images in haematology
Acquired haemophilia A in hepatocellular carcinoma
A 62-year-old woman presented with epistaxis and non-traumatic severe ecchymosis of her trunk and right arm (left). Previous medical history included refractory multifocal hepatocellular carcinoma (HCC) with right atrial thrombus and multiple pulmonary emboli. Computed tomography scanning demonstrated HCC within the right lobe of the liver with inferior vena cava extension (right). Enoxaparin was stopped and epistaxis controlled locally. Coagulation tests showed an activated partial thromboplastin time ratio of >700 (normal range 085–11) that did not correct on mixing with normal plasma. Anti-activated factor X activity was absent. Factor VIII levels were found to be 2 iu/dl (normal range 50–200) and the Bethesda assay showed a factor VIII inhibitor at 415 Bethesda units (BU)/ ml, confirming a diagnosis of acquired haemophilia A (AHA). Autoimmune and viral screens were negative. Acquired haemophilia A is a rare and life-threatening bleeding disorder caused by the development of autoantibod-
ª 2013 John Wiley & Sons Ltd British Journal of Haematology, 2014, 164, 617
ies against factor VIII clotting factor. The clinical presentation differs from that of hereditary haemophilia A, with the majority of patients bleeding into skin and soft tissues, as seen in the left image. AHA can be associated with malignancy, and one previous case in association with HCC has been reported [Neilson, R.F., Walker, I.D., Roberson, M. (1993) Factor VIII inhibitor associated with hepatocellular carcinoma. Clinical & Laboratory Haematology, 15, 145–148]. This was a rare and challenging case, which posed complicated management dilemmas. In particular, the conflicting treatments of thromboembolic disease and this life-threatening bleeding disorder, followed by the risk versus benefit of attempting AHA antibody eradication given the severity and refractory nature of her underlying malignancy. James Croft and Satyajit Sahu Department of Haematology, Eastbourne District General Hospital, Eastbourne, East Sussex, UK. E-mail: [email protected]
First published online 18 October 2013 doi:10.1111/bjh.12610
Acquired haemophilia A in hepatocellular carcinoma
A 62-year-old woman presented with epistaxis and non-traumatic severe ecchymosis of her trunk and right arm (left). Previous medical history included refractory multifocal hepatocellular carcinoma (HCC) with right atrial thrombus and multiple pulmonary emboli. Computed tomography scanning demonstrated HCC within the right lobe of the liver with inferior vena cava extension (right). Enoxaparin was stopped and epistaxis controlled locally. Coagulation tests showed an activated partial thromboplastin time ratio of >700 (normal range 085–11) that did not correct on mixing with normal plasma. Anti-activated factor X activity was absent. Factor VIII levels were found to be 2 iu/dl (normal range 50–200) and the Bethesda assay showed a factor VIII inhibitor at 415 Bethesda units (BU)/ ml, confirming a diagnosis of acquired haemophilia A (AHA). Autoimmune and viral screens were negative. Acquired haemophilia A is a rare and life-threatening bleeding disorder caused by the development of autoantibod-
ª 2013 John Wiley & Sons Ltd British Journal of Haematology, 2014, 164, 617
ies against factor VIII clotting factor. The clinical presentation differs from that of hereditary haemophilia A, with the majority of patients bleeding into skin and soft tissues, as seen in the left image. AHA can be associated with malignancy, and one previous case in association with HCC has been reported [Neilson, R.F., Walker, I.D., Roberson, M. (1993) Factor VIII inhibitor associated with hepatocellular carcinoma. Clinical & Laboratory Haematology, 15, 145–148]. This was a rare and challenging case, which posed complicated management dilemmas. In particular, the conflicting treatments of thromboembolic disease and this life-threatening bleeding disorder, followed by the risk versus benefit of attempting AHA antibody eradication given the severity and refractory nature of her underlying malignancy. James Croft and Satyajit Sahu Department of Haematology, Eastbourne District General Hospital, Eastbourne, East Sussex, UK. E-mail: [email protected]
First published online 18 October 2013 doi:10.1111/bjh.12610
