Patient case (clinical description): A 20-year-old man comes to the emergency due to bleeding after a tooth extraction.  The bleeding has persisted for approximately 30 minutes despite constant direct pressure.  He is a known case of Hemophilia type A treated with FVIII. Blood pressure is 95/60 mm Hg and pulse is 105/min. His weight is 70 Kg. Family history is positive for Hemophilia type A in his maternal uncle. He also has a lipoma on his left arm which he plans to remove surgically. His FVIII activity is 40%.

Patient's own account: I got my tooth extracted and I could not stop bleeding for 30 min. I'm a 20 yo dude but I have Hemophilia type A treated with FVIII. My blood pressure was 95/60 and my pulse 105/min. I'm 70 kg. My uncle on my mom's side also has type A hemophilia. I also have a fatty swelling on my left arm and I was supposed to remove it. My FVIII activity was 40%.

Clinical trial: Emicizumab in Acquired Hemophilia A

Eligibility criteria:
Inclusion Criteria:

          -  Patients diagnosed with AHA based on a reduced FVIII activity (<50 %) and positive
             FVIII inhibitor (>0.6 BU/ml) at screening (local laboratory)

          -  Signed informed consent form by the participant or a Person who is legally authorized
             to sign on behalf of the participant before any study specific tests or procedures are
             done

          -  Male or female patients aged 18 years or older at the time of informed consent

          -  Ability to understand and follow study-related instructions

          -  Current bleeds due to AHA at the time of screening

        Exclusion Criteria:

          -  Congenital hemophilia A

          -  Treatment with aPCC within the last 48 h before first study treatment or planned
             treatment with aPCC during the course of the study

          -  Treatment of AHA within the days before study enrollment with more than 100 mg
             prednisolone (or equivalent) per day or prednisolone for more than 2 days or with
             other immunosuppressive drugs (e.g. rituximab, cyclophosphamide). IST for other
             concomitant disorders (e.g. autoimmune disorders) is not an exclusion criterion and
             can be continued at the investigator's discrétion

          -  Therapy (current or planned during the emicizumab treatment period) with
             immunospuppressive or immune modulating drugs that were not already given on a regular
             basis before first diagnosis of AHA

          -  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

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

          -  Patients who are at high risk for TMA (e.g., have a previous medical or family history
             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 the
             discretion of the investigator

          -  Contraindications according to the local SmPC of emicizumab (see 16.1 Appendix I)

          -  Current treatment with emicizumab at time of screening

          -  History of clinically significant hypersensitivity associated with monoclonal antibody
             therapies or components of the emicizumab injection by the discretion of the
             investigator

          -  Concurrent disease, treatment, or abnormality in clinical laboratory tests that could
             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 participation in and
             completion of the study

          -  Addiction or other diseases that preclude the patient from appropriately assessing the
             nature and scope as well as possible consequences of the clinical study by the
             discretion of the investigator

          -  Pregnant or breast-feeding women

          -  Women of childbearing potential unless women who meet the following criteria:

               1. Post-menopausal (12 months natural amenorrhea or 6 months amenorrhea with serum
                  FSH > 40 U/mL)

               2. Postoperatively (six weeks after bilateral ovariectomy with or without
                  hysterectomy)

               3. Regular and correct use of a contraceptive method with error rate <1% per year
                  such as implants, depot injections, oral contraceptives or intrauterine devices

               4. Sexual abstinence

               5. Vasectomy of the partner

          -  Men of sexual activity with women of childbearing potential who are not willing to use
             an effective barrier method of contraception during and up to 3 months after the end
             of therapy

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

          -  Receipt of an investigational drug concurrently or within 5 half-lives before
             administration of the study drug
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 18 Years old.


Eligibility (NLI label): Contradiction