Caplacizumab

CABLIVI is indicated for the treatment of adult and pediatric patients 12 years of age and older with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy. CABLIVI is a von Willebrand factor (vWF)-directed antibody fragment indicated for the treatment of adult and pediatric patients 12 years of age and older with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy.

Caplacizumab-yhdp targets the A1-domain of vWF, and inhibits the interaction between vWF and platelets, thereby reducing both vWF-mediated platelet adhesion and platelet consumption.

CABLIVI should be administered upon the initiation of plasma exchange therapy. The recommended dose of CABLIVI is as follows: First day of treatment: 11 mg bolus intravenous injection at least 15 minutes prior to plasma exchange followed by an 11 mg subcutaneous injection after completion of plasma exchange on day 1. Subsequent treatment during daily plasma exchange: 11 mg subcutaneous injection once daily following plasma exchange. Treatment after the plasma exchange period: 11 mg subcutaneous injection once daily for 30 days beyond the last plasma exchange. If after initial treatment course, sign(s) of persistent underlying disease such as suppressed ADAMTS13 activity levels remain present, treatment may be extended for a maximum of 28 days. Discontinue CABLIVI if the patient experiences more than 2 recurrences of aTTP, while on CABLIVI.

CABLIVI is contraindicated in patients with a previous severe hypersensitivity reaction to caplacizumab-yhdp or to any of the excipients. Hypersensitivity reactions have included urticaria. Previous severe hypersensitivity reaction to caplacizumab-yhdp or any of the excipients.

Hemorrhage: Serious and fatal bleeding can occur. Risk of bleeding is increased in patients with underlying coagulopathies or on concomitant antiplatelet agents or anticoagulants. If clinically significant bleeding occurs, interrupt treatment. Withhold CABLIVI 7 days prior to elective surgery, dental procedures, or other invasive interventions.

The following clinically significant adverse reactions are also discussed in other sections of the labeling: Hemorrhage In adults, the most common adverse reactions (incidence >15%) are epistaxis, headache, and gingival bleeding. In pediatric patients, the most frequently reported adverse reactions are epistaxis and tachycardia.

Concomitant use of anticoagulants or antiplatelet agents with CABLIVI may increase the risk of bleeding. Monitor closely for bleeding with concomitant use. Concomitant Use of Anticoagulants, Thrombolytic Drugs, Heparin or Antiplatelet Agents Concomitant use of CABLIVI with any anticoagulant, thrombolytic drugs, heparin or antiplatelet agent may increase the risk of bleeding. Avoid concomitant use when possible. Assess and monitor closely for bleeding with concomitant use.

Store refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light. Do not freeze. Unopened vials may be stored in the original carton at room temperature up to 30°C (86°F) for a single period of up to 2 months. Do not return CABLIVI to the refrigerator after it has been stored at room temperature.