The acquired von Willebrand Syndrome (AvWS) is a bleeding disorder with laboratory findings similar to those of the congenital form. Its presentation is more common in adults than in children and is mainly associated with lymphoproliferative and myeloproliferative diseases and rarely with autoimmune diseases including systemic lupus erythematosus (SLE). Here, we report the case of a girl with SLE and AvWS with mucosal bleeding and low plasma levels of von Willebrand factor and factor VIII (FVIII) with failure to respond to treatment with first- and second-line therapies. The patient finally responded to rituximab. To our knowledge this is the first reported case of SLE associated with AvWS, that responded to rituximab.

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