Acquired Hemophilia Presenting with Upper Extremity Venous Thrombosis

In contrast to the typical presentation of swelling, pain, a rash, or darkened skin in venous thrombosis of the extremity, the typical appearance of acquired hemophilia is mucocutaneous bleeding. Both diseases may be associated with an elevated D-dimer concentration. Venous thrombosis is uncommon in patients with acquired hemophilia, which has a prevalence of one to four in one million per year. Although the typical appearance of erythema and swelling of the extremity is highly suggestive of venous thrombosis, a prolongation of activated partial thromboplastin time should not be ignored. Here, we describe two patients with acquired hemophilia misdiagnosed as venous thrombosis, highlighting the importance of differential diagnosis through familiarization of their clinical manifestations. The appropriate diagnostic strategy and treatment for acquired hemophilia were also reviewed.