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

• Tipo: Artigo em Revista Científica Internacional
• Data: 2001

Autores

Barbot, J (Autor) Outra; Costa, E (Autor) FFUP; Guerra, M (Autor) Outra; Barreirinho, MS (Autor) Outra; Isvarlal, P (Autor) Outra; Robles, R (Autor) Outra; Gerritsen, HE (Autor) Outra; Lammle, B (Autor) Outra; Furlan, M (Autor) Outra

Revista

Título: British Journal of Haematology

Vol. 113

Páginas: 649-651

ISSN: 0007-1048

Editora: Wiley-Blackwell

Indexação

ISI Web of Knowledge - 88 Citações

Scopus - 104 Citações

Classificação Científica

FOS: Ciências médicas e da saúde > Medicina clínica

Outras Informações

ID Authenticus: P-000-V9D

DOI: 10.1046/j.1365-2141.2001.02808.x

Abstract (EN): 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 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.

Idioma: Inglês

Tipo (Avaliação Docente): Científica