Thrombotic Thrombocytopenic Purpura Evaluation and Management
| Autor: | Nuñez Zuno JA; Universidad Nacional Autónoma de México, Khaddour K; Rosalind Franklin University of Medicine |
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| Jazyk: | angličtina |
| Zdroj: | 2022 Jan. |
| Abstrakt: | In 1924, Moschcowitz described the fatal case of a 16-year-old-girl who coursed with fever, weakness, transient focal neurologic symptoms, severe thrombocytopenia, and microangiopathic hemolytic anemia. This was the first description of thrombotic thrombocytopenic purpura (TTP), a rare and life-threatening condition. The majority of cases are related to the severe deficiency of ADAMTS-13, the specific Von Willebrand Factor (vWF) cleave protease, most frequently acquired via autoantibodies. TTP is considered as a hematologic emergence because, even with treatment, the mortality rounds 10% to 20%. The differential diagnosis is challenging given their significant overlap in clinical presentation with numerous conditions. Long-term follow-up is necessary to evaluate the sequelae, to identify other autoimmune disorders, and because the tendency to relapse is the rule. (Copyright © 2022, StatPearls Publishing LLC.) |
| Databáze: | MEDLINE |
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