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AL-Kadhimi, Munaf; Wah, Fernanda; AL-Dawoodi, Tamarah; Peguero, Julio; T Campos, Luis
Case Reports International, 08/2016, Letnik: 5, Številka: 2Journal Article
Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening syndrome typically characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA). Both genetic and idiopathic TTP are caused by deficient activity of the serine protease ADAMTS-13, allowing its normal substrate, von Willebrand factor, to polymerize and bind platelets under high sheer conditions. Autoimmune TTP is typically treated by plasmapheresis and corticosteroids. Here, we describe a 69-year-old patient presenting with thrombopenia comorbid with "warm" autoimmune hemolytic anemia (WAIHA). Treatment with steroids and plasma exchange initiated at the beginning and patient acquired a clinical as well as biological response. Thrombotic thrombocytopenic purpura (TTP) is a life-threatening condition typically (but not always) associated with autoimmune or genetic inactivation of the protease ADAMTS-13, for which consistently effective therapeutic interventions remain elusive.
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Leto | Faktor vpliva | Izdaja | Kategorija | Razvrstitev | ||||
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JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
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in: SICRIS
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