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Acquired hemophilia in patients with hematologic malignancies
S Sallah1, N P Nguyen, J M Abdallah
1Department of Medicine, Division of Hematology/Oncology, University of Tennessee, Memphis, Tenn. 38163, USA.
Objectives:
To evaluate the occurrence of acquired hemophilia in patients with hematologic malignancies and to assess their response to treatment.
Design:
Data on 8 patients with hematologic neoplastic disorders and inhibitor against factor VIII were analyzed retrospectively.
Setting:
Three large tertiary-care centers.
Results:
All 8 patients presented with spontaneous or posttraumatic hemorrhages. The mean inhibitor titer at the time of diagnosis was 79 Bethesda units (BU), and residual factor VIII activity was detectable in 3 patients. The inhibitor disappeared in 5 patients after a mean of 92 days, but persisted in the 3 other patients. The patients who achieved complete resolution of their circulating anticoagulant had lower mean inhibitor titers at the time of diagnosis than those who had persistent inhibitor (27 BU vs. 167 BU, respectively). Two patients died as a result of major hemorrhages that did not respond to treatment.
Conclusions:
Antibodies against factor VIII may be responsible for some bleeding episodes in patients with lymphoid or myeloid malignancies. Acquired hemophilia in this setting should be differentiated from other causes of bleeding because the approach to treatment is different. No conclusion can be drawn regarding the association between the activity of the underlying illness and the inhibitor titer, although it appears that at least in some patients such a relationship may exist. The underlying pathogenetic mechanisms responsible for the production of autoantibodies against factor VIII remain unclear, but we provide a few explanations in this article.