Case study: bruising and bleeding secondary to acquired haemophilia

An 88-year-old Caucasian female was admitted from a care home with extensive bruising of the right upper limb. On examination, she was found to have generalised significant bruising all over her body (figure 1) without any fractures clinically or radiologically. Initially, a safeguarding alert was raised about possible non-accidental injuries, but closed later following a prompt investigation of the matter.

A CGA (comprehensive geriatric assessment) was performed including a medication review and reconciliation. The trauma and orthopaedics specialists reviewed her and advised a conservative management.

Her admission blood tests showed a haemoglobin of 48, normal platelet count (221) and a raised aPTT at 133 (activated partial thromboplastin time) requiring blood transfusions. She was not on any anti-platelets or anti-coagulant medications and no thromboprophylaxis was offered as she had extensive bruising. She sustained a witnessed fall with head injury on the ward.

A CT scan (computed tomography) of the head showed an acute right occipital subdural haematoma. It was managed conservatively as advised by the local neurosurgery team. Repeat blood tests showed a drop in haemoglobin and a further rise in aPTT.

A specialist haematology opinion was sought and tests performed for haemophilia screening after other causes of bruising and bleeding were excluded. Later, a diagnosis of acquired haemophilia was established. She was transferred to a local haemophilia unit and started on prednisolone, Factor Eight Inhibitor Bypassing Activity (FEIBA) and mycophenolate.

Acquired haemophilia is an autoimmune disorder caused by auto-antibodies inhibiting Factor VIII and its role in the clotting pathway. It does this by reducing thrombin formation on activated platelets, causing potentially critical, serious bleeding. More than 80% of those affected are males and females above the age of 65.¹

A typical case of acquired haemophilia presents with spontaneous subcutaneous haematomas with widespread bruising, in the absence of a personal or family history of haemophilia. Other signs include epistaxis, haematuria, gastrointestinal bleeding and intracranial bleeding. There is usually an increased aPTT.²

Primarily, treatment of acquired haemophilia should involve controlling the bleeding by achieving haemostasis. For minor bleeding, a medication review must be performed including stopping antiplatelet and anticoagulant agents. Desmopressin (DDAVP) can be used to induce a temporary rise in Factor VIII levels, as well as Human Factor VIII. If these don’t work, bypassing agents can be used, the 2 most common being Prothrombin Complex

Concentrate (PCC) and Factor Eight Inhibitor Bypassing Activity (FEIBA). Both aim to achieve haemostasis by producing thrombin at the bleeding site. In cases where haemostasis is not achieved with bypassing agents, porcine Factor VIII can be considered.

Once bleeding is controlled, treatment should be given to eradicate the inhibitory autoantibody. This is usually attained using immunosuppressive agents, such as steroids and cyclophosphamide. Second line agents include Rituximab, calcineurin inhibitors and mycophenolate.² Optimisation of treatment can also be achieved by better control of underlying conditions related to acquired haemophilia, including autoimmune disorders, pregnancy and underlying malignancies.⁴

Remission of acquired haemophilia is usually linked to high levels of Factor VIII. For the first 6 months of remission, monthly monitoring of aPTT and Factor VIII activity is recommended, then two or three months up to 12 months, then six monthly thereafter.1

Learning points

Whilst managing an older person with extensive bruising and bleeding acquired haemophilia should be considered in the diagnosis and haematology opinion be sought when appropriate.

Hardeep Galsinh, Locum senior house officer, UHB NHS Foundation Trust

Sowjanya Potturu, Consultant Geriatrician, UHB NHS Foundation Trust

Kanwaljit Singh, Consultant Geriatrician, UHB NHS Foundation Trust

References

1. Janbain M, Leissinger C, Kruse-Jarres R. Acquired hemophilia A: emerging treatment options. J Blood Med. 2015; 6: 143–150. doi: 10.2147/JBM.S77332
2. NHS England. Clinical Commissioning Policy:The use of Rituximab as a second line agent for the eradication of inhibitors in patients with Acquired Haemophilia. Reference: NHS England F02/P/a. p1-12.
3. Zeng Y, Zhou R, Duan X, Long D, Yang S. Interventions for treating acute bleeding episodes in people with acquired hemophilia A. Cochrane Database Syst Rev. 2014 Aug 28;(8):CD010761. doi:10.1002/14651858.CD010761.pub2.
4. Baudo F, de Cataldo F. The problem of acquired haemophilia. Blood. 2015 Feb 12;125(7):1052-3. doi: 10.1182/blood-2014-12-618959

Patient consent: Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient/proxy.