Document Detail

Biomarkers of the alternative pathway and terminal complement activity at presentation confirms the clinical diagnosis of aHUS and differentiates aHUS from TTP.
MedLine Citation:
PMID:  24695849     Owner:  NLM     Status:  Publisher    
Atypical hemolytic uremic syndrome (aHUS) is characterized by dysregulated complement activity, the development of a thrombotic microangiopathy (TMA) and widespread end organ injury. aHUS remains a clinical diagnosis without an objective laboratory test to confirm the diagnosis. We performed a retrospective analysis of 103 patients enrolled in the Ohio State University TTP/aHUS Registry presenting with an acute TMA. Nineteen patients were clinically categorized as aHUS based upon the following criteria: 1) platelet count <100 x 10(9)/L, 2) serum creatinine >2.25 mg/dl, and 3) ADAMTS13 activity >10%. Sixteen of 19 patients were treated with plasma exchange therapy, with 6/16(38%) responding to PEX. Nine patients were treated with eculizumab with 7/9 (78%) responding to therapy. In contrast to TTP patients, no aHUS patients demonstrated ULWVF multimers at presentation. Median markers of generalized complement activation (C3a), alternative pathway (Bb), classical/lectin pathway (C4d), and terminal complement activation (C5a and C5b-9) were increased in the plasma of these 19 patients. Compared to a cohort of ADAMTS13 deficient TTP patients (n=38), C5a and C5-9 were significantly higher in the 19 patients clinically characterized as aHUS, suggesting that pretreatment measurements of complement biomarkers C5a and C5b-9 may confirm the diagnosis of aHUS and differentiate it from TTP.
Spero R Cataland; V Michael Holers; Susan Geyer; Shangbin Yang; Haifeng M Wu
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-4-2
Journal Detail:
Title:  Blood     Volume:  -     ISSN:  1528-0020     ISO Abbreviation:  Blood     Publication Date:  2014 Apr 
Date Detail:
Created Date:  2014-4-3     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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