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Resistenza alla proteina C attivata

Le informazioni riportate non sono consigli medici e potrebbero non essere accurate. I contenuti hanno solo fine illustrativo e non sostituiscono il parere medico: leggi le avvertenze.
Resistenza alla proteina C attivata (APCR)
Malattia rara
Specialitàematologia
Classificazione e risorse esterne (EN)
ICD-9-CM289.81
OMIM188055
MeSHD020016
Modifica dati su Wikidata · Manuale

La resistenza alla proteina C attivata (o APCR, sigla del nome inglese Activated protein C resistance) è un disordine dell'emostasi caratterizzato da una debole risposta anticoagulante alla proteina C attivata (APC). Ciò causa un accresciuto rischio di eventi trombotici venosi, che possono dare problemi circolatori come l'embolia polmonare.[1]

Questo disordine della coagulazione può essere congenito o acquisito, la forma ereditaria si trasmette in modo autosomico dominante.[2]

Fisiopatologia

La proteina C attivata (con la proteina S come cofattore) degrada i fattori Va e VIIIa. La resistenza alla proteina C attivata è l'incapacità della proteina C di inattivare uno o entrambi i fattori attivati, V o VIII. Ciò risulta in una prolungata generazione di trombina che può condurre ad uno stato di ipercoagulativo.[3]

Condizioni associate

È stato riportato che il tromboembolismo venoso è associato a resistenza alla proteina C attivata in una percentuale di casi che potrebbe arrivare al 64%.[4]

Note

  1. ^ B. Dahlbäck, The discovery of activated protein C resistance, in Journal of Thrombosis and Haemostasis, vol. 1, n. 1, 2003, pp. 3–9, DOI:10.1046/j.1538-7836.2003.00016.x. URL consultato il 26 agosto 2012.
  2. ^ T Koster, JP Vandenbroucke, FR Rosendaal, H de Ronde, E Briët, RM Bertina, Venous thrombosis due to poor anticoagulant response to activated protein C: Leiden Thrombophilia Study, in The Lancet, vol. 342, n. 8886-8887, 1993, pp. 1503–1506, DOI:10.1016/S0140-6736(05)80081-9. URL consultato il 26 agosto 2012.
  3. ^ Gerry A.F. Nicolaes, Björn Dahlbäck, Congenital and Acquired Activated Protein C Resistance, in Seminars in Vascular Medicine, vol. 03, n. 1, 2003, pp. 033–046, DOI:10.1055/s-2003-38331. URL consultato il 26 agosto 2012.
  4. ^ DR. Sheppard, Activated protein C resistance: the most common risk factor for venous thromboembolism., in J Am Board Fam Pract, vol. 13, n. 2, pp. 111-5, PMID 10764192.
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