{"id":10324,"date":"2020-04-23T04:03:03","date_gmt":"2020-04-23T02:03:03","guid":{"rendered":"https:\/\/www.synevo.ro\/product\/factor-ix-inhibitori\/"},"modified":"2025-07-11T14:37:54","modified_gmt":"2025-07-11T11:37:54","slug":"factor-ix-inhibitori","status":"publish","type":"product","link":"https:\/\/old.synevo.md\/ru\/shop\/factor-ix-inhibitori\/","title":{"rendered":"\u0418\u043d\u0433\u0438\u0431\u0438\u0442\u043e\u0440 \u0444\u0430\u043a\u0442\u043e\u0440\u0430 IX"},"content":{"rendered":"

Informa\u021bii generale Factor IX-inhibitori<\/strong><\/em><\/p>\n

Hemofilia este o afec\u021biune mo\u0219tenit\u0103, produs\u0103 de o deficien\u021b\u0103 a unuia dintre factorii de coagulare din s\u00e2nge. Poate fi din cauza unui defect al genei pentru factorul de coagulare. Genele pentru factorii VIII \u0219i IX sunt prezente la nivelul cromozomului X. Hemofilia B este o boal\u0103 mo\u0219tenit\u0103 printr-un model recesiv legat de X, afect\u00e2nd astfel \u00een mare parte b\u0103rba\u021bii, unde b\u0103rba\u021bii afecta\u021bi vor avea 100% fiice purt\u0103toare, \u00een timp ce niciunul dintre fiii lor nu vor fi afecta\u021bi. Femeile purt\u0103toare vor avea o \u0219ans\u0103 de 50% de a na\u0219te fii afecta\u021bi \u0219i o \u0219ans\u0103 de 50% de a na\u0219te fiice purt\u0103toare1.<\/p>\n

Hemofilia B dob\u00e2ndit\u0103 este o afec\u021biune autoimun\u0103 rar\u0103, care apare la pacien\u021bii f\u0103r\u0103 antecedente familiale de tulbur\u0103ri de s\u00e2ngerare sau hemoragie, datorit\u0103 ac\u021biunii unor autoanticorpi care se dezvolt\u0103 \u00eempotriva factorilor de coagulare. Pacien\u021bii cu hemofilie dob\u00e2ndit\u0103 au, de obicei, boli autoimune preexistente, afec\u021biuni maligne sau infec\u021bii precum HIV, hepatita B sau C1,2.<\/p>\n

Inhibitorii factorului IX apar la pacien\u021bii cu hemofilie B sever\u0103 dup\u0103 transfuzia cu factor IX. Pacien\u021bii ce prezint\u0103 inhibitori ai factorului IX pot dezvolta, de asemenea, reac\u021bii anafilactice ca r\u0103spuns la perfuziile cu factor IX. Apari\u021bia unor \u00eenhibitori ai factorului IX dob\u00e2ndi\u021bi la persoanele anterior s\u0103n\u0103toase, este extrem de rar\u01031,3.<\/p>\n

Inhibitorii factorilor de coagulare (numi\u021bi \u0219i anticoagulante circulante sau inactivatori) sunt anticorpi produ\u0219i endogen care interfer\u0103 cu procesul de coagulare. Acestia inhibitori nu sunt prezen\u021bi \u00een mod normal \u00een plasm\u0103, \u00eens\u0103 atunci c\u00e2nd sunt prezen\u021bi, acestia sunt m\u0103surabili \u0219i exprima\u021bi \u00een unit\u0103\u021bi de titru Bethesda (BU). Titrurile de 5 BU sunt clasificate ca r\u0103spunsuri sc\u0103zute iar titrurile cu valori de 10 BU sunt considerate crescute. Inhibitorii factorului IX apar la 3% p\u00e2n\u0103 la 5% dintre pacien\u021bii cu hemofilie B (Christmas disease) ca r\u0103spuns impotriva factorului IX, concentratul de complex de protrombin\u0103 sau transfuziei cu plasm\u0103. Inhibitori ai unor al\u021bi factori de coagulare sunt extrem de rari, deoarece \u0219i deficien\u021bele acestor factori sunt rare5,6.<\/p>\n

Inhibitorii complic\u0103 episoadele de s\u00e2ngerare deoarece scad sensibilitatea la tratament. Prezen\u021ba inhibitorilor trebuie suspectat\u0103 dac\u0103 s\u00e2ngerarea nu se opre\u0219te dup\u0103 \u00eenlocuirea factorilor de coagulare, la un pacient care a r\u0103spuns anterior la administrarea acestora. De asemenea, pot ap\u0103rea reac\u021bii anafilactice la ac\u021biunea inhibitorilor de factor IX1,7.<\/p>\n

