Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients

• Published in: Journal of thrombosis and thrombolysis Vol. 42; no. 4; pp. 586 - 592
• Main Authors: Berti de Marinis, Giulia, Novello, Stefano, Ferrari, Silvia, Barzon, Isabella, Cortella, Irene, Businaro, Maria Antonietta, Fabris, Fabrizio, Lombardi, Anna Maria
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.11.2016; Springer Nature B.V
• Subjects: Acute Disease; ADAMTS13 Protein - blood; Adult; Aged; Cardiology; Cranial Nerve Diseases - blood; Cranial Nerve Diseases - etiology; Epilepsy, Partial, Motor - blood; Epilepsy, Partial, Motor - etiology; Female; Hematology; Humans; Kidney Diseases - blood; Male; Mean Platelet Volume; Medicine; Medicine & Public Health; Middle Aged; Neoplasms - blood; Thrombotic Microangiopathies - blood; Thrombotic Microangiopathies - complications; TTP; Thrombotic microangiopathy; ADAMTS13 activity; Epilepsy
• ISSN: 0929-5305; 1573-742X
• DOI: 10.1007/s11239-016-1395-7

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.