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ACQUIRED HAEMOPHILIA A IN A POSTPARTUM PATIENT: DIAGNOSIS AND MANAGEMENT

Rosnah, B; Marini, R; Shafini, M Y; Haslina, M N Noor

Pathology - Journal of the RCPA: 2009 - Volume 41 - Issue - p 75
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Haematology Department, Health Campus, Universiti Sains Malaysia, Kubang Kerian Kelantan, Malaysia

Acquired haemophilia A is a potentially life threatening haemorrhagic disorder caused by spontaneous occurrence of autoantibodies directed against coagulation factor VIII. It has a mortality rate of up to 22%. We report a 40‐year‐old woman, para 5 who presented with 1 day history of painless haematuria. Seventy days earlier the patient experienced secondary postpartum haemorrhage following spontaneous vaginal delivery due to retained placenta, and was discharged well after ERPOC (evacuation of retained products of conception). Clinical examination was normal. Routine coagulation studies confirmed steady prolongation of activated partial thromboplastin time (APTT) up to 112 s with low factor VIII and high titre of factor VIII inhibitor (16.5 Bethesda units). Diagnosis of acquired haemophilia A was confirmed and she was treated with corticosteroid therapy, recombinant factor VII and intravenous immunoglobulin. The haematuria settled after 48 hours with normalisation of APTT and the level of factor VIII. After 2 months, the coagulation factor study revealed normal factor VIII level and disappearance of factor VIII inhibitors.

© 2009 Royal College of Pathologists of Australasia