HUS and atypical HUS

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http://hdl.handle.net/10138/190710

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Jokiranta , T S 2017 , ' HUS and atypical HUS ' , Blood , vol. 129 , no. 21 , pp. 2847-2856 . https://doi.org/10.1182/blood-2016-11-709865

Title: HUS and atypical HUS
Author: Jokiranta, T. Sakari
Contributor: University of Helsinki, Research Programs Unit
Date: 2017-05-25
Language: eng
Number of pages: 10
Belongs to series: Blood
ISSN: 0006-4971
URI: http://hdl.handle.net/10138/190710
Abstract: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by Shiga toxin-producing Escherichia coli (STEC) infection, as atypical HUS (aHUS), usually caused by uncontrolled complement activation, or as secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection with STEC, whereas aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation. Among the 30% to 50% of patients with HUS who have no detectable complement defect, some have either impaired diacylglycerol kinase epsilon (DGK epsilon) activity, cobalamin C deficiency, or plasminogen deficiency. Some have secondary HUS with a coexisting disease or trigger such as autoimmunity, transplantation, cancer, infection, certain cytotoxic drugs, or pregnancy. The common pathogenetic features in STEC-HUS, aHUS, and secondary HUS are simultaneous damage to endothelial cells, intravascular hemolysis, and activation of platelets leading to a procoagulative state, formation of microthrombi, and tissue damage. In this review, the differences and similarities in the pathogenesis of STEC-HUS, aHUS, and secondaryHUSare discussed. Commonfor the pathogenesis seems to be the vicious cycle of complement activation, endothelial cell damage, platelet activation, and thrombosis. This process can be stopped by therapeutic complement inhibition in most patients with aHUS, but usually not those with a DGK epsilon mutation, and some patients with STEC-HUS or secondary HUS. Therefore, understanding the pathogenesis of the different forms of HUS may prove helpful in clinical practice.
Subject: HEMOLYTIC-UREMIC SYNDROME
THROMBOTIC THROMBOCYTOPENIC PURPURA
PAROXYSMAL-NOCTURNAL HEMOGLOBINURIA
ALTERNATIVE COMPLEMENT PATHWAY
CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME
SYSTEMIC-LUPUS-ERYTHEMATOSUS
TISSUE FACTOR ACTIVITY
FACTOR-H
SHIGA-TOXIN
ENDOTHELIAL-CELLS
3122 Cancers
3121 General medicine, internal medicine and other clinical medicine
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