Factor XI deficiency( hemophilia c)

The disease is transmitted by an autosomal type of inheritance, which causes them to suffer from both sexes.

There are latent( latent), small and severe forms of the disease. In the first form, which is more than 50% of all cases of hemophilia C, bleeding occurs occasionally, only with large injuries and surgeries. With a mild form of the disease, spontaneous bleeding is minimal or absent, but bleeding due to injuries and surgical interventions naturally occurs. Small surgical interventions( removal of teeth, polyps of the intestine, etc.) are rarely accompanied by bleeding. For the severe form of the disease, moderate spontaneous hemorrhage( nasal bleeding, easy bruising, etc.) is characteristic, as well as prolonged and profuse bleeding during surgical interventions. Occasionally, subcutaneous and intermuscular hematomas and acute hemorrhages in the joints are observed. In this regard, the disease acquires some resemblance to the lightest varieties of hemophilia A or B.

In women, menstrual bleeding does not always occur, and mostly only during puberty. Very rarely( in isolated cases) postpartum haemorrhage is recorded. Most women, including those with very low level of factor XI in plasma, do not require transfusion prevention and therapy in childbirth. In contrast, trauma and surgical interventions for hemophilia C can be complicated by severe and life-threatening bleeding. Often such bleeding becomes a complete surprise to the treating doctors, since nothing allows you to suspect violations in the blood clotting system.

Treatment. Reliable stop and prevention of bleeding is provided by intravenous injection of natural, freshly frozen or dry plasma. The period of effect of the factor XI introduced into the bloodstream after the first transfusion is about 60 hours, with repeated administration increases to 120 hours or more. Therefore, to ensure normal blood coagulation in the postoperative period, intravenous plasma injections are sufficient in the first days every 48 hours and then after 72 hours.

From non-specific treatments for this disease, e-aminocaproic acid is most successfully used to prevent and stopbleeding with minor injuries and operations( cuts, tooth extraction).