Acquired haemophilia A in a patient with neoplastic disease Case report

Abstract

Acquired haemophilia A (AH) is a rare autoimmune disorder caused by inhibitory antibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous personal or family history of bleeding. Morbidity and mortality are high, primarily because of bleeding and adverse effects of immune therapy. In about 12% of cases AH is secondary to malignancy, with two-thirds of associated malignancy being solid tumors. Authors present a case of a 67-year-old patient with AH who experienced several bleeding episodes, which were treated with factor VIII “by-passing agents” including activated prothrombin complex concentrates (aPCC, FEIBA) and recombinant activated factor VIIa (rVIIa, NovoSeven). The patient was resistant to many inhibitor eradication procedures. In the 8th year of follow-up severe gastrointestinal bleeding episodes occurred, diagnosed as due to small intestine tumor with hepatic metastases. The two surgical procedures have been performed, but the patient died in the postoperative period due to cardiac arrest. The postmortem histopathological examination of the small intestine tumor revealed non-Hogdkin lymphoma. The purpose of this report is to increase awareness of the possibility of AH as the reason of bleeding in patients with malignancy as well as to stress, that all invasive procedures in AH are burdened with a high risk of complications which include fatal bleedings despite adequate administration of factor VIII by-passing agents.