Jurnal Internasional Pengobatan defisiensi faktor langka selain hemofilia

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Jurnal Internasional Pengobatan defisiensi faktor langka selain hemofilia

Abstract

Lack of fibrinogen, prothrombin, factor V (FV), FVII, FVIII, FIX, FX, FXII, and so-called rare coagulation disorders (RCD), can lead to coagulopathy that leads to spontaneous or posttrauma. and postoperative bleeding. RCD is characterized by a variety of symptoms, from mild to severe, which can vary significantly from 1 disease to another and from 1 patient to another. The most typical symptom of all RCDs is mucosal bleeding and bleeding during invasive procedures, while other life-threatening symptoms such as central nervous system bleeding and hemarthrocess are mostly only present in some deficiencies (afibrinogenemia, FX, and FXIII). In contrast to hemophilia A and B and von Willebrand's disease, RCDs are much smaller, ranging from 1 case at 500,000 to 1 in 2 million in the general population. Their clinical heterogeneity associated with a low number of patients has caused delays in the development of appropriate therapy. Indeed, similar heterogeneity can also be found in available care products, ranging from specific recombinant proteins to treat patients who lack FVII- and FXIII to the absence of special products to treat patients with FII or FV deficiency, for whom concentrated or frozen plasma complex prothrombin fresh, to date, the only choice. Recent developments from the new hemostatic approach to hemophilia, such as the use of nonsubstitutive therapies such as RNA disorders, anti-tissue factor pathway inhibitors, and gene therapy aimed at improving patient quality of life may also have an important role in the treatment of future RCD patients. .

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