tailieunhanh - Chapter 110. Coagulation Disorders (Part 6)

Factor XI Deficiency: Treatment The treatment of FXI deficiency is based on the infusion of FFP at doses of 15–20 mL/kg to maintain trough levels ranging from 10 to 20%. Because FXI has a half-life of 40–70 h, the replacement therapy can be given on alternate days. The use of antifibrinolytic drugs is beneficial to control bleeds, with the exception of hematuria or bleeds in the bladder. The development of a FXI inhibitor was observed in 10% of severely FXI-deficient patients who received replacement therapy. Other Rare Bleeding Disorders Collectively, the inherited disorders resulting from deficiencies of clotting factors other than. | Chapter 110. Coagulation Disorders Part 6 Factor XI Deficiency Treatment The treatment of FXI deficiency is based on the infusion of FFP at doses of 15-20 mL kg to maintain trough levels ranging from 10 to 20 . Because FXI has a half-life of 40-70 h the replacement therapy can be given on alternate days. The use of antifibrinolytic drugs is beneficial to control bleeds with the exception of hematuria or bleeds in the bladder. The development of a FXI inhibitor was observed in 10 of severely FXI-deficient patients who received replacement therapy. Other Rare Bleeding Disorders Collectively the inherited disorders resulting from deficiencies of clotting factors other than FVIII FIX and FXI Table 110-1 represent a group of rare bleeding diseases. The bleeding symptoms in these patients vary from asymptomatic dysfibrinogenemia or FVII deficiency to life-threatening FX or FXIII deficiency . There is no pathognomonic clinical manifestation that suggests one specific disease but overall in contrast to hemophilia hemarthrosis is a rare event and bleeding in the mucosal tract or after umbilical cord clamping is common. Individuals heterozygous for plasma coagulation deficiencies are often asymptomatic. The laboratory assessment for the specific deficient factor following screening with general coagulation tests Table 110-1 will establish the diagnosis. Replacement therapy using fresh frozen plasma FFP or PCCs containing prothrombin FVII FIX and FX provides adequate hemostasis in response to bleeds or as prophylactic treatment. The use of PCCs should be carefully monitored and avoided in patients with underlying liver disease or those at high risk for thrombosis because of the risk of DIC. Familial Multiple Coagulation Deficiencies Several bleeding disorders are characterized by the inherited deficiency of more than one plasma coagulation factor. To date the genetic defects in two of these diseases have been characterized and they provide new insights into the regulation of