Lombardy diagnostic and therapeutic network of thrombotic microangiopathy.

I ManciniP AgostiM BoscarinoB FerrariA ArtoniR PallaM SpreaficoG CrovettiE VolpatoS RossiniC NovelliS GattilloL BarcellaM SalmoiraghiA FalangaFlora Peyvandinull null

Published in: Orphanet journal of rare diseases (2022)

Severe ADAMTS13 deficiency distinguished two groups of patients with largely overlapping clinical features but different treatment and disease course. This study provides a feasible model implemented in a large Italian region for the practical clinical approach to TMAs and underlines the importance of urgent ADAMTS13 activity testing for an accurate differential diagnosis and therapeutic approach.

Keyphrases

early onset
high resolution
replacement therapy
mass spectrometry
drug induced