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Acute renal failure is prevalent in patients with thrombotic thrombocytopenic purpura associated with low plasma ADAMTS 13 activity
Author(s) -
Zafrani L.,
Mariotte E.,
Darmon M.,
Canet E.,
Merceron S.,
Boutboul D.,
Veyradier A.,
Galicier L.,
Azoulay E.
Publication year - 2015
Publication title -
journal of thrombosis and haemostasis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.947
H-Index - 178
eISSN - 1538-7836
pISSN - 1538-7933
DOI - 10.1111/jth.12826
Subject(s) - adamts13 , thrombotic thrombocytopenic purpura , medicine , thrombotic microangiopathy , cardiology , von willebrand factor , platelet , disease
Summary Background Among patients with thrombotic microangiopathies, acute kidney injury ( AKI ) is the hallmark of hemolytic uremic syndrome ( HUS ) and is largely underestimated in patients with thrombotic thrombocytopenic purpura ( TTP ). Objective We sought to report AKI features and outcomes in patients with TTP . Methods We conducted a retrospective study of 92 patients with TTP assessed by low ADAMTS 13 activity (< 10%) between 2001 and 2013. A logistic regression identified variables independently associated with AKI . Results Among the 92 patients, 54 (58.7%) presented with AKI , including 25 (46.3%) with stage 3 AKI . Fourteen (27.4%) patients had a nephrotic‐range proteinuria and 21 (45.6%) had hemoglobinuria. Hematuria and leucocyturia were detected in 19 (41.3%) and 16 patients (36.4%), respectively. Renal replacement therapy ( RRT ) was required in 14 patients (25.9%). Six months after TTP remission, RRT ‐free patients had median ( IQR ) MDRD (Modification of Diet in Renal Disease formula estimating the glomerular filtration rate) of 93 mL min −1 per 1.73 m 2 (68.8–110) and three patients required long‐term dialysis. Mild or moderate chronic renal disease occurred in 23/54 (42.6%) AKI patients. By multivariate analysis, serum level of complement component 3 at admission was the only factor independently associated with AKI ( OR per 0.25 unit decrease of C3, 0.85; CI , 1.82–8.33; P = 0.001). Conclusions In patients with TTP , AKI is present in more than half the patients, and half of those will have lasting renal effects. Further studies to better understand the pathophysiology of renal involvement in patients with TTP and to identify a subset of patients with TTP syndrome overlapping HUS are warranted.