Korean J Intern Med.  2020 Jan;35(1):25-40. 10.3904/kjim.2019.388.

Consensus regarding diagnosis and management of atypical hemolytic uremic syndrome

Affiliations
  • 1Division of Nephrology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
  • 2Division of Pediatric Nephrology, Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Korea
  • 3Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 4Division of Hematology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
  • 5Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 6Department of Pathology, Seoul National University Hospital, Seoul, Korea
  • 7Division of Nephrology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 8Division of Nephrology, Department of Internal Medicine, Korea University Anam Hospital, Seoul, Korea
  • 9Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 10Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
  • 11Department of Internal Medicine, CHA University School of Medicine, Seongnam, Korea

Abstract

Thrombotic microangiopathy (TMA) is defined by specific clinical characteristics, including microangiopathic hemolytic anemia, thrombocytopenia, and pathologic evidence of endothelial cell damage, as well as the resulting ischemic end-organ injuries. A variety of clinical scenarios have features of TMA, including infection, pregnancy, malignancy, autoimmune disease, and medications. These overlapping manifestations hamper differential diagnosis of the underlying pathogenesis, despite recent advances in understanding the mechanisms of several types of TMA syndrome. Atypical hemolytic uremic syndrome (aHUS) is caused by a genetic or acquired defect in regulation of the alternative complement pathway. It is important to consider the possibility of aHUS in all patients who exhibit TMA with triggering conditions because of the incomplete genetic penetrance of aHUS. Therapeutic strategies for aHUS are based on functional restoration of the complement system. Eculizumab, a monoclonal antibody against the terminal complement component 5 inhibitor, yields good outcomes that include prevention of organ damage and premature death. However, there remain unresolved challenges in terms of treatment duration, cost, and infectious complications. A consensus regarding diagnosis and management of TMA syndrome would enhance understanding of the disease and enable treatment decision-making.

Keyword

Thrombotic microangiopathies; Atypical hemolytic uremic syndrome; Complement pathway, alternative; Diagnosis, differential; Eculizumab
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