I Mancini, P Agosti, M Boscarino, B Ferrari, A Artoni, R Palla, M Spreafico, G Crovetti, E Volpato, S Rossini, C Novelli, S Gattillo, L Barcella, M Salmoiraghi, A Falanga, F Peyvandi
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA) requiring urgent treatment. Standardization of its diagnosis and optimal management is challenging. This study aimed to evaluate the role of centralized, rapid testing of ADAMTS13 in patients experiencing acute TMAs requiring plasma-exchange (PEX) and to estimate the incidence of TTP in a large Italian Region. METHODS: We perfomed a cohort study in the frame of the project "Set-up of a Lombardy network for the study and treatment of patients undergoing apheresis", including 11 transfusion centers in the Region...
June 23, 2022: Orphanet Journal of Rare Diseases