Literature DB >> 6812613

Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrand's disease.

E G Tuddenham, R S Lane, F Rotblat, A J Johnson, T J Snape, S Middleton, P B Kernoff.   

Abstract

Factor VIII was purified from cryoprecipitate by ion exchange chromatography on solid phase polyelectrolyte E-5 (PE-E5). The product was highly purified (3.5 u VIII:C/mg protein) compared to conventional concentrate (0.3 u VIII:C/mg protein) with low fibrinogen, low isoagglutinin titre, and a ratio of factor VIII coagulant activity (VIII:C) to factor VII related antigen (VIIIR:Ag) of 16:1. Trial infusions of this material (PE VIII) were given to three patients with severe haemophilia A and one patient with homozygous von Willebrand's disease. These patients also each received separate infusions of intermediate purity concentrate (IPC) for comparison. There were no adverse effects. The mean half life of VIII:C after PE VIII infusion in the haemophiliacs was 10.9 h and after IPC was 12.1 h, a statistically insignificant difference. The survival of factor VIII coagulant antigen (VIII:CAg) was similar to that of VIII:C. In contrast, the half life of VIII:C and of VIII:CAg was very short after infusion of PE VIII in the patient wih von Willebrand's disease (2.4 h). IPC when infused in this patient produced a typical secondary rise of VIII:C. Two bleeding episodes in severe haemophiliacs were satisfactorily treated with PE VIII. PE-E5 deserves further study as a means of preparing clinical concentrates of factor VIII.

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Year:  1982        PMID: 6812613     DOI: 10.1111/j.1365-2141.1982.tb03888.x

Source DB:  PubMed          Journal:  Br J Haematol        ISSN: 0007-1048            Impact factor:   6.998


  31 in total

1.  The "normal" factor VIII concentration in plasma.

Authors:  Saulius Butenas; Behnaz Parhami-Seren; Anetta Undas; David N Fass; Kenneth G Mann
Journal:  Thromb Res       Date:  2010-05-07       Impact factor: 3.944

2.  Pharmacokinetics and hemostatic effect of different factor VIII/von Willebrand factor concentrates in von Willebrand's disease type III.

Authors:  S Lethagen; E Berntorp; I M Nilsson
Journal:  Ann Hematol       Date:  1992-12       Impact factor: 3.673

3.  The ADAMTS13 metalloprotease domain: roles of subsites in enzyme activity and specificity.

Authors:  Rens de Groot; David A Lane; James T B Crawley
Journal:  Blood       Date:  2010-07-20       Impact factor: 22.113

4.  A sequence variation scan of the coagulation factor VIII (FVIII) structural gene and associations with plasma FVIII activity levels.

Authors:  Kevin R Viel; Deepa K Machiah; Diane M Warren; Manana Khachidze; Alfonso Buil; Karl Fernstrom; Juan C Souto; Juan M Peralta; Todd Smith; John Blangero; Sandra Porter; Stephen T Warren; Jordi Fontcuberta; Jose M Soria; W Dana Flanders; Laura Almasy; Tom E Howard
Journal:  Blood       Date:  2007-01-05       Impact factor: 22.113

Review 5.  Insight into the structure, function, and biosynthesis of factor VIII through recombinant DNA technology.

Authors:  R J Kaufman
Journal:  Ann Hematol       Date:  1991-09       Impact factor: 3.673

6.  Influence of single nucleotide polymorphisms in factor VIII and von Willebrand factor genes on plasma factor VIII activity: the ARIC Study.

Authors:  Marco Campos; Ashley Buchanan; Fuli Yu; Maja Barbalic; Yang Xiao; Lloyd E Chambless; Kenneth K Wu; Aaron R Folsom; Eric Boerwinkle; Jing-fei Dong
Journal:  Blood       Date:  2012-01-04       Impact factor: 22.113

7.  Cloning and characterization of two cDNAs coding for human von Willebrand factor.

Authors:  J E Sadler; B B Shelton-Inloes; J M Sorace; J M Harlan; K Titani; E W Davie
Journal:  Proc Natl Acad Sci U S A       Date:  1985-10       Impact factor: 11.205

8.  The von Willebrand factor D'D3 assembly and structural principles for factor VIII binding and concatemer biogenesis.

Authors:  Xianchi Dong; Nina C Leksa; Ekta Seth Chhabra; Joseph W Arndt; Qi Lu; Kevin E Knockenhauer; Robert T Peters; Timothy A Springer
Journal:  Blood       Date:  2019-01-14       Impact factor: 22.113

9.  Function of von Willebrand factor after crossed bone marrow transplantation between normal and von Willebrand disease pigs: effect on arterial thrombosis in chimeras.

Authors:  T C Nichols; C M Samama; D A Bellinger; J Roussi; R L Reddick; M Bonneau; M S Read; O Bailliart; G G Koch; M Vaiman
Journal:  Proc Natl Acad Sci U S A       Date:  1995-03-28       Impact factor: 11.205

10.  The pro-polypeptide of von Willebrand factor is required for the formation of a functional factor VIII-binding site on mature von Willebrand factor.

Authors:  A Leyte; J Voorberg; H B Van Schijndel; B Duim; H Pannekoek; J A Van Mourik
Journal:  Biochem J       Date:  1991-02-15       Impact factor: 3.857

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