Publicação
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
| dc.contributor.author | Barbot, José | |
| dc.contributor.author | Costa, Elísio | |
| dc.contributor.author | Guerra, Marisol | |
| dc.contributor.author | Barreirinho, Maria Sameiro | |
| dc.contributor.author | Isvarlal, Pratima | |
| dc.contributor.author | Robles, Rodolfo | |
| dc.contributor.author | Gerritsen, Helena | |
| dc.contributor.author | Lammle, Bernhard | |
| dc.contributor.author | Furlan, Miha | |
| dc.date.accessioned | 2008-02-21T10:58:21Z | |
| dc.date.available | 2008-02-21T10:58:21Z | |
| dc.date.issued | 2001 | |
| dc.description.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. | en |
| dc.identifier.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 | en |
| dc.identifier.issn | 1365-2141 | |
| dc.identifier.uri | http://hdl.handle.net/10198/509 | |
| dc.language.iso | eng | en |
| dc.publisher | Blackwell | en |
| dc.relation.publisherversion | http://www.blackwell-synergy.com/doi/abs/10.1046/j.1365-2141.2001.02808.x | |
| dc.subject | VWF - cleaving protease | en |
| dc.subject | Deficiency | en |
| dc.subject | Fresh-frozen plasma | en |
| dc.subject | Chronic haemolytic anemia | en |
| dc.subject | Thrombotic thrombo-cytopenic purpura | en |
| dc.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 | en |
| dc.type | journal article | |
| dspace.entity.type | Publication | |
| rcaap.rights | openAccess | |
| rcaap.type | article | en |