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

Detalhes bibliográficos
Autor(a) principal: Barbot, José
Data de Publicação: 2001
Outros Autores: Costa, Elísio, Guerra, Marisol, Barreirinho, Maria Sameiro, Isvarlal, Pratima, Robles, Rodolfo, Gerritsen, Helena, Lammle, Bernhard, Furlan, Miha
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10198/509
Resumo: 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.
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spelling 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 proteaseVWF - cleaving proteaseDeficiencyFresh-frozen plasmaChronic haemolytic anemiaThrombotic thrombo-cytopenic purpuraWe 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.BlackwellBiblioteca Digital do IPBBarbot, JoséCosta, ElísioGuerra, MarisolBarreirinho, Maria SameiroIsvarlal, PratimaRobles, RodolfoGerritsen, HelenaLammle, BernhardFurlan, Miha2008-02-21T10:58:21Z20012001-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10198/509engBarbot, 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. 649-6511365-2141info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-11-21T10:03:28Zoai:bibliotecadigital.ipb.pt:10198/509Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T22:54:17.535348Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv 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
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
spellingShingle 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
Barbot, José
VWF - cleaving protease
Deficiency
Fresh-frozen plasma
Chronic haemolytic anemia
Thrombotic thrombo-cytopenic purpura
title_short 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_sort 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
author Barbot, José
author_facet Barbot, José
Costa, Elísio
Guerra, Marisol
Barreirinho, Maria Sameiro
Isvarlal, Pratima
Robles, Rodolfo
Gerritsen, Helena
Lammle, Bernhard
Furlan, Miha
author_role author
author2 Costa, Elísio
Guerra, Marisol
Barreirinho, Maria Sameiro
Isvarlal, Pratima
Robles, Rodolfo
Gerritsen, Helena
Lammle, Bernhard
Furlan, Miha
author2_role author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Biblioteca Digital do IPB
dc.contributor.author.fl_str_mv Barbot, José
Costa, Elísio
Guerra, Marisol
Barreirinho, Maria Sameiro
Isvarlal, Pratima
Robles, Rodolfo
Gerritsen, Helena
Lammle, Bernhard
Furlan, Miha
dc.subject.por.fl_str_mv VWF - cleaving protease
Deficiency
Fresh-frozen plasma
Chronic haemolytic anemia
Thrombotic thrombo-cytopenic purpura
topic VWF - cleaving protease
Deficiency
Fresh-frozen plasma
Chronic haemolytic anemia
Thrombotic thrombo-cytopenic purpura
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.
publishDate 2001
dc.date.none.fl_str_mv 2001
2001-01-01T00:00:00Z
2008-02-21T10:58:21Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10198/509
url http://hdl.handle.net/10198/509
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 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
1365-2141
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Blackwell
publisher.none.fl_str_mv Blackwell
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
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