Head of the Department of Pediatric Hematology and Oncology Clinical Hospital Centre Rijeka Rijeka, Primorsko-Goranska, Croatia
Background: Acquired haemophilia A is a rare but potentially life-threatening haemorrhagic disorder caused by autoantibodies directed mostly against coagulation factor VIII. Age distribution is bimodal, with a first peak occurring among young women in the postpartum period, and a second major peak of incidence among elderly patients. The disorder is rare in children less than 16 years old, and the incidence is 0.45/million/year.
Aims: The aim of this work is to present an extremely rare case of a 14-year-old girl with acquired haemophilia.
Methods: Case presentation
Results: A 14-year-old girl was admitted to the Clinical Hospital Centre Rijeka with a 2-year history of bleeding tendency into the skin, muscles and soft tissues. Past medical and family histories were negative. Prior to admission the girl was seen by several paediatricians, rheumatologists and orthopaedists. An isolated prolongation of the activated partial thromboplastin was found, with FVIII levels 5% and FVIII inhibitor titre 3.5 BU. The fifth day she developed spontaneous extensive muscle bleed of the right thigh. The treatment was started with recombinant activated factor VII (rFVIIa), and progression of bleeding ceased. Because of further unavailability of bypassing therapy, anti-haemorrhagic treatment was continued with recombinant FVIII concentrates. Along with the control of acute bleeding, the patient underwent immunosuppressive therapy with corticosteroids in combination with cyclophosphamide to eradicate autoantibodies. Factor VIII inhibitor levels gradually decreased. The girl was discharged on day 15, and remained on oral prednisolone and cyclophosphamide over the next 6 weeks. At 5-year follow-up, she has complete sustained clinical and laboratory response.
Conclusion(s): Although very uncommon, the diagnosis of acquired haemophilia should be considered in any child who presents with unexplained bleeding and a prolonged activated partial thromboplastin time. Acquired autoantibodies directed against coagulation factors may result in serious, life-threatening bleeding. The management of acute bleeding and the inhibitor eradication are the mainstay of treatment.
