Protocolo Assistencial Purpura Trombocitopênica Trombótica

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare, severe, and potentially fatal thrombotic microangiopathy characterized by thrombocytopenia, microangiopathic hemolytic anemia, and widespread microthrombi formation, usually associated with severe deficiency of the ADAMTS13 enzyme. Given the high mortality rate in the absence of treatment and the need for immediate intervention, early recognition and standardized management are essential to reduce morbidity and mortality. This Standard Operating Procedure aims to compile updated scientific evidence and establish guidelines for the diagnosis, risk stratification, treatment, and monitoring of TTP at the Hospital de Clínicas of the Federal University of Uberlândia. The protocol addresses the pathophysiological aspects of the disease, clinical and laboratory criteria for diagnostic suspicion, with emphasis on the use of the PLASMIC and French predictive scores, as well as differential diagnoses. It also outlines recommended therapeutic strategies, including therapeutic plasma exchange, corticosteroid therapy, and adjunctive treatments, in addition to criteria for therapeutic modification, hospital discharge, transfer, and clinical-laboratory follow-up. The proposed standardization of care aims to optimize patient management, promote clinical safety, and improve outcomes in individuals affected by this medical emergency.