Blood Res  
Prognostic utility of ADAMTS13 activity for the atypical hemolytic uremic syndrome (aHUS) and comparison of complement serology between aHUS and thrombotic thrombocytopenic purpura
Jisu Oh1, Doyeun Oh1, Seon Ju Lee2, Jeong Oh Kim2, Nam Keun Kim2, So Young Chong1, Ji Young Huh3, Ross I. Baker4, and the Korean TTP Registry Investigators
1Department of Internal Medicine, CHA Bundang Medical Center, CHA University, 2Institute for Clinical Research, School of Medicine CHA University, 3Department Laboratory Medicine, CHA Bundang Medical Center, CHA University, Seongnam, Korea, 4Western Australian Centre for Thrombosis and Haemostasis, Murdoch University, Perth, Australia
Correspondence to: Doyeun Oh, M.D. Ph.D.
Department of Internal Medicine, CHA Bundang Medical Center, CHA University, 59 Yatapro, Bundang-gu, Seongnam, Gyeonggi, 13496, Korea
E-mail: doh@cha.ac.kr
Published online: September 11, 2019.
© The Korean Journal of Hematology. All rights reserved.

Abstract
Background: Atypical hemolytic uremic syndrome (aHUS) involves dysregulation of the complement system, but whether this also occurs in thrombotic thrombocytopenic purpura (TTP) remains unclear. Although these conditions are difficult to differentiate clinically, TTP can be distinguished by low (<10%) ADAMTS13 activity. The aim was to identify the differences in complement activation products between TTP and aHUS and investigate ADAMTS13 activity as a prognostic factor in aHUS.
Methods: We analyzed patients with thrombotic microangiopathy diagnosed as TTP (N=48) or aHUS (N=50), selected from a Korean registry (N=551). Complement activation products in the plasma samples collected from the patients prior to treatment and in 40 healthy controls were measured by ELISA.
Results: The levels of generalized (C3a), alternate (factor Bb), and terminal (C5a and C5b-9) markers were significantly higher (all P<0.01) in the patients than in the healthy controls. Only the factor Bb levels significantly differed (P=0.008) between the two disease groups. In aHUS patients, high normal ADAMTS13 activity (≥77%) was associated with improved treatment response (OR, 6.769; 95% CI, 1.605–28.542; P=0.005), remission (OR, 6.000; 95% CI, 1.693–21.262; P=0.004), exacerbation (OR, 0.242; 95% CI, 0.064–0.916; P=0.031), and disease-associated mortality rates (OR, 0.155; 95% CI, 0.029–0.813; P=0.017).
Conclusions: These data suggest that complement biomarkers, except factor Bb, are similarly activated in TTP and aHUS patients, and ADAMTS13 activity can predict the treatment response and outcome in aHUS patients.
Keywords: Thrombotic thrombocytopenic purpura, Atypical hemolytic uremic syndrome, Complement, High ADAMTS13 activity, Treatment outcomes


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