anti-von Willebrand Factor
Acquired thrombotic thrombocytopenic purpura (TTP) (in combination with plasma exchange and immunosuppressive therapy).
Targets the A1 domain of von Willebrand factor (vWF) and inhibits the interaction between vWF and platelets, which subsequently inhibits platelet adhesion and platelet consumption.
Increased platelet count and cessation of plasma exchange.
Absorption: IV administration results in complete bioavailability; 90% absorbed following subcutaneous administration.
Distribution: Minimally distributed to tissues.
Metabolism and Excretion: Target-bound caplacizumab metabolized by liver; unbound drug is broken down by proteolytic enzymes. Unbound drug excreted in the urine.
TIME/ACTION PROFILE (plasma concentrations)
Use Cautiously in:
- Underlying coagulopathy (↑ risk of bleeding)
- Severe hepatic impairment
- OB: Use only if potential maternal benefit justifies potential fetal risk; monitor neonates for bleeding
- Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant
- Pedi: Safety and effectiveness not established.
Adverse Reactions/Side Effects
MS: back pain, myalgia
Neuro: paresthesia, fatigue, headache
Misc: fever, hypersensitivity reactions
* CAPITALS indicate life-threatening.
Underline indicate most frequent.
IV Subcut (Adults): First day of treatment– 11 mg via IV bolus given ≥15 min before plasma exchange, then followed by 11 mg via subcut injection after completion of plasma exchange; Subsequent days of treatment during daily plasma exchange– 11 mg via subcut injection once daily after completion of plasma exchange; Treatment after plasma exchange period– 11 mg via subcut injection once daily for 30 day following last daily plasma exchange. If signs of persistent disease continue, treatment may be extended for up to an additional 28 days. Discontinue drug if patients experiences ≥2 recurrences of acquired TTP while on therapy.
Lyophilized powder for injection (requires reconstitution): 11 mg/vial
- Monitor for signs and symptoms of bleeding (epistaxis, gingival bleeding, upper gastrointestinal hemorrhage, and menorrhagia) during therapy. Risk is increased in patients with underlying coagulopathies (hemophilia, other coagulation factor deficiencies) or in patients taking drugs affecting hemostasis and coagulation. Interrupt therapy if significant bleeding occurs; may administer von Willebrand factor concentrate to rapidly correct hemostasis. Monitor for bleeding if caplacizumab is restarted.
- Deficient knowledge, related to medication regimen (Patient/Family/Teaching)
- First Day: administer 11 mg bolus IV push at least 15 min prior to plasma exchange followed by an 11 mg subcut injection after completion of plasma exchange. Subsequent days of treatment during daily plasma exchange: administer 11 mg subcut injection once daily following plasma exchange.
- Withhold therapy 7 days prior to elective surgery, dental procedures, or other invasive interventions.
- IV Push: Give first dose via IV push. Ensure vial and diluent syringe are at room temperature. Attach provided syringe of 1 mL Sterile Water for Injection to vial adaptor to reconstitute caplacizumab for 11 mg/mL single dose solution. Do not remove syringe from vial adaptor. Gently swirl vial until completely dissolved; do not shake. Solution is clear and colorless; do not administer solutions that are cloudy, discolored, or contain particulate matter. Withdraw reconstitutes solution from vial into syringe. Administer full amount of reconstituted solution. For initial IV push injection, if using an IV line, connect glass syringe to a standard Luer lock (and not a needleless connector); flush with 0.9% NaCl or D5W. Use solution immediately or within 4 hrs of reconstitution if refrigerated.
- Subcut Reconstitute subsequent doses using technique above. Administer subsequent doses subcut into abdomen avoiding 2 inches around navel; rotate quadrants with each injection. Pinch skin and inject at 45° to 90° angle; do not rub injection site. Patient and caregiver can be trained to administer subcut injections.
- Instruct patient to take caplacizumab as directed. Administer doses missed during plasma exchange period as soon as possible. Doses missed after plasma exchange period can be administered within 12 hrs of scheduled time of administration. Beyond 12 hours, omit missed dose and administer next daily dose according to usual dosing schedule. Advise patient to read Instructions for Use before starting and with each Rx refill in case of changes.
- Advise patient that bruising and bleeding may occur more easily, nose bleeds and bleeding of gums may occur, and may take longer than usual to stop bleeding. Advise patients to notify health care professional immediately if excessive bleeding or bruising occur.
- Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. Increased risk of bleeding in mother, fetus, and neonate; monitor closely.
Increased platelet count and cessation of plasma exchange in patients with acquired TTP.
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