Diagnostic Relevance of ADAMTS13 Activity: Evaluation of 28 Patients with Thrombotic Thrombocytopenic Purpura - Hemolytic Uremic Syndrome Clinical Diagnosis

作者：Vucelic Dragica^*; Mikovic Danijela; Rajic Zoran; Savic Nebojsa; Budisin Zivko; Antonijevic Nebojsa M; Obradovic Slobodan; Jevtic Dragana; Palla Roberta; Valsecchi Carla; Peyvandi Flora

来源：Srpski Arhiv za Celokupno Lekarstvo, 2013, 141(7-8): 466-474.

DOI：10.2298/SARH1308466V

• thrombotic thrombocytopenic purpura
• hemolytic uremic syndrome
• ADAMTS13
• diagnosis

Introduction The significance of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motif-13) activity for diagnosis and therapy of thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) is still a controversial issue.
Objective The aim of this report was to analyze the value of ADAMTS13 measurements in the diagnosis of TTP and HUS.
Methods At presentation, we analyzed patients with idiopathic TTP (n=18), secondary TTP (n=4), diarrhea positive HUS (n=3) and diarrhea negative HUS (n=3) treated in Belgrade, Serbia from 2004 to 2010. ADAMTS13 activity from acute phase samples was measured using the residual collagen binding activity assay at the Haemophilia and Thrombosis Centre, Milan, Italy.
Results There was a significant correlation between reduced ADAMTS13 activity and idiopathic UP diagnosis (p=0.000) as well as between lower ADAMTS13 activities and higher reticulocytes (p=0.017) and lactate dehydrogenase levels (p=0.027). Significant correlation was also found between higher protease activity and diagnosis of HUS (p=0.000). There was a statistically significant correlation between higher ADAMTS13 activities and higher platelets count (p=0.002), blood urea nitrogen (p=0.000), and creatinine level (p=0.000).
Conclusion Severe ADAMTS13 deficiency points at the diagnosis of idiopathic TTP and it is present in the secondary TTP but not in HUS.

• 出版日期2013-8
• 单位中国人民解放军军事医学科学院