Marstacimab

HYMPAVZI is indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adults and pediatric patients 6 years of age and older with: • hemophilia A (congenital factor VIII deficiency) with or without factor VIII inhibitors, or • hemophilia B (congenital factor IX deficiency) with or without factor IX inhibitors. HYMPAVZI is a tissue factor pathway inhibitor (TFPI) antagonist indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adults and pediatric patients 6 years of age and older with: • hemophilia A (congenital factor VIII deficiency) with or without factor VIII inhibitors, or • hemophilia B (congenital factor IX deficiency) with or without factor IX inhibitors.

Marstacimab‑hncq is a human monoclonal IgG1 antibody directed against the Kunitz domain 2 (K2) of TFPI to neutralize TFPI activity and enhance coagulation. TFPI is the primary inhibitor of the extrinsic coagulation cascade and negatively regulates thrombin generation within the extrinsic pathway of coagulation by inactivating the protease functions of FXa/FVIIa/TF complex. TFPI binds to and inhibits the factor Xa active site via its second Kunitz inhibitor domain (K2).

• See Full Prescribing Information for important dosing and administration instructions. • Recommended Dosage in Adults and Pediatric Patients 12 Years of Age and Older: o Loading dose: 300 mg (two 150 mg injections) by subcutaneous injection. o Maintenance dose: One week after the loading dose, initiate maintenance dosing of 150 mg every week by subcutaneous injection on the same day each week, at any time of day. o Dose adjustment to 300 mg subcutaneous injection weekly can be considered.

None. None.

• Thromboembolic Events: Thromboembolic events may occur. Interrupt HYMPAVZI prophylaxis if symptoms occur. • Hypersensitivity: Hypersensitivity reactions may occur. In the event of a severe allergic reaction, discontinue HYMPAVZI. • Embryofetal Toxicity: May cause fetal harm. Advise females of reproductive potential of the potential risk to the fetus and to use effective contraception. • Increased Laboratory Values of Fibrin D-dimer and Prothrombin Fragment 1.2: HYMPAVZI can cause sporadic or transient increases in fibrin D-dimer and prothrombin fragment 1.2 above physiological values.

The following clinically significant adverse reactions are described elsewhere in the labeling: • Thromboembolic Events • Hypersensitivity Adverse reactions reported in ≥2% of HYMPAVZI-treated patients were injection site reaction, headache, pyrexia, arthralgia, diarrhea, pruritus, and rash.

Partial Thromboplastin Time (aPTT) and Prothrombin Time (PT) No clinically significant differences in standard measures of coagulation including activated partial thromboplastin time (aPTT) and prothrombin time (PT) were observed following marstacimab‑hncq therapy.

• Store refrigerated at 36°F to 46°F (2°C to 8°C) in the original carton to protect from light. • If needed, HYMPAVZI may be stored one time at room temperature [up to 86°F (30°C)] in its original carton to protect from light for up to 7 days. Once stored at room temperature, do not return to the refrigerator and discard after 7 days. • Do not freeze. • Do not shake.