Tranexamic acid may be given alone or together with standard doses of coagulation factor concentrates. (Level 4) [ [45] ] Tranexamic acid should not be given to patients with FIX deficiency receiving prothrombin complex Adriamycin concentrates,

as this will exacerbate the risk of thromboembolism. (Level 5) [ [46] ] If treatment with both agents is deemed necessary, it is recommended that at least 12 h elapse between the last dose of APCC and the administration of tranexamic acid. (Level 5) [ [46] ] In contrast, thromboembolism is less likely when tranexamic acid is used in combination with rFVIIa to enhance hemostasis. (Level 4) [ [47] ] Epsilon aminocaproic acid (EACA) is similar to tranexamic acid, but is less widely used as it has a shorter plasma half-life, is less potent, and is more toxic [40]. EACA is typically administered to adults orally or intravenously every 4–6 h up to a maximum of 24 g day−1 in an adult. A 250 mg mL−1 syrup formulation is also available. Gastrointestinal upset is a common complication; reducing

the dose often helps. Myopathy is a rare adverse reaction specifically reported in association with aminocaproic acid therapy (but not tranexamic acid), typically occurring after administration of high doses for several weeks. The myopathy is often painful BGJ398 mw and associated with elevated levels of creatine kinase and even myoglobinuria. Full resolution may be expected once drug treatment is stopped. Bleeding in patients with hemophilia MCE公司 can occur at different sites (Tables 1–1 and 1–1), each of which requires specific management. As a general principle in case of large internal hemorrhage, hemoglobin should be checked and corrected while other measures are being planned. Measures of hemodynamic stability, such as pulse and blood pressure, should be monitored as indicated. A joint bleed is defined as an episode characterized by rapid loss of range of motion as

compared with baseline that is associated with any combination of the following: pain or an unusual sensation in the joint, palpable swelling and warmth of the skin over the joint [1]. The onset of bleeding in joints is frequently described by patients as a tingling sensation and tightness within the joint. This “aura” precedes the appearance of clinical signs. The earliest clinical signs of a joint bleed are increased warmth over the area and discomfort with movement, particularly at the ends of range. Later symptoms and signs include pain at rest, swelling, tenderness, and extreme loss of motion. A re-bleed is defined as worsening of the condition either on treatment or within 72 h after stopping treatment [1]. A target joint is a joint in which 3 or more spontaneous bleeds have occurred within a consecutive 6-month period. Following a joint bleed, flexion is usually the most comfortable position, and any attempt to change this position causes more pain.