Acquired haemophilia A in an elderly patient

Published Date: 19th July 2016

Publication Authors: Mavinamane S

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Background

Acquired haemophilia A is a rare condition that can have potentially life-threatening consequences. We report a case of acquired haemophilia A in an elderly patient who had presented to hospital with recurrent bleeding episodes.

Case presentation

A 73 year old female nursing home resident with advanced dementia and previous stroke was admitted with bilateral arm swelling and bruising. She had also been experiencing recurrent vaginal bleeding and had recent admissions under the Gynaecology team where no cause was found for the bleeding.

Investigations

Investigations revealed a haemoglobin level of 7.1 g/dL. Her platelet count, prothrombin time and mean corpuscular volume were within normal limits. However her activated partial thromboplastin time (APTT) was prolonged at 45 s. Intrinsic pathway factor assay investigations showed normal Factor IX, XI and XII levels, but low Factor VIII with no correction in APTT mixing studies indicating strong inhibition of Factor VIII. This is consistent with a diagnosis of acquired haemophilia A.

Treatment

The patient was commenced on prednisolone 40 mg once daily, as well as fluconazole 50 mg once daily and co-trimoxazole 480 mg once daily as fungal and pneumocystis pneumonia prophylaxis in view of the longterm steroid use. 2 weeks after starting the prednisolone, her APTT had decreased to 30 s which was within normal limits and haemoglobin was 12.0 g/dL. At this point the prednisolone dose was tapered.

Discussion

Acquired haemophilia A has an incidence of 1.5 per million per year. It typically has a bimodal age distribution with the first peak in young adults, commonly women in the postpartum period. The second peak occurs in the elderly and may be idiopathic, although frequently associated with malignancy, drugs and viral infections. Acquired haemophilia A occurs due to the development of autoantibodies to Factor VIII. The two main aims of treatment are to control the bleeding and to eradicate the inhibitor by immunosuppression. Bleeding can be treated with activated prothrombin complex concentrate or recombinant Factor VIIa, which are thought to be more effective than Factor VIII or desmopressin. Immunosuppression can be achieved using steroids alone or in combination with a cytotoxic agent such as cyclophosphamide. Rituximab has been used recently but its use has not been shown to be associated with significantly better outcomes. 10–20% of patients may relapse within the first 6 months after immunossuppression is stopped. Acquired haemophilia has a mortality rate of 8–22%.

Learning points

Acquired haemophilia should be considered in elderly patients who present with recurrent bleeding episodes. Prompt diagnosis and treatment is essential to avoid the life-threatening complications of acquired haemophilia.

Jacob, A; Mavinamane, S. (2013).  Acquired haemophilia A in an elderly patient: A case report . European Journal of Internal Medicine. 24 (Supp 1), e163. 

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