NDLI: Clinical evaluation of thrombotic microangiopathy: identification of patients with suspected atypical hemolytic uremic syndrome

Content Provider	Springer Nature : BioMed Central
Author	Shen, Yu-Min
Abstract	Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syndrome consists of hemolytic anemia with schistocytosis, thrombocytopenia, significant renal damage, and/or other organ system dysfunction(s). Patients with aHUS may succumb to the complications of the disease with the very first manifestation; surviving patients often suffer from progressive organ dysfunction with significant morbidity and mortality despite plasma infusion or plasma exchange. Eculizumab, a humanized monoclonal antibody to C5, was approved for treatment of aHUS in 2011. This is an expensive but highly effective therapy changing the lives and improving the outcome of patients with aHUS. Making timely and accurate diagnosis of aHUS can be life-saving if eculizumab treatment is begun promptly. Finding a genetic mutation in a complement regulatory protein is diagnostic with the appropriate clinical syndrome, but at least 30 % of patients do not have defined or reported mutations. Thus the diagnosis rests on the clinical acumen of the physician. However, the clinical manifestations of aHUS are shared by other etiologies of thrombotic microangiopathy. While laboratory finding of undetectable ADAMTS13 activity defines TTP, distinguishing aHUS from the other causes of TMA remains an art. In addition, aHUS can be unmasked by conditions with enhanced complement activation, such as systemic lupus erythematosus, pregnancy, malignant hypertension, and hematopoietic stem cell transplantation. Thus if TMA occurs in the setting of enhanced complement activation, one must consider aHUS as an underlying etiology, especially if treatment of the condition does not resolve the TMA.
Related Links	https://thrombosisjournal.biomedcentral.com/counter/pdf/10.1186/s12959-016-0114-0.pdf
Ending Page	158
Page Count	8
Starting Page	151
File Format	HTM / HTML
ISSN	14779560
DOI	10.1186/s12959-016-0114-0
Journal	Thrombosis Journal
Issue Number	1
Volume Number	14
Language	English
Publisher	BioMed Central
Publisher Date	2016-10-04
Access Restriction	Open
Subject Keyword	Angiology Cardiology Hematology Thrombotic microangiopathy Atypical hemolytic uremic syndrome Thrombotic thrombocytopenic purpura Complement dysregulation
Content Type	Text
Resource Type	Review
Subject	Hematology
Journal Impact Factor	2.6/2023
5-Year Journal Impact Factor	3.1/2023

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