title: Two elderly patients with difficult-to-treat acquired hemophilia A.
abstract: We herein report the cases of two elderly patients with acquired hemophilia A (AHA) for whom treatment was difficult.An 89-year-old woman (Case 1) was admitted to our department with subcutaneous hemorrhage and melena. Her activated partial thromboplastin time (APTT), factor VIII activity, and factor VIII inhibitor level were 127.7 seconds, 1.0%, and 48 BU/mL, respectively, which was suggestive of AHA. The administration of prednisolone (PSL 0.5 mg/kg) was initiated. After 3 weeks, PSL was combined with cyclophosphamide (CPA 50 mg). Two months after the start of treatment, her factor VIII inhibitor level decreased to 3.4 BU/mL. However, hemorrhagic signs were repeatedly observed during the discontinuation of recombinant activated factor VII (rFVIIa) preparation; bleeding control became insufficient, and pneumonia developed, thus leading to a fatal outcome.An 81-year-old woman (Case 2) was admitted to our department with subcutaneous hemorrhage, anemia (Hb: 9.2 g/dL), and a prolonged APTT (78.7 seconds). Her factor VIII activity was reduced to 0.9%, and her factor VIII inhibitor level was markedly increased to 1,364.9 BU/mL, suggesting AHA. Treatment with PSL (0.5 mg/kg) was initiated. After one month, it was combined with CPA (50 mg); however, her hemorrhagic signs were protracted, and her Hb level decreased to 8.0 g/dL. Subsequently, pneumonia occurred. However, weekly rituximab therapy (375 mg/m2) for 4 weeks decreased her factor VIII inhibitor level, leading to the disappearance of the inhibitor at 1 year and 5 months. During this period, there were no episodes requiring the administration of bypassing agents, such as rFVIIa.
authors.affiliation: Department of Internal Medicine and Hematology, Aiiku Hospital.
authors.forename: Makoto
authors.initials: M
authors.lastname: Saito
authors.forename: Masanobu
authors.initials: M
authors.lastname: Morioka
mesh_terms: D000369:Aged, 80 and over; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005169:Factor VIII; D005260:Female; D006467:Hemophilia A / Q000188:drug therapy*; D006470:Hemorrhage; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D005169:Factor VIII