Lombardy diagnostic and therapeutic network of thrombotic microangiopathy

• DOI: 10.1186/s13023-022-02400-y
• Publication Date: 2022-06-23T12:15:04Z
• Authors: 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; ; Flora Peyvandi; Andrea Artoni; Barbara Ferrari; Roberta Palla; Ilaria Mancini; Pasquale Agosti; Marta Spreafico; Giovanni Crovetti; Elisabetta Volpato; Silvano Rossini; Anna Falanga; Laura Russo; Luca Barcella; Salvatore Gattillo; Chiara Novelli; Enrico Capuzzo; Marco D’Agostino; Porcari Moreno; Inzoli Alessandro; Pagani Ambrogio
• Keywords: ADAMTS13; Thrombotic microangiopathy

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA) requiring urgent treatment. Standardization of its diagnosis and optimal management challenging. This study aimed to evaluate the role centralized, rapid testing ADAMTS13 in patients experiencing acute TMAs plasma-exchange (PEX) estimate incidence TTP large Italian Region.We perfomed cohort frame project "Set-up Lombardy network for treatment undergoing apheresis", including 11 transfusion centers Region. Consecutive referred from 2014 2016 with PEX were enrolled. Centralized activity was performed at Milan Hemophilia Thrombosis Center within 24 h.Forty-three TMA (44 events) enrolled, whom 35 (81%) had severe deficiency. Patients deficiency younger, mainly women, higher prevalence autoimmune disorders lower cancer. Clinical laboratory characteristics without largely overlapped, platelet count being only baseline marker that significantly differed between two patient groups (ADAMTS13 < 10% vs ≥ 10%: median difference -27 × 109/l, 95% CI - 37 3). initiated all patients, but soon discontinued cases In this group, mortality rate no episode exacerbations or relapses 6 months occured. The estimated average annual acquired events 1.17 [0.78-1.55] per million people.Severe distinguished overlapping clinical features different disease course. provides feasible model implemented region practical approach underlines importance an accurate differential therapeutic approach.