Abstract
ABSTRACT Thrombocytopenic thrombotic purpura (TTP) is a severe hemorrhagic syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia and microvascular occlusion, besides the associated symptoms that may or may not be present: fever, neurological and renal impairment. The pathophysiology involves the autoimmune or genetic deficiency of a metalloproteinases activity (ADAMTS-13), responsible for the von Willebrand Factor cleavage. The treatment is based on plasmapheresis; and in acute or recurrent cases, corticosteroids and immunosuppressants are associated. In this article, we will discuss a case report about this disease, initially treated in the Emergency Room and followed in the Intensive Care Unit of a public reference hospital in Sao Paulo city, Brazil. All clinical diagnostic criteria were completely filled, facilitating the therapeutic approach of the patient. The report evidences that rapid intervention when made early diagnosis evolves with a good prognosis, and this pathology must be present as a differential diagnosis in the medical routine.
Highlights
The report evidences that rapid intervention when made early diagnosis evolves with a good prognosis, and this pathology must be present as a differential diagnosis in the medical routine
A group of serious diseases known as microangiopathic hemolytic anemia, represented by hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP), is characterized by the triad: microangiopathic hemolytic anemia, microvascular occlusion and thrombocytopenia[1]; and due to the similarity of the clinical manifestations, in the past, they were considered one single disease[2]
The diagnostic criteria for previously defined microangiopathic hemolytic anemia were based on the presence of: 1) microangiopathic hemolytic anemia; 2) thrombocytopenia; and 3) organic disorders without signs of disseminated intravascular coagulation (DIC)[5]; these are detailed in the Table
Summary
A group of serious diseases known as microangiopathic hemolytic anemia, represented by hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP), is characterized by the triad: microangiopathic hemolytic anemia, microvascular occlusion and thrombocytopenia[1]; and due to the similarity of the clinical manifestations, in the past, they were considered one single disease[2]. The clinical suspicion in favor of TTP or HUS is due to the presence of neurological changes or acute kidney injury, respectively. Despite the great clinical and histological similarity between TTP and HUS, the diagnosis must be performed, since both present independent and differentiated treatment and evolution[4]. The diagnostic criteria for previously defined microangiopathic hemolytic anemia were based on the presence of: 1) microangiopathic hemolytic anemia; 2) thrombocytopenia; and 3) organic disorders without signs of DIC[5]; these are detailed in the Table.
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call