Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients
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- 作者：Giulia Berti de Marinis ; Stefano Novello…
- 关键词：TTP ; ADAMTS13 activity ; Epilepsy ; Thrombotic microangiopathy
- 刊名：Journal of Thrombosis and Thrombolysis
- 出版年：2016
- 出版时间：November 2016
- 年：2016
- 卷：42
- 期：4
- 页码：586-592
- 全文大小：509 KB
- 刊物类别：Medicine
- 刊物主题：Medicine & Public Health
Cardiology
Hematology - 出版者：Springer Netherlands
- ISSN：1573-742X
- 卷排序：42
文摘
Differential diagnosis between thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMA) is usually difficult because of frequently overlapping clinical presentations. Severely depressed ADAMTS13 activity (<10 %) seems distinctive for TTP because of its pathogenetic role. However a long debate exists in the literature about its sensibility and specificity. Our aim was to search for clinical differences between TMA patients referred to our laboratory, comparing them for protease activity <10 versus ≥10 %. ADAMTS13 activity ≥10 % patients (n = 73) showed a higher prevalence of drug- (p = 0.005) and cancer-associated (p < 0.001) TMA. Mean platelet count and renal dysfunction prevalence was lower (p < 0.001), while neurological impairment was more frequent (p = 0.001) in the <10 % ADAMTS13 activity group (n = 109), confirming previous literature findings. When taken neurological manifestations singularly, epilepsy (p = 0.04), focal motor deficit (p < 0.001) and cranial nerve palsy (p = 0.007) were more frequent in the <10 % activity group. In our case series, a <10 % ADAMTS13 activity depicts a group of patients with clinical features similar to TTP patients. Focal motor impairment or epileptic manifestations could further address toward a TTP diagnosis. Studies about treatment efficacy and follow-up are advised to determine whether laboratory findings can guide therapeutic decisions.