Treatment of women with bleeding disorders.45
| Agent . | Dose . | Disease Indication . | Level* . | Mechanism . | Side Effects . |
|---|---|---|---|---|---|
| * Level of Evidence: A indicates that the recommendation is based on clinical trial data; B indicates the recommendation is based on laboratory data; and C indicates the recommendation is based on opinion of experienced clinicians. It should be noted that the level of recommendation is based on general bleeding symptoms and not on menorrhagia. | |||||
| Abbreviations: vWD, von Willebrand disease; vWF, von Willebrand factor; ASA, aspirin; OCP, oral contraceptive; DIC, disseminated intravascular coagulation | |||||
| Desmopressin (DDAVP) | 0.3 μg/kg IV | Type 1 vWD Hemophilia A carrier Platelet dysfunction | (A) | Releases vWF from endothelial cells by binding to ADH | Flushing, headache, tachycardia, hyponatremia, volume overload, tachyphylaxis |
| Stimate (Intranasal) (1.5 mg/mL) | 150 μg/nostril < 50 kg, one nostril ≥ 50 kg, both nostrils | (A) | V2 receptor and activating CAMP | ||
| von Willebrand factor concentrate | 40 U/kg, then 25 U/kg q 12–24 h | Type II, III vWD Type 1 unresponsive to DDAVP | (A) | Replaces vWF | Allergic reaction, hepatitis, transmissible agent |
| Factor VIII concentrate (Recombinant) | 50 U/kg, then 25 U/kg q 8–12 h | Factor VIII deficiency, severe or moderate Acquired vWD | (A) | Replaces factor VIII | Allergic reaction |
| Factor IX concentrate (Recombinant) | 75 U/kg, then 38 U/kg q 12–24 h | Factor IX deficiency Hemophilia B carrier | (A) | Replaces factor IX | Allergic reaction |
| Plasma | 5 units = 20% level or 10 mL/kg | Factor II, V, XI deficiency Liver disease Vitamin K deficiency DIC, TTP Marrow failure (malignancy, chemotherapy) | (A) | Replaces factors | Fever, chills, hepatitis, transmissible agent hepatitis, HIV |
| Retested plasma | (C) | ||||
| Cryoprecipitate | 6 bags = 1200 mg fibrinogen | Factor I deficiency Dysfibrinogenemia Factor XIII deficiency Uremic bleeding, DIC | (C) | Replaces fibrinogen Replaces factor XIII | Fever, chills transmissible agent hepatitis, HIV |
| Factor VIIa (Recombinant) | 90 μg/kg q 2–3 h 20 μg/kg q 6–8 h | Factor VIII inhibitor Factor VII deficiency Glanzmann thrombasthenia | (A) (A) | Activates tissue factor Replaces factors VII, IX | Thrombosis |
| Autoplex, FEIBA | 75–100 U/kg, then 50 U/kg q 6–8 h | Factor VIII inhibitor | (A) | Replaces factor IX Activates tissue factor | Thrombosis, HIV, hepatitis, inhibitor transmissible agent |
| Platelet transfusion | 1 unit/10 kg (to 60 kg) | Thrombocytopenia Bernard-Soulier syndrome Glanzmann thrombasthenia ASA platelet defect Platelet-type vWD DIC | (A) | Replaces platelets, restores platelet function | Fever, allergic reactions |
| Amicar (epsilon amino caprioc acid) | 50 mg/kg q 6–8 h | Congenital bleeding disorder | (A) | Prevents lysis of clots by inhibiting plasminogen binding to fibrin | Nausea, vomiting, kidney sludge, stone |
| Tranexamic acid | 4 g/day (15 mg/kg) | vWD Hemophilia A, B carrier Factor deficiencies | (C) | Prevents lysis of clots by inhibiting plasminogen binding to fibrin | Nausea, vomiting, diarrhea, hypotension |
| Estrogens | Mid-dose OCP | vWD Hemophilia A,B carrier Factor deficiencies | (B,C) | Increases factor levels Endometrial changes | Nausea, headache, thrombosis, cardiovascular risk |
| Hepatitis A Vaccine | Congenital bleeding disorder | (A) | Protects from hepatitis A, B | Soreness at site, nonspecific symptoms | |