Recomand\u0103ri pentru determinarea Factor IX \u2013 inhibitori<\/strong><\/p>\n

Detectarea \u0219i masurarea inhibitorului de coagulare la factorul IX, la pacien\u021bii cu hemofilie B<\/strong><\/em>8<\/p>\n

Preg\u0103tire pacient8<\/strong> Este recomandat ca proba sa fie recoltat\u0103 a jeun, \u00eenainte de tratamentul cu warfarina sau heparin\u0103.
\nSpecimen recoltat<\/strong> \u2013 s\u00e2nge venos8<\/p>\n

Recipient de recoltare<\/strong> \u2013 vacutainer cu citrat de sodiu 3.2%8<\/p>\n

Prelucrare necesar\u0103 dup\u0103 recoltare <\/strong>Proba de s\u00e2nge se recomand\u0103 a fi centrifugat\u0103, dup\u0103 care se alicoteaz\u0103 plasma \u00eentr-un tub steril. Plasma alicotat\u0103 se centrifugheaz\u0103 \u0219i se alicoteaz\u0103 \u00een trei tuburi separate sterile. Plasma se depoziteaz\u0103 (nu mai mult de 4 ore de la recoltare) la temperaturi cuprinse \u00eentre -20 \u0219i -40oC8.<\/p>\n

Volum prob\u0103<\/strong> \u2013 3 mL (c\u00e2te 1 mL \u00een fiecare tub)8<\/p>\n

Cauze de respingere a probei<\/strong>8<\/p>\n

Specimen hemolizat
\nSpecimen icteric
\nSpecimen lipemic
\nStabilitate prob\u0103 \u2013 congelat\u0103, timp de 14 zile8<\/p>\n

Metod\u0103<\/strong> \u2013 optical clot based8<\/p>\n

Valori de referin\u021b\u0103<\/strong> \u2013 pentru adul\u021bi: 65-140%8<\/p>\n

Limite \u0219i interferen\u021be<\/strong><\/p>\n

Un anticoagulant puternic poate determina un rezultat fals pozitiv pentru un anumit inhibitor al factorului (de exemplu, factorul VIII sau IX)8.<\/p>\n

\u0411\u0438\u0431\u043b\u0438\u043e\u0433\u0440\u0430\u0444\u0438\u044f<\/p>\n

    \n
  1. Alshaikhli A, Rokkam VR. Hemophilia B. [Updated 2022 Feb 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK560792\/ (Accesat la data de 15.04.2022)<\/li>\n
  2. Chitlur, M., Warrier, I., Rajpurkar, M., & Lusher, J. M. (2009). Inhibitors in factor IX deficiency a report of the ISTH-SSC international FIX inhibitor registry (1997-2006). Haemophilia : the official journal of the World Federation of Hemophilia, 15(5), 1027\u20131031. https:\/\/doi.org\/10.1111\/j.1365-2516.2009.02039.x<\/li>\n
  3. Mansouritorghabeh, H., & Mohades, S. T. (2020). Is the Detection of Factor IX Inhibitors in Hemophilia B Orphan than Factor VIII Inhibitors in Hemophilia A? A Concise, Systematic Review. Cardiovascular & hematological disorders drug targets, 20(3), 185\u2013190. https:\/\/doi.org\/10.2174\/1871529X20666200701104143<\/li>\n
  4. Hay, C. R., Brown, S., Collins, P. W., Keeling, D. M., & Liesner, R. (2006). The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation. British journal of haematology, 133(6), 591\u2013605. https:\/\/doi.org\/10.1111\/j.1365-2141.2006.06087.x<\/li>\n
  5. Verghese, P., Darrow, S., Kurth, M. H., Reed, R. C., Kim, Y., & Kearney, S. (2013). Successful management of factor IX inhibitor-associated nephrotic syndrome in a hemophilia B patient. Pediatric nephrology (Berlin, Germany), 28(5), 823\u2013826. https:\/\/doi.org\/10.1007\/s00467-012-2397-0<\/li>\n
  6. Peyvandi, F., Garagiola, I., & Young, G. (2016). The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet (London, England), 388(10040), 187\u2013197. https:\/\/doi.org\/10.1016\/S0140-6736(15)01123-X<\/li>\n
  7. Fassel, H., & McGuinn, C. (2021). Haemophilia: factoring in new therapies. British journal of haematology, 194(5), 835\u2013850. https:\/\/doi.org\/10.1111\/bjh.17580<\/li>\n
  8. Mayo Clinic. Mayo Medical Laboratories. Test Catalog. [on-line]: https:\/\/www.mayocliniclabs.com\/test-catalog\/overview\/607425#Overview (Accesat la data de 15.04.2022)<\/li>\n<\/ol>","protected":false},"excerpt":{"rendered":"

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