Please use this identifier to cite or link to this item: http://hdl.handle.net/10198/509
Title: Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease
Authors: Barbot, José
Costa, Elísio
Guerra, Marisol
Barreirinho, Maria Sameiro
Isvarlal, Pratima
Robles, Rodolfo
Gerritsen, Helena
Lammle, Bernhard
Furlan, Miha
Keywords: VWF - cleaving protease
Deficiency
Fresh-frozen plasma
Chronic haemolytic anemia
Thrombotic thrombo-cytopenic purpura
Issue Date: 2001
Publisher: Blackwell
Citation: Barbot, José; Costa, Elísio; Guerra, Marisol; Barreirinho, Maria Sameiro; Isvarlal, Pratima; Robles, Rodolfo; Gerritsen, Helena; Lammle, Bernhard; Furlan, Miha (2001) - Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease. British Journal of Hematology. ISSN 1365-2141. 113:3, p. 649-651
Abstract: 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.
URI: http://hdl.handle.net/10198/509
ISSN: 1365-2141
Publisher Version: http://www.blackwell-synergy.com/doi/abs/10.1046/j.1365-2141.2001.02808.x
Appears in Collections:DTDT - Artigos em Revistas Indexados ao ISI/Scopus

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