Abstract
Introduction Venous thrombosis is rare in the setting of factor VIII (FVIII) deficiency. Cases
of deep vein thrombosis (DVT) have been described in hemophiliacs after recent major
surgery, or in association with the administration of FVIII concentrate and activated
prothrombin complex concentrates, but occurrence of spontaneous DVT is even more uncommon.
Aim We describe the challenging management of extensive DVT in a patient with acquired
hemophilia A with concurrent hemorrhagic manifestations and review similar published
cases.
Methods We summarize a series of 10 cases with the following demographics: 6 males and 4
females; median age at presentation of 65 (21–80); mean inhibitor titer of 68.5 Bethesda
Units (BU 1.9 to BU 350).
Results Four cases were idiopathic and six had associated conditions (cancer [two cases],
recent pregnancy [two cases], and recent surgery [two cases]). Three cases had an
inferior vena cava filter inserted for acute lower limb DVT/pulmonary embolism. Inhibitor
eradication was achieved with high-dose steroids with or without cyclophosphamide,
and adjunct Rituximab administration was used in three cases. One patient received
concurrent therapeutic plasma exchange (TPE). Inhibitor eradication was fastest with
concurrent TPE at 6 days (range: 6–733 days). The 30-day survival was 90%.
Conclusions There was adequate response of inhibitors to immunosuppression with steroids and
cyclophosphamide therapy. For more refractory disease, Rituximab is emerging as a
beneficial and cost-effective adjunct with better rates of complete remission, and
the threshold for its use may be lowered in this complex cohort with dual competing
pathologies.
Keywords
acquired hemophilia - deep vein thrombosis - thromboembolism
