトリウム [DR:D00972]
トリアムシノロンアセトニド [DR:D00983]
ヒドロコルチゾン [DR:D00088]
コルチゾン酢酸エステル [DR:D00973]
トリアムシノロン [DR:D00385]
ベタメタゾン [DR:D00244]
プレドニゾロン [DR:D00472]
メチルプレドニゾロン [DR:D00407]
メチルプレドニゾロン酢酸エステル [DR:D00979]
リンク   
ICD-11: 3A20
ICD-10: D59.0 D59.1
MeSH: D000744
文献    
  著者
Barcellini W
  タイトル
New Insights in the Pathogenesis of Autoimmune Hemolytic Anemia.
  雑誌
Transfus Med Hemother 42:287-93 (2015)
DOI:10.1159/000439002
文献    
  著者
Zanella A, Barcellini W
  タイトル
Treatment of autoimmune hemolytic anemias.
  雑誌
Haematologica 99:1547-54 (2014)
DOI:10.3324/haematol.2014.114561
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