Acquired immune-mediated von Willebrand syndrome accompanied by antiphospholipid syndrome

Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder with laboratory findings resembling those of congenital von Willebrand disease. AvWS usually occurs in association with a variety of underlying disorders, such as lymphoproliferative disease or cardiovascular disease, but autoimmune AvWS is very rare. We now describe the case of a 42-year-old woman with autoimmune AvWS with concurrent antiphospholipid syndrome (APS). The patient was suffering from epistaxis and menorrhagia from few years prior to referral. She was referred to our hospital because of Activated Partial Thromboplastin Time (APTT) prolongation. Autoimmune AvWS was diagnosed as hemostatic and immunological analyses showed very low (<6%) levels of vWF ristocetin cofactor activity, low (6%) levels of vWF antigen and the presence of anti-vWF antibodies (IgG1 and IgG2) as well as antiphospholipid antibodies. Steroid therapy led to prompt AvWS remission, but deep vein thrombosis occurred in the left leg, on day 36 and APS was diagnosed. A combination of steroid and anti-coagulant was given for approximately 3 years. Thrombosis has not recurred; but AvWS re-exacerbated with steroid tapering. This is the first case report of autoimmune AvWS concurrent with APS, and the long-term disease course described here can be beneficial to clinicians.