Case ReportsThrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case reportPiton, Gaëla; Navellou, Jean Christophea; Morati, Pierreb; Patry, Cyrillea; Carbonnel, Franckc; Capellier, GillesaAuthor