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

被引:1
作者
Vucelic, Dragica [1 ]
Mikovic, Danijela [2 ]
Rajic, Zoran [3 ]
Savic, Nebojsa [4 ]
Budisin, Zivko [5 ]
Antonijevic, Nebojsa M. [6 ,7 ]
Obradovic, Slobodan [8 ]
Jevtic, Dragana [7 ,9 ]
Palla, Roberta [10 ,11 ]
Valsecchi, Carla [10 ,11 ]
Peyvandi, Flora [10 ,11 ]
机构
[1] Clin Ctr Serbia, Clin Digest Surg, Belgrade 11000, Serbia
[2] Blood Transfus Inst Serbia, Belgrade, Serbia
[3] Clin Ctr Serbia, Clin Hematol, Belgrade 11000, Serbia
[4] Clin Ctr Serbia, Clin Vasc & Endovasc Surg, Belgrade 11000, Serbia
[5] Univ Childrens Hosp, Belgrade, Serbia
[6] Clin Ctr Serbia, Clin Cardiol, Belgrade 11000, Serbia
[7] Univ Belgrade, Sch Med, Belgrade, Serbia
[8] Mil Med Acad, Clin Emergency Internal Med, Belgrade 11002, Serbia
[9] Mother & Child Hlth Care Inst Serbia Dr Vukan Cup, Belgrade, Serbia
[10] Univ Studi Milano, Fdn IRCCS Ca Granda Osped Maggiore Policli, A Bianchi Bonomi Haemophilia & Thrombosis Ctr, UOS Dipartimentale Diagnosi & Terapia Coagulopati, Milan, Italy
[11] Luigi Villa Fdn, Milan, Italy
关键词
thrombotic thrombocytopenic purpura; hemolytic uremic syndrome; ADAMTS13; diagnosis; VON-WILLEBRAND-FACTOR; FACTOR-CLEAVING PROTEASE; ANTIBODIES; OUTCOMES; PLASMA;
D O I
10.2298/SARH1308466V
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:466 / 474
页数:9
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