Acquired haemophilia in the elderly is a severe disease: report of five new cases

Abstract

Acquired haemophilia is a rare but life-threatening bleeding disease that can be observed in males or females at various ages. In the present study, we report on five cases of acquired factor (F) VIII inhibitors diagnosed in the elderly population over a period of 5 years between 1995 and 1999 in our hospital. The median age of the patients at the time of diagnosis was 76.2 years (66-92 years). In all cases, the diagnosis was suggested by mild to severe bleeding with no previous bleeding history. While the absence of associated conditions is frequently reported especially among the elderly, in our series an underlying disease was found in four out of the five cases: kidney tumour (two cases) and autoimmune disease (two cases). The bleeding was controlled in four patients using porcine FVIII (two cases) or recombinant FVIIa (two cases). The inhibitors were completely resolved in two patients (kidney tumour, GoodPasture syndrome) by treatment of the underlying disease. However, three patients died as direct or indirect consequence of having an inhibitor. Our series confirms and extends previous data reporting the complexity and severity of this disorder. Because bleeding is often severe, a prompt and correct diagnosis is required to provide adequate therapeutic options that take the advanced age of the patients into account.