Table 6. Nontransfusional Therapies for Treatment or Prevention of Bleeding in Patients With Bleeding Disorders
AntifibrinolyticsInclude ε-aminocaproic acid and tranexamic acid
Interfere with the formation of the fibrinolytic enzyme plasmin from its precursor plasminogen
First-line therapy in rare disorders of fibrinolysis (α2-antiplasmin and PAI-1 deficiencies)56
Systemic or topical antifibrinolytics may be used alone to treat or prevent mucosal bleeding in patients with coagulation factor disorders, thus avoiding systemic hemostatic therapies1,6668,73
Effective, first-line therapy for menorrhagia (with or without hormonal therapies)25,80,85
Tranexamic acid used anecdotally in patients with bleeding disorders, including as an adjunct to factor replacement for major surgery in patients with hemophilia78,81,84 and as a sole systemic hemostatic agent primarily for minor procedures1,66
Also used for treatment of bleeding and for hemostatic coverage for minor surgery in patients with platelet function disorders; may be used adjunctively with platelet transfusion and other nontransfusional therapies in these patients83
Theoretical risk for thrombogenesis; may be increased in patients who receive concurrent factor replacement or who have certain bleeding disorders (eg, dysfibrinogenemia, in which antifibrinolytics are contraindicated)
DesmopressinTransiently increases VWF and FVIII levels89 and augments platelet adhesiveness and aggregation68
Agent of choice for treatment or prevention of bleeding in most patients with type 1 VWD; however, patients with type 3 VWD and most patients with type 2 VWD typically require factor concentrates containing FVIII and VWF90
Other desmopressin-responsive bleeding disorders include mild hemophilia A and platelet function disorders, in which the use of desmopressin for surgical coverage has been described21,87; also commonly used for uremic bleeding88
Specifically used for treatment of menorrhagia in women with bleeding disorders25,80,91
Patients, especially children, receiving desmopressin should be closely monitored for hyponatremia22,87
Vitamin KMay be used in vitamin K deficiency and for bleeding caused by overmedication with warfarin
Widely available and inexpensive
Should be given intravenously or orally (not subcutaneously) for best absorption and fastest response9294
  • FVIII, factor VIII; PAI, plasminogen activator inhibitor; VWD, von Willebrand disease; VWF, von Willebrand factor.