title: Correlation between ADAMTS13 activity and neurological impairment in acute thrombotic microangiopathy patients.
abstract: 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.
authors.affiliation: Emergency Department, University Hospital of Padova, Via Giustiniani n.2, 35128, Padua, Italy. giulia.bdm@gmail.com.
authors.forename: Giulia
authors.initials: G
authors.lastname: Berti de Marinis
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Novello
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Ferrari
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Isabella
authors.initials: I
authors.lastname: Barzon
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Irene
authors.initials: I
authors.lastname: Cortella
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Maria Antonietta
authors.initials: MA
authors.lastname: Businaro
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Fabris
authors.affiliation: Department of Medicine, Internal Medicine, Padova University, Padua, Italy.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Lombardi
mesh_terms: D000071120:ADAMTS13 Protein / Q000097:blood*; D000208:Acute Disease; D000328:Adult; D000368:Aged; D003389:Cranial Nerve Diseases / Q000097:blood* / Q000209:etiology; D020938:Epilepsy, Partial, Motor / Q000097:blood* / Q000209:etiology; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000097:blood; D008297:Male; D063847:Mean Platelet Volume; D008875:Middle Aged; D009369:Neoplasms / Q000097:blood; D057049:Thrombotic Microangiopathies / Q000097:blood* / Q000150:complications
chemical_list: D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13 activity; Epilepsy; TTP; Thrombotic microangiopathy