
The thrombotic microangiopathic diseases, which include acquired and congenital TTP and HUS, are most frequently acute disease entities. Untreated, these diseases are associated with a lethal course in many cases. Deficiency of the von Willebrand cleaving enzyme, ADAMTS13, is a decisive pathophysiological defect in most cases of thrombotic microangiopathic diseases. Early recognition and a secure distinction from other conditions that resemble TMA diseases is essential in order to institute adequate treatment and to obtain a favourable outcome in terms of both survival and development of serious sequelae.
Diagnosis, Differential, Early Diagnosis, Purpura, Thrombocytopenic, Purpura, Thrombotic Thrombocytopenic, Thrombotic Microangiopathies, Hemolytic-Uremic Syndrome, Humans, Prognosis
Diagnosis, Differential, Early Diagnosis, Purpura, Thrombocytopenic, Purpura, Thrombotic Thrombocytopenic, Thrombotic Microangiopathies, Hemolytic-Uremic Syndrome, Humans, Prognosis
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