Hereditary bleeding disorders include a group of diseases with abnormalities of coagulation. Prior to 1990, infection with hepatitis C virus (HCV) was mainly transmitted via pooled plasma products as a treatment for hereditary bleeding disorders. Anti-HCV positivity in these patients may be as high as >70% in some areas, while some of them have also been coinfected with human immunodeficiency v...

inhibitor development is a lifelong challenge for patients with bleeding disorders who received replacement therapy. most commonly, inhibitor formation was observed in hemophilia a patients but patients with rare bleeding disorders (rbd) especially patients with deficiency of factor xiii (fxiii) and factor v (fv) can develop an inhibitor against exogenous factors. several factors considered as ...

Excessive bleeding after dental procedures are one of the most frequent complications occurring in patients with hereditary bleeding disorders. In this retrospective study we collected data from 10 years of experience in the oral care of patients with congenital haemorrhagic disorders in three Italian Hemophilia Centers. Between 1993 and 2003, 247 patients with inherited bleeding disorders unde...

rare bleeding disorders (rbds) are a heterogeneous group of disorders including different types of coagulation factor deficiencies. the disorders are inherited in an autosomal recessive manner with different frequencies varying from 1:500000 to 1:2000000. patients affected with rbds are presented with a wide spectrum of clinical manifestations ranging from mild to life threatening bleeding diat...

The problem of impaired hemostasis remains relevant even today. Rare bleeding disorders that cause life-threatening in patient are often overlooked by clinicians. blood coagulation a genetically determined group coagulopathies caused deficiency plasma proteins involved hemostasis, as well fibrinogen, prothrombin, factor V (FV), factors and VIII (FV+FVIII), VII (FVII), X (FX), XI (FXI), XII (FXI...

Background: Coagulation plays an important role in haemostasis. Bleeding disorders caused by deficiency of certain coagulation factors such as hemophilia and Von Willebrand disease can affect haemostasis and may endanger life. Aim: To put a focus on the aetiology, pathogenesis, methods of diagnosis and lines of management of bleeding disorders. Conclusion: Bleeding disorders usually result from...

Mild hereditary bleeding disorders presenting with mucocutaneous haemorrhages are usually difficult to diagnose. We measured thrombin generation in platelet-poor plasma (TG-PPP) in 206 patients with a clinically unequivocal bleeding tendency: 45 with von Willebrand disease (vWD), 49 with platelet aggregation/secretion defects (PASD), 10 with a combination of both and 102 who did not fit the dia...