Acquired von Willebrand syndrome: a rare disorder of heterogeneous etiology.

CONTEXT: Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that mimics the inherited form of von Willebrand disease (VWD) in terms of laboratory findings and clinical presentation.

AIMS: To study the etiology of acquired VWD.

SETTINGS AND

DESIGN

The patients referred from various hospitals in and out of Mumbai were included in the study.

MATERIALS AND

METHODS

Six patients with AVWS diagnosed at this center over the last 10 years were analyzed against 171 patients with inherited VWD. The differential diagnosis of AVWS was made based on reduced levels of von Willebrand antigen and von Willebrand ristocetin cofactor, decrease in ristocetin induced platelet aggregation, absence of correction in mixing studies with no prior history of bleeding problems and a negative family history for bleeding disorders.

RESULTS

In three patients, the disease was associated with systematic lupus erythematosus, out of which one was also associated with Kikuchi lymphadenitis and second with autoimmune hemolytic anemia. Fourth case was associated with hypothyroidism and fifth was a case of dermatitis and vitiligo. The last patient was a case of hemophilia A with Burkitts lymphoma, who developed autoantibodies to von Willebrand factor. Except two patients, all other patients responded to immune suppressive therapy with corticosteroids, while the patient with hypothyroidism responded to oral thyroxine.

CONCLUSION

AVWS is a rare condition and may often be missed or diagnosed as inherited disease associated with heterogeneous disease conditions.