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.
Highlights
The von Willebrand disease comprises most common inherited bleeding disorder in the world with prevalence about 1% - 2% [1]
In 1968, Simon et al described a case of acquired von Willebrand syndrome (AvWS) in systemic lupus erythematosus (SLE) for the first time [2]
In comparison with congenital von Willebrand disease (vWD), haemorrhagic manifestations including mucocutanous and gastrointestinal bleeding and post trauma haemorrhage are similar, which are compatible with types I and II vWD [4]
Summary
The von Willebrand disease (vWD) comprises most common inherited bleeding disorder in the world with prevalence about 1% - 2% [1]. The bone stone of treatment comprises elimination of underlying disease. It is not successful in the case of acute bleeding and necessary invasive procedure [6]. The. Presented with Mucocutaneous Bleeding and Treated with rFVIIa other therapeutic options are infusion of high dose immunoglobulin, plasma exchange, Corticosteroid, Tranexamic acid and recombinant activated Factor VII (rFVIIa). We present our experience on a case with AvWS in association with SLE. The therapeutic regimen including Methylprednisolone (one gr/day IV) × five and infusion of FFP and tranexamic acid was done without response to bleeding. The surgery for refuting vascular complications and removal of emerged haematoma and overcoming bleeding was done using infusion of rFVIIa successfully
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