caplacizumab

General

Pronunciation:
kap-la-siz-ue-mab

Trade Name(s)

Cablivi

Ther. Class.

none assigned

Pharm. Class.

anti-von Willebrand Factor

monoclonal antibodies

Indications

Acquired thrombotic thrombocytopenic purpura (in combination with plasma exchange and immunosuppressive therapy).

Action

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.

Therapeutic Effect(s):

Increased platelet count and cessation of plasma exchange.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability; 90% absorbed following SUBQ 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.

Half-life: Unknown.

TIME/ACTION PROFILE (plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
SUBQ	Unknown	6–7 hr	Unknown

Contraindication/Precautions

Contraindicated in:

Hypersensitivity.

Use Cautiously in:

Underlying coagulopathy (↑ risk of bleeding)
Severe hepatic impairment
OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk;
Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
Pedi: Safety and effectiveness not established in children.

Adverse Reactions/Side Effects

Derm: urticaria

Hemat: BLEEDING

MS: back pain, myalgia

Neuro: fatigue, headache, paresthesia

Resp: dyspnea

Misc: fever, hypersensitivity reactions

* CAPITALS indicate life-threatening.
Underline indicate most frequent.

Interactions

Drug-Drug

↑ risk of bleeding with anticoagulants, aspirin, clopidogrel, ticagrelor, prasugrel, fibrinolytics, NSAIDs, SNRIs, or SSRIs ; avoid concurrent use.

Route/Dosage

IV SUBQ (Adults): 1st day of treatment: 11 mg IV bolus given ≥15 min before plasma exchange, then followed by 11 mg SUBQ after completion of plasma exchange; Subsequent days of treatment during daily plasma exchange: 11 mg SUBQ once daily after completion of plasma exchange; Treatment after plasma exchange period: 11 mg SUBQ once daily for 30 days 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 patient experiences ≥2 recurrences of acquired thrombotic thrombocytopenic purpura while on therapy.

Availability

Lyophilized powder for injection: 11 mg/vial

Assessment

Monitor for signs/symptoms of bleeding (epistaxis, gingival bleeding, upper GI hemorrhage, menorrhagia) during therapy. If significant bleeding occurs, hold and consider administering vWF concentrate as indicated. If caplacizumab resumed, continue to monitor for bleeding.

Implementation

IV Push: Reconstitution: Bring vial and diluent syringe to room temperature. Attach provided syringe of 1 mL sterile water for injection to vial adaptor. Do not remove syringe from vial adaptor. Gently swirl vial until completely dissolved; do not shake. Solution is clear and colorless; do not administer if cloudy, discolored, or contains particulates. Concentration: 11 mg/mL. Withdraw reconstituted solution from vial into syringe. For initial IV push injection, if using an IV line, connect glass syringe to a standard Luer lock; flush with 0.9% NaCl or D5W. Health care provider to give 1st dose immediately or within 4 hr of reconstitution if refrigerated.
SUBQ Reconstitute subsequent doses using technique above. Administer SUBQ into abdomen avoiding 2 inches around navel; rotate quadrants with each injection. Pinch skin and inject at 45°–90° angle; do not rub injection site.

Patient/Family Teaching

Explain purpose and side effects of medication. Advise patient to read Patient Information before starting therapy.
Instruct patient to administer doses missed during plasma exchange period as soon as possible. Administer doses missed after plasma exchange period within 12 hr; if >12 hr, omit missed dose and administer according to usual schedule.
Instruct patient or caregiver on appropriate steps for measuring accurate dose, administration technique and disposal of equipment, if self-administration is appropriate.
Advise patient that bruising and bleeding may occur more easily and may take longer than usual to stop bleeding. Advise patients to notify health care provider immediately if excessive bleeding or bruising occur.
Caution patient to notify health care provider and hold caplacizumab for 7 days prior to elective surgery, dental procedures or other invasive procedures.
Rep: Inform patient of ↑ risk of bleeding in pregnancy. Advise females of reproductive potential to notify health care provider if pregnancy is planned or suspected or if breastfeeding. Monitor exposed neonate for signs of bleeding.

Evaluation/Desired Outcomes