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

• Type: Article in International Scientific Journal
• Date: 2001

Authors

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

Journal

Title: British Journal of Haematology

Vol. 113

Pages: 649-651

ISSN: 0007-1048

Publisher: Wiley-Blackwell

Indexing

ISI Web of Knowledge - 88 Citations

Scopus - 104 Citations

Scientific classification

FOS: Medical and Health sciences > Clinical medicine

Other information

Authenticus ID: 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.

Language: English

Type (Professor's evaluation): Scientific