Ten years of prophylactic treatment with fresh-frozen plasma in a child with ch...

• Author(s): Barbot, José ; Costa, Elísio ; Guerra, Marisol ; Barreirinho, Maria Sameiro ; Isvarlal, Pratima ; Robles, Rodolfo ; Gerritsen, Helena ; Lammle, Bernhard ; Furlan, Miha
• Date: 2001
• Persistent ID: http://hdl.handle.net/10198/509
• Origin: Biblioteca Digital do IPB
• Subject(s): VWF - cleaving protease; Deficiency; Fresh-frozen plasma; Chronic haemolytic anemia; Thrombotic thrombo-cytopenic purpura
• Document Type: Article
• Language: English

Description

We report the results of 10 years of prophylactic fresh-frozen plasma (FFP) infusion therapy in a 14-year-old girl with chronic relapsing thrombotic thrombocytopenic purpura (TTP), in whom a severe congenital von Willebrand factor (VWF)-cleaving protease deficiency has been documented. Severe haemolytic crises triggered by infections were prevented and her present renal and neurological functions have been preserved. Sequential measurements of protease activity and platelet count after FFP infusion led us to conclude tentatively that 5% may be sufficient to degrade very large and adhesive VWF multimers.