Acquired haemophilia A in a patient who is a Jehovah's Witness

Abstract

We detail the case of a man in his 80s who was a Jehovah's Witness, presenting to hospital for the second time in 1 week with atraumatic, severe bruising affecting his right thigh and flank. He was subsequently diagnosed with idiopathic acquired haemophilia A (AHA) and was urgently treated with recombinant factor concentrate and immunosuppressive therapy. Management of his bleeding disorder and resultant severe anaemia was adapted in line with his religious beliefs. AHA is a rare bleeding disorder which should be considered in patients with an isolated prolonged activated partial thromboplastin time and a history of recent or acute bleeding. Prompt diagnosis and management are essential as delays may result in increased mortality. Given that this patient declined blood transfusion, management of his bleeding disorder presented a unique challenge.

Keywords: Ethics; Haematology (drugs and medicines); Haematology (incl blood transfusion).

Figure 1

The top (A) and bottom (C) plots show factor VIII (FVIII) and haemoglobin (Hb) levels, respectively, versus days since diagnosis of acquired haemophilia A. Normal range for FVIII lies between the red dashed lines in (A). The treatment schematic (B) illustrates the treatments given and the days on which they were administered.

Figure 2

Axial CT of the head showing a moderate volume acute subdural haematoma with associated mass effect and midline shift (A) and subsequent progressive midline shift to the left (B). Please note that the presence of the patient’s cochlear implant has resulted in interference affecting the right side.