TY - JOUR
T1 - Diagnostic relevance of ADAMTS13 activity
T2 - Evaluation of 28 patients with thrombotic thrombocytopenic purpura - Hemolytic uremic syndrome clinical diagnosis
AU - Vučelić, Dragica
AU - Miković, Danijela
AU - Rajić, Zoran
AU - Savić, Nebojša
AU - Budišin, Živko
AU - Antonijević, Nebojša M.
AU - Obradović, Slobodan
AU - Jevtić, Dragana
AU - Palla, Roberta
AU - Valsecchi, Carla
AU - Peyvandi, Flora
PY - 2013
Y1 - 2013
N2 - 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 TTP 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 pro- tease 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.
AB - 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 TTP 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 pro- tease 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.
KW - ADAMTS13
KW - Diagnosis
KW - Hemolytic uremic syndrome
KW - Thrombotic thrombocytopenic purpura
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U2 - 10.2298/SARH1308466V
DO - 10.2298/SARH1308466V
M3 - Article
C2 - 24073552
AN - SCOPUS:84940312847
VL - 141
SP - 466
EP - 474
JO - Srpski Arhiv Za Celokupno Lekarstvo
JF - Srpski Arhiv Za Celokupno Lekarstvo
SN - 0370-8179
IS - 7-8
ER -