| Hepatitis B Vaccine | (A) | ||||
| Agent . | Dose . | Disease Indication . | Level* . | Mechanism . | Side Effects . |
|---|---|---|---|---|---|
| * Level of Evidence: A indicates that the recommendation is based on clinical trial data; B indicates the recommendation is based on laboratory data; and C indicates the recommendation is based on opinion of experienced clinicians. It should be noted that the level of recommendation is based on general bleeding symptoms and not on menorrhagia. | |||||
| Abbreviations: vWD, von Willebrand disease; vWF, von Willebrand factor; ASA, aspirin; OCP, oral contraceptive; DIC, disseminated intravascular coagulation | |||||
| Desmopressin (DDAVP) | 0.3 μg/kg IV | Type 1 vWD Hemophilia A carrier Platelet dysfunction | (A) | Releases vWF from endothelial cells by binding to ADH | Flushing, headache, tachycardia, hyponatremia, volume overload, tachyphylaxis |
| Stimate (Intranasal) (1.5 mg/mL) | 150 μg/nostril < 50 kg, one nostril ≥ 50 kg, both nostrils | (A) | V2 receptor and activating CAMP | ||
| von Willebrand factor concentrate | 40 U/kg, then 25 U/kg q 12–24 h | Type II, III vWD Type 1 unresponsive to DDAVP | (A) | Replaces vWF | Allergic reaction, hepatitis, transmissible agent |
| Factor VIII concentrate (Recombinant) | 50 U/kg, then 25 U/kg q 8–12 h | Factor VIII deficiency, severe or moderate Acquired vWD | (A) | Replaces factor VIII | Allergic reaction |
| Factor IX concentrate (Recombinant) | 75 U/kg, then 38 U/kg q 12–24 h | Factor IX deficiency Hemophilia B carrier | (A) | Replaces factor IX | Allergic reaction |
| Plasma | 5 units = 20% level or 10 mL/kg | Factor II, V, XI deficiency Liver disease Vitamin K deficiency DIC, TTP Marrow failure (malignancy, chemotherapy) | (A) | Replaces factors | Fever, chills, hepatitis, transmissible agent hepatitis, HIV |
| Retested plasma | (C) | ||||
| Cryoprecipitate | 6 bags = 1200 mg fibrinogen | Factor I deficiency Dysfibrinogenemia Factor XIII deficiency Uremic bleeding, DIC | (C) | Replaces fibrinogen Replaces factor XIII | Fever, chills transmissible agent hepatitis, HIV |
| Factor VIIa (Recombinant) | 90 μg/kg q 2–3 h 20 μg/kg q 6–8 h | Factor VIII inhibitor Factor VII deficiency Glanzmann thrombasthenia | (A) (A) | Activates tissue factor Replaces factors VII, IX | Thrombosis |
| Autoplex, FEIBA | 75–100 U/kg, then 50 U/kg q 6–8 h | Factor VIII inhibitor | (A) | Replaces factor IX Activates tissue factor | Thrombosis, HIV, hepatitis, inhibitor transmissible agent |
| Platelet transfusion | 1 unit/10 kg (to 60 kg) | Thrombocytopenia Bernard-Soulier syndrome Glanzmann thrombasthenia ASA platelet defect Platelet-type vWD DIC | (A) | Replaces platelets, restores platelet function | Fever, allergic reactions |
| Amicar (epsilon amino caprioc acid) | 50 mg/kg q 6–8 h | Congenital bleeding disorder | (A) | Prevents lysis of clots by inhibiting plasminogen binding to fibrin | Nausea, vomiting, kidney sludge, stone |
| Tranexamic acid | 4 g/day (15 mg/kg) | vWD Hemophilia A, B carrier Factor deficiencies | (C) | Prevents lysis of clots by inhibiting plasminogen binding to fibrin | Nausea, vomiting, diarrhea, hypotension |
| Estrogens | Mid-dose OCP | vWD Hemophilia A,B carrier Factor deficiencies | (B,C) | Increases factor levels Endometrial changes | Nausea, headache, thrombosis, cardiovascular risk |
| Hepatitis A Vaccine | Congenital bleeding disorder | (A) | Protects from hepatitis A, B | Soreness at site, nonspecific symptoms | |
| Hepatitis B Vaccine | (A) | ||||