Consensus opinion for the selection and use of therapeutic products for the treatment of haemophilia in Spain

Batlle, J.; Villar, Ana; Liras, Antonio; Alonso, C.; Altisent, Carmen; Brito, Dilia; Moreno, Mercedes Martínez; Lucia, F.; Sedano, Carmen; Prieto, Manuel Ángel Rodríguez; Calvente, N; Aznar, José A.; Jiménez, V.; Soriano, Vincent; Martorell, Javier R; Iruin, Gemma; Bergua, Juan; Aguilar, Carlos

doi:10.1097/mbc.0b013e328300c814

Cited by 16 publications

(14 citation statements)

References 38 publications

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“…However, until these studies are concluded, we think that it is unjustified to modify the current evidence-based guidelines of most hemophilia organizations which recommend that, where available, rFVIII products are the treatment of choice of hemophilia A patients and that FVIII inhibitors must be tolerized with the same agent used at the time of inhibitor detection [71][72][73][74][75].…”

Section: Discussionmentioning

confidence: 99%

Immune tolerance induction for patients with severe hemophilia A: a critical literature review

Franchini

Lippi²

2011

J Thromb Thrombolysis

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The development of inhibitors that neutralize the function of clotting factor VIII (FVIII) is currently the most challenging complication associated with the treatment of hemophilia A as it increases the disease-related morbidity and mortality. Immune tolerance induction (ITI) is the only documented strategy to eradicate persistent inhibitors in severe hemophilia A patients. Several studies have been conducted so far to identify patient- and treatment-related factors associated with greater ITI success. The currently available literature on ITI in hemophilia A will be critically reviewed in this article. In particular, we will focus on the role of the type of FVIII product on ITI outcome by analyzing the clinical and experimental data.

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Section: Discussionmentioning

confidence: 99%

Immune tolerance induction for patients with severe hemophilia A: a critical literature review

Franchini

Lippi²

2011

J Thromb Thrombolysis

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“…In 1994, the Scientific Committee of the RVEF issued several guidelines to provide physicians treating hemophilia patients throughout the country with clear consensus guidelines for the appropriate choice of factor replacement products. 1 A recent Spanish epidemiological study has estimated that 2518 individuals have hemophilia A and 387 have hemophilia B. 2,3 The estimated prevalence of hemophilia, therefore, would be $1 in 7857 males.…”

Section: Care For Patients With Bleeding Disorders In Spainmentioning

confidence: 99%

“…1,17 The antifibrinolytic drugs tranexamic acid, registered under the name Amchafibrín, and epsilon-aminocaproic acid, registered under the name Caproamín Fides, are both from Rottapharm Madaus, Spain. DDAVP, registered as Minirín for intravenous administration and Octostím as a spray for nasal pulverization (Ferring Laboratories), is the treatment of choice in VWD patients responsive to DDAVP, as determined by a test infusion.…”

Section: Treatment Strategy For Von Willebrand Disease In Spainmentioning

confidence: 99%

Diagnosis and Management ofvon Willebrand Disease in Spain

Batlle

Pérez‐Rodríguez

Pinto

et al. 2011

Semin Thromb Hemost

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The correct diagnosis and classification of Von Willebrand disease (VWD) is important for therapy and genetic counseling but is made difficult due to the variability of its clinical expression and limitations of laboratory methods. A national registry of VWD patients has been initiated in Spain. The results of a concise survey on the diagnosis of VWD show the frequency of VWD is fivefold greater in Spain than that expected from epidemiological studies in other European countries; this may result from overdiagnosis and/or a higher prevalence of VWD. These results clearly reinforce the need for the Spanish VWD registry. A consensus guideline for optimal treatment of VWD is being elaborated in Spain. Desmopressin (DDAVP) is the choice of treatment in responsive VWD patients. Von Willebrand factor concentrates (VWF/factor VIII) are used in individuals nonresponsive to DDAVP, when DDAVP is contraindicated, or in VWD types 2B and 3.

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“…U pacjentów leczonych czynnikami osoczopochodnymi powinno się stopniowo przechodzić na czynniki rekombinowane [44][45][46]. Zwłaszcza dzieci powinny mieć dostęp do preparatów rekombinowanych.…”

Section: Opinie Ekspertówunclassified

Jak wybrać koncentrat czynnika krzepnięcia dla dziecka chorego na hemofilię?

Klukowska¹

2014

Acta Haematologica Polonica

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Leczenie chorych na hemofilię opiera się na substytucji niedoborowego czynnika krzepnięcia krwi. W ciężkiej postaci hemofilii, aby zapobiec krwawieniom do stawów oraz rozwojowi zmian degeneracyjnych, metodą z wyboru w leczeniu, zwłaszcza dzieci, jest profilaktyczne podawanie koncentratu czynnika krzepnięcia [1][2][3]. Wcześnie wprowadzona profilaktyka -przed pojawieniem się zmian a c t a h a e m a t o l o g i c a p o l o n i c a 4 5 ( 2 0 1 4 ) 4 9 -5 3 i n f o r m a c j e o a r t y k u l e Historia artykułu: Słowa kluczowe: hemofilia rekombinowane koncentraty czynnika VIII i IX osoczopochodne koncentraty czynnika VIII i IX dzieci Keywords: Haemophilia Recombinant factor VIII and IX concentrates Plasma-derived factor VIII and IX concentrates Children a b s t r a c t The introduction of the coagulation factor VIII and IX concentrates has changed the treatment of haemophilia patients dramatically. The concentrates were produced from pooled human plasma in the beginning. During the 1990s of the 20th century, recombinant factor VIII was accessed, then factor IX was introduced. The modern methods of elimination and inactivation of viruses led to the development of safe plasma-derived products. However, minimal risk of viral transmission is still greater by plasma-derived than recombinant concentrates. The efficacy of both products is very good in treatment of bleeds as well as in surgery and prophylaxis. The pharmacokinetics of plasma-derived and recombinant factor VIII is similar. Nevertheless, the recovery of recombinant factor IX is lower than plasma-derived, therefore the doses of recombinant factor IX should be 30% higher. The half-life of both factor IX products is almost the same. In developed countries recombinant coagulation factor concentrates are recommended treatment of choice for patients with haemophilia and almost all children are being treated with them.

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Consensus opinion for the selection and use of therapeutic products for the treatment of haemophilia in Spain

Cited by 16 publications

References 38 publications

Immune tolerance induction for patients with severe hemophilia A: a critical literature review

Immune tolerance induction for patients with severe hemophilia A: a critical literature review

Diagnosis and Management ofvon Willebrand Disease in Spain

Jak wybrać koncentrat czynnika krzepnięcia dla dziecka chorego na hemofilię?

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