Von Willebrand disease, angiodysplasia and atorvastatin


We read with interest the report of a patient with severe type I von Willebrand disease (VWD) and angiodysplasia responding to atorvastatin (Sohal & Laffan, 2008). As a direct result of this report we reviewed the case of a 59-year-old man with Type 2A VWD. During the period 1992–2003 he required a total of 235 units of blood to be transfused for anaemia due to bleeding from angiodysplasia of the small and large intestine. Subsequently, he was admitted to hospital on 30 occasions in 2007, receiving a total of 70 units of blood; in 2008 there were a further 18 admissions with 52 units of blood transfused. Unsurprisingly, he has developed a number of red cell antibodies, including Anti-Fya, Anti-K and Anti-Kpa.

In addition to supportive blood transfusions we have tried to manage his symptoms with intermediate purity factor replacement in the form of Haemate P. However, he has struggled with peripheral venous access and recurrent portocath thromboses followed by failure of a surgically created arteriovenous fistula in the arm.

He has also received adjunctive therapy in the form of ethinyloestradiol in 2004 and thalidomide in 2005, but these were stopped due to intolerant side effects. He also suffered a myocardial infarction in 2004 following the administration of recombinant factor VIIa during an acute bleeding episode.

We commenced our patient on atorvastatin 10 mg nocte in August 2008, increasing the dose by 10 mg per month until November 2008. He was maintained on 40 mg nocte for 3 months, with a noticeable reduction in blood transfusion requirements. In January 2009, the dose of atorvastatin was increased to 80 mg nocte. The patient has now been on this dose for the past 9 months and has not required hospital admission or a single unit of blood to be transfused during this period (Fig 1).

Figure 1.

 Effect of atorvastatin dose on blood transfusion requirements. A downward trend in bleeding episodes and the need for blood transfusion was observed as the dose of atorvastatin was increased from 10 to 40 mg. At a dose of 80 mg atorvastatin there have been no bleeding episodes and no blood transfusions during the past 9 months.