In hemophiliac patients, which of the following should not be given?
Correct Answer: Platelet factor
Description: Antifibrinolytic Drugs:
Bleeding in the gum, gastrointestinal tract, and during oral surgery requires the use of oral antifibrinolytic drugs such as ε-aminocaproic acid (EACA) or tranexamic acid to control local hemostasis. The duration of the treatment depending on the clinical indication is 1 week or longer. Tranexamic acid is given at doses of 25 mg/kg three to four times a day. EACA treatment requires a loading dose of 200 mg/kg (maximum of 10 g) followed by 100 mg/Kg per dose (maximum 30 g/d) every 6h.
General considerations regarding the treatment of bleeds in hemophilia include the following: (1) Treatment should begin as soon as possible because symptoms often precede objective evidence of bleeding; because of the superior efficacy of early therapeutic intervention, classic symptoms of bleeding into the joint in a reliable patient, headaches, or automobile or other accidents require prompt replacement and further laboratory investigation, (2) Drugs that hamper platelet function, such as aspirin or aspirin containing drugs, should be avoided; to control pain, drugs such as ibuprofen or propoxyphene are preferred. FVIII and FIX are dosed in units. One unit is defined as the amount of FVIII (100 ng/mL) or FIX (5 μg/mL) in 1 mL of normal plasma. One unit of FVIII per kilogram of body weight increases the plasma level by 2%. One can calculate the dose needed to increase FVIII levels to 100% in a 70-kg severe hemophilia patient (<1%) using the simple formula below. Thus, 3500 units of FVIII will raise the circulating level to 100%.
FVIII dose (IU) = Target FVIII levels – FVIII baseline levels x Body weight (kg) x 0.5 unit/kg.
The doses for FIX replacement are different from those for FVIII, because FIX recover after infusion is usually only 50% of the predicted value. Therefore, the formula for FIX replacement is as follows:
FIX dose (IU) = Target FIX levels – FIX baseline levels x Body weight (Kg) x 1 unit/Kg.
The FVIII half-life of 8-12 h requires injections twice a day to maintain therapeutic levels, whereas the FIX half-life is longer, 24 h, so that once-a-day injection is sufficient. In specific situations such as after surgery, continuous infusion of factor may be desirable because of its safety in achieving sustained factor levels at a lower total cost. Cryoprecipitate is enriched with FVIII protein (each bag contains 80 IU of FVIII) and was commonly used for the treatment of hemophilia A decades ago; it is still in use in some developing countries, but because of the risk of bloodborne diseases, this product should be avoided in hemophilia patients when factor concentrates are available.
Reference: Harrison’s Principles of Internal Medicine 20th ed page no 832
Category:
Pathology
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