TITLE:
Acquired von Willebrand Syndrome in a Male with Systemic Lupus Erythematosus Presented with Mucocutaneous Bleeding and Treated with rFVIIa
AUTHORS:
Maryam Hami, Hassan Ahmadnia, Zahra Rezaieyazdi, Hassan Mansouritorghabeh
KEYWORDS:
Acquired von Willebrand Disease; Lupus Erythematosus Systemic; rFVIIa; Bleeding
JOURNAL NAME:
International Journal of Clinical Medicine,
Vol.5 No.1,
January
8,
2014
ABSTRACT: Background: Systemic lupus erythematosis (SLE) is
a disorder with multiple organ involvement. Haematological abnormalities have
been addressed in it, but acquired von Willebrand syndrome is a rarer
phenomenon in current disease. The Case: We report acquired von Willebrand
syndrome and SLE in a man with brown rash on face, gingival bleeding, easy
bruising and epistaxis and laboratory finding of decreased complement, high
level of anti-nuclear antibody and anti-DNA. These findings confirmed the
diagnosis of SLE. He underwent kidney biopsy and experienced severe pain at the
site of biopsy, but the ultra-sonography evaluation showed small sub capsular
haematoma at the site of biopsy. During the next 48 hours, gradually APTT
prolongation was continued and haematocrit dropped. In spite of FFP infusion
and taking tranexamic acid every eight hours, there wasn’t any improvement in
haemostatic condition. He received Methylprednisolone and Cyclophosphamid
pulses. The patient underwent surgery to roll out vascular complication, but
there wasn’t any vascular problem. On the third day, recombinant activated
factor VII was infused every two hours until oozing was stopped.