LETTER TO THE EDITOR Gastrointestinal bleeding due to angiodysplasia in patients with type 1 von Willebrand disease: report on association and management S. SIRAGUSA,* A. MALATO,* L. LO COCO,* V. CIGNA,* G. SACCULLO,* I. ABBENE,* R. ANASTASIO,* D. CARAMAZZA,* R. PATTI,  M. ARCARA  and G. DI VITA   *Haematology and Thrombosis/Haemostasis Unit, Department of Oncology; and  Department of Surgical and Oncological Science, Division of General Surgery, University of Palermo, Palermo, Italy The presence of gastrointestinal angiodysplasia (GI-A) can represent an urgent haematological problem as it produces serious bleeding, which is usually untreatable. It has been recognized that G-IA frequently occurs in patients with type 2–3 and acquired von Willebrand disease (VWD) but the strength of this association is unclear. One hypoth- esis relays on the lack of high molecular weight (HMW) molecules of von Willebrand factor (VWF) in the plasma [1]. Other findings suggest a potential role being played by the 3916T mutation of the esone 28 coding factor vW (domain A1) [2]. Angiodys- plastic lesions can be found along the entire length of the GI tract, from the tongue to the colon. Although there are several diagnostic methods such as endos- copy, push enteroscopy, helical computed tomogra- phy (CT), mesenteric angiography and, more recently, video capsule endoscopy [3] available, the identification of angiodysplasia remains extremely difficult to document and most of the patients remain undiagnosed. Thereby, most patients are treated solely medically with several and mainly ineffective approaches [3]. Most previous publications on VWD and angi- odysplasia have been reports of the association itself and there is little published data on the management and follow-up of affected patients. In addition, no such association has yet been documented in patients with type 1 VWD. We report our experiences in the management and follow-up of three patients suffer- ing from angiodysplasia and type 1 VWD, according to the current classification [4]. Their baseline phenotypic characteristics are outlined in Table 1; investigation and management of individual patients are summarized in Table 2. The first patient was a 49-year-old female with inherited type 1 VWD but no serious bleeding tendency. She presented with melena and iron- deficiency anaemia. Activity of both von Willebrand factor antigen (VWF:Ag) and von Willebrand factor Ristocetin co-factor activity (VWF:RCo) was reduced to <20%. GI endoscopy and colonoscopy proved normal at the time of her first hospital admission. The patient was treated with blood replacement, tranexamic acid and desmopressin (DDAVP) infusion, but these latter two approaches did not effectively reduce the bleeding. We therefore initiated VWF/factor VIII (FVIII) concentrate (Hae- mate P Ò , CSL Behring) at the therapeutic dosage, 40–50 IU kg )1 every day and then every two days for 4 weeks following hospital discharge. She did not display any bleeding symptoms for the next 5 months, when she then experienced a new incident of melena and serious anaemia (haemoglobin <7.0 g dL )1 ). Mesenteric angiography and capsular endoscopy were thus performed: the latter procedure revealed two small angiodysplastic lesions in the ileum, clinically insignificant. We therefore initiated medical therapy with oestrogen and Haemate P Ò 30 IU kg )1 every alternate day for 8 weeks. How- ever, the patient experienced an adverse event to oestrogen therapy and serious bleeding when the VWF concentrate was withdrawn. Symptoms of anaemia continued for the next 22 months; GI bleeding was always reduced during prophylaxis with Haemate P Ò but commenced again whenever this was suspended. At that time, we repeated GI Correspondence: Sergio Siragusa, MD, Haematology and Thrombosis/Haemostasis Unit, Department of Oncology, Univer- sity Hospital of Palermo, Via del Vespro 127, I-90127 Palermo, Italy. Tel./fax: + 39 091 6554574; e-mail: sergio.siragusa@unipa.it Accepted after revision 1 October 2007 Haemophilia (2008), 14, 150–152 DOI: 10.1111/j.1365-2516.2007.01596.x Ó 2007 The Authors 150 Journal compilation Ó 2007 Blackwell Publishing Ltd