Literature DB >> 20877667

Need for Prophylactic Treatment in Adult Haemophilia A Patients.

Berthold Siegmund1, Heinrich Richter, Hartmut Pollmann.   

Abstract

BACKGROUND AND METHODS: A single centre study including 52 German patients aged ≥16 years with severe haemophilia A was performed to compare the amount of clotting factor and outcome between on-demand therapy (26 patients) and continuous prophylaxis (26 patients) over 1 year.
RESULTS: Prophylaxis reduced the number of bleeds significantly. Compared to on-demand treatment (20.5 ± 3.0 bleeds/year/patient), under prophylaxis 7.8 ± 1.3 bleeds/year/patient were observed. Joint bleeds were reduced from 12.2 ± 1.5 to 4.7 ± 1.0/year/ patient. In the on-demand group 38% of the patients suffered from more than 2 bleeds/month, whereas in the prophylaxis group no patient was found with more than 2 bleeds/month. Mean annual factor VIII (FVIII) consumption increased from 767 ± 110 IU/kg body weight under on-demand treatment to 2,841 ± 341 IU/kg body weight under continuous prophylaxis, displaying a nearly fourfold increase in FVIII consumption. Furthermore, prophylaxis implies a more than four-fold increase in treatment days which escalated from a mean weekly injection rate of 0.56 ± 0.08 FVIII injections/week when bleeds were treated on demand to 2.52 ± 0.30 FVIII injections/week during prophylaxis.
CONCLUSION: Even though the results reflect a benefit also for prophylactically treated patients regarding their bleeding frequency, one has to take into account a substantial increase of the costs for coagulation concentrates when all patients with severe haemophilia A switch to continuous prophylaxis.

Entities:  

Year:  2009        PMID: 20877667      PMCID: PMC2941834          DOI: 10.1159/000225965

Source DB:  PubMed          Journal:  Transfus Med Hemother        ISSN: 1660-3796            Impact factor:   3.747


  24 in total

1.  A survey of factor prophylaxis in boys with haemophilia followed in North American haemophilia treatment centres.

Authors:  V S Blanchette; M McCready; C Achonu; M Abdolell; G Rivard; M J Manco-Johnson
Journal:  Haemophilia       Date:  2003-05       Impact factor: 4.287

2.  Sucrose formulated recombinant human antihemophilic factor VIII is safe and efficacious for treatment of hemophilia A in home therapy--International Kogenate-FS Study Group.

Authors:  T C Abshire; H H Brackmann; I Scharrer; K Hoots; C Gazengel; J S Powell; E Gorina; E Kellermann; E Vosburgh
Journal:  Thromb Haemost       Date:  2000-06       Impact factor: 5.249

3.  Episodic versus prophylactic infusions for hemophilia A: a cost-effectiveness analysis.

Authors:  P S Smith; S M Teutsch; P A Shaffer; H Rolka; B Evatt
Journal:  J Pediatr       Date:  1996-09       Impact factor: 4.406

Review 4.  Haemophilias A and B.

Authors:  Paula H B Bolton-Maggs; K John Pasi
Journal:  Lancet       Date:  2003-05-24       Impact factor: 79.321

5.  Long-term therapy and on-demand treatment of children and adolescents with severe haemophilia A: 12 years of experience.

Authors:  H H Brackmann; H J Eickhoff; J Oldenburg; U Hammerstein
Journal:  Haemostasis       Date:  1992

Review 6.  Hemophilia A--from basic science to clinical practice.

Authors:  Jens Klinge; Natalya M Ananyeva; Charlotte A E Hauser; Evgueni L Saenko
Journal:  Semin Thromb Hemost       Date:  2002-06       Impact factor: 4.180

7.  A longitudinal study of orthopaedic outcomes for severe factor-VIII-deficient haemophiliacs. The Orthopaedic Outcome Study Group.

Authors:  L M Aledort; R H Haschmeyer; H Pettersson
Journal:  J Intern Med       Date:  1994-10       Impact factor: 8.989

8.  Long-term major joint outcomes in young adults with haemophilia: interim data from the HGDS.

Authors:  Y Su; W-Y Wong; A Lail; S M Donfield; S Konzal; E Gomperts
Journal:  Haemophilia       Date:  2007-07       Impact factor: 4.287

9.  Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.

Authors:  Marilyn J Manco-Johnson; Thomas C Abshire; Amy D Shapiro; Brenda Riske; Michele R Hacker; Ray Kilcoyne; J David Ingram; Michael L Manco-Johnson; Sharon Funk; Linda Jacobson; Leonard A Valentino; W Keith Hoots; George R Buchanan; Donna DiMichele; Michael Recht; Deborah Brown; Cindy Leissinger; Shirley Bleak; Alan Cohen; Prasad Mathew; Alison Matsunaga; Desiree Medeiros; Diane Nugent; Gregory A Thomas; Alexis A Thompson; Kevin McRedmond; J Michael Soucie; Harlan Austin; Bruce L Evatt
Journal:  N Engl J Med       Date:  2007-08-09       Impact factor: 91.245

Review 10.  Quality of life assessment in haemophilia.

Authors:  Monika Bullinger; Sylvia von Mackensen
Journal:  Haemophilia       Date:  2004-03       Impact factor: 4.287

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  1 in total

1.  Controlled, cross-sectional MRI evaluation of joint status in severe haemophilia A patients treated with prophylaxis vs. on demand.

Authors:  J Oldenburg; R Zimmermann; O Katsarou; G Theodossiades; E Zanon; B Niemann; E Kellermann; B Lundin
Journal:  Haemophilia       Date:  2014-12-02       Impact factor: 4.263

  1 in total

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