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.

Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation

“…[4][5][6][7][8][9][10][11][12][13][14][15][16] This is likely to have led to the reporting of more severely affected and younger patients. This view is supported by the finding that the patients reported in this series 1,20,21 are significantly older than previously reported, have a lower incidence of pregnancy-related acquired hemophilia, and a higher incidence of malignancy.…”

“…29,30 The studies reporting immunosuppressive regimens have almost invariably been singlecenter cohorts that have not included controls. [4][5][6][7][8][9][10][11][12][13][14][15][16] These studies will tend to preferentially report good outcomes as opposed to average or poor outcomes and journals will similarly be more likely to publish good outcome studies. One randomized study published to date found no difference between nonremitters randomized to cyclophosphamide and those that continued with steroids after an initial 3-week course of steroids.…”

“…The clinical characteristics of the disease have been previously described. [1][2][3] The literature describing acquired hemophilia A is based on tertiary referral single-center cohorts, [4][5][6][7][8][9][10][11][12][13][14][15][16] larger retrospective surveys of referral center patients, 1,5,[17][18][19] and patients reported because they have been treated for bleeding. [20][21][22][23] These reports have been combined in a review and meta-analysis.…”

“…22,23 Data on inhibitor eradication, however, are based on relatively small, uncontrolled single-center cohorts [4][5][6][7][8][9][10][11][12][13][14][15][16] and a metaanalysis. 24 These reports are potentially unrepresentative because referral center patients may be a subgroup of patients.…”

Treatment of acquired hemophilia by the Bonn-Malmö Protocol: documentation of an in vivo immunomodulating concept

“…Bleeding events are often fulminant in cancer patients, though inhibitor titers are low. 14 In the study of Sallah and Wan, 5 CR was achieved in only up to 70% of cancer patients. Cancer treatment alone eradicated the inhibitor in 22% of the patients.…”