Parahemophilia

Parahemophilia is a hereditary abnormality of blood clotting

Parahemophilia is a rare, mutational disorder of the primary blood coagulation system. It is characterized by an inability to produce sufficient amounts of prothrombin and fibrinogen. Common factors for this



Parahemophilia is a hereditary disorder of hemostasis that is extremely rare (1 case per 50–70 thousand people). It manifests itself as frequent, recurring nosebleeds with decreased blood clotting. They cause parahemorrhagic diathesis - other types of bleeding (melena, hemorrhagic cystitis), as well as increased fragility of blood vessels during subcutaneous injuries and operations, and ineffectiveness of blood transfusions. The typical course of the disease with hemorrhagic diathesis is combined with fragility of the nasal septum, hemorrhages in the conjunctiva and under the mucous membranes of the eyelids. Diagnostics is helped by coagulological studies, including tests for congenital platelet aggregation, plasma tests of thromboplastin and prothrombin time, tests of APTT, fibrinogen, von Willebrand factor with quantitative platelet count with their morphological study to exclude tumor and immunological etiology, ultrasound sonography, angioscanning of vessels with contrast and subsequent rheography.