CASE REPORT Acquired von Willebrand’s disease associated with gastrointestinal angiodysplasia: a case report P. K. GUPTA, M. KANNAN, T. CHATTERJEE, A. DIXIT, M. MAHAPATRA, V. P. CHOUDHRY and R. SAXENA Department of Haematoloy, All India Institute of Medical Sciences, New Delhi, India Summary. Acquired von Willebrand syndrome (AVWS) is a rare and probably underestimated bleeding disorder which mimics most of the clinical symptoms and laboratory features of hereditary von Willebrand disease (VWD) in patients devoid of both personal and family history of bleeding diathesis. In this study, we present a case of 55 yrs male patient, presented with gastrointestinal bleeds since three years, diagnosed to have AVWS with inhibitors. From this study it is concluded that AVWS is rare and it is important to diagnose this bleeding disorder so that appropriate treatment with plasmapheresis and IV:Ig can effectively correct the haemostatic defect and manage severe bleeding in these patients. Keywords: acquired von Willebrand’s disease, gas- trointestinal angiodysplasia, India Introduction Acquired von Willebrand syndrome (AVWS) is a rare and probably underestimated bleeding disorder which mimics most of the clinical symptoms and laboratory features of hereditary von Willebrand’s disease (VWD) in patients devoid of both personal and family history of bleeding diathesis [1]. AVWS and congenital VWD both result from a defect in von Willebrand factor (VWF), a large multimeric glycoprotein (GP) that binds to platelet receptors GPIb and GPIIb/IIIa and is involved in adhesion and aggregation of platelets at the site of vascular injury [2]. The revised classification of hereditary VWD [3] includes three major types: type 1 and type 3 are, respectively, related to partial or a total quantitative defect while type 2 (variants including 2A, 2B, 2M, 2N) results from a quantitative defect of VWF. Although, VWD is the most common inherited bleeding disorder, with a prevalence of about 1% [4], more than 300 cases of AWVS have been reported since it was initially described in 1968. It is likely, however, that this number is underestimated as isolated case reports of AVWS are not published and its diagnosis remains difficult. The mechanisms involved in AWVS remain diffi- cult to characterize, but the three main ones postu- lated include the presence of circulating antibodies that inactivate functional domains of VWF or form a complex with VWF, which is rapidly cleared from the circulation [5,6], selective adsorption of VWF onto abnormal cells [7] and increased proteolysis of VWF induced by enzymes not clearly identified, although platelet proteases could be involved [8]. AVWS has been reported in association with lymphoproliferative disorders, monoclonal gammo- pathies, kidney neoplasm, autoimmune disorders, myeloproliferative syndromes, several congenital heart diseases, angiodysplasia, hypothyroidism, drugs and other diseases [9–14]. In this study, we present a case report of an Indian patient of gastrointestinal (GI) angiodysplasia diag- nosed to have AVWS with inhibitors. Case report A 55-year-old male patient, presented with GI bleeds over 3 years was reported in the present study. He had received multiple blood transfusions, but with no improvement. He was diagnosed both clinically Correspondence: Prof. Renu Saxena, Department of Haematology, I.R.C.H. Building (1st floor), All India Institute of Medical Sci- ences, Ansari Nagar, New Delhi 110 029, India. Tel.: 91-011-6593642; fax: 91-011-6862663; e-mail: renusax@hotmail.com Accepted after revision 8 April 2004 Haemophilia (2006), 12, 452–455 DOI: 10.1111/j.1365-2516.2006.01301.x 452 Ó 2006 Blackwell Publishing Ltd