Emicizumab in Patients With Acquired Hemophilia A

Inclusion Criteria:

• Signed Informed Consent/Assent Form

• Age ≥18 years at time of signing Informed Consent Form

• Ability to comply with the study protocol, in the investigator's judgment

• Diagnosis of AHA based on a reduced FVIII activity (<50 %) and positive FVIIIinhibitor (>0.6 BU/ml) at screening (local laboratory)

• Current bleeding due to AHA at the time of screening

• Plan to be adherent to emicizumab prophylaxis during the study

• For women of childbearing potential who meet the following criteria:

• Refrain from heterosexual intercourse or use contraceptive methods that result in afailure rate of <1% per year during the study period A woman with ≥ 12 continuousmonths of amenorrhea with no identified cause other than menopause and has notundergone surgical sterilization (removal of ovaries and/or uterus). use of combinedoral or injected hormonal contraceptive, bilateral tubal ligation, malesterilization, hormone- releasing intrauterine devices, and copper intrauterinedevices.

Exclusion Criteria:

• Congenital hemophilia A

• Treatment with aPCC within the last 24 hours before first study treatment or plannedtreatment with aPCC during the course of the study

• Known positive lupus anticoagulant at the time of screening

• Severe uncontrolled infection at the time of screening

• Signs of active disseminated intravascular coagulation at the time of screening -

• Emicizumab ⎯ AHA Emi Version 1.0 20

• Current treatment for thromboembolic disease or signs of current thromboembolicdisease at time of screening

• Patients who are at high risk for TMA (e.g., have a previous medical or familyhistory of TMA), in the investigator's judgment

• Known severe congenital or acquired thrombophilia

• Life expectancy <3 months at the time of screening

• Other conditions that substantially increase risk of bleeding or thrombosis by thediscretion of the investigator

• Contraindications according to the Investigator's Brochure of emicizumab

• Current treatment with emicizumab at time of screening

• History of clinically significant hypersensitivity associated with monoclonalantibody therapies or components of the emicizumab injection by the discretion ofthe investigator

• Concurrent disease, treatment, or abnormality in clinical laboratory tests thatcould interfere with the conduct of the study, may pose additional risk, or would,in the opinion of the local investigator, preclude the patient's safe participationin and completion of the study

• Addiction or other diseases that preclude the patient from appropriately assessingthe nature and scope as well as possible consequences of the clinical study by thediscretion of the investigator

• Pregnant or breast-feeding women

• Would refuse treatment with blood or blood products, if necessary.

• Subject is in custody by order of an authority or a court of law

• Treatment with any of the following:

• An investigational drug to treat or reduce the risk of hemophilic bleeds within 5half-lives of last drug administration before Study Day 1

• A non-hemophilia-related investigational drug within the last 30 days or 5half-lives- before Study Day 1, whichever is longer

• An investigational drug concurrently

• History of clinically significant hypersensitivity associated with monoclonalantibody therapies or components of the emicizumab injection