aHUSとアフェレシス(<特集>小児疾患とアフェレシス)

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  • 芦田,明
    大阪医科大学医学部泌尿生殖・発達医学講座小児科
  • 玉井,浩
    大阪医科大学医学部泌尿生殖・発達医学講座小児科

書誌事項

タイトル別名
  • Atypical Hemolytic Uremic Syndrome and Apheresis Therapy

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Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury. HUS comprises two major clinical categories: diarrhea-associated, typical HUS (D+HUS) and the diarrhea negative atypical form known as atypical HUS (aHUS). aHUS is rare and has a poor prognosis with a high mortality rate and a high rate of progression to end-stage renal failure in comparison with the typical form. Recent studies have revealed that complement dysregulation is one of the major reasons for the variable etiology of aHUS. Plasma exchange (PE) has been recommended as a first-line rescue therapy for such aHUS episodes, and for prevention of relapse. In addition, eculizumab (a humanized monoclonal anti-C5 antibody) has been used for complement-associated HUS therapy, and has been shown to be effective and safe. Therefore, eculizumab therapy, instead of plasma therapy, could become a first-line treatment for complement-associated HUS. With the increase in treatment options, it will be important to differentiate cases for which plasma exchange is best indicated. This review summarizes current knowledge regarding the pathophysiology of aHUS, the accuracy of diagnosis, and the available treatment options.

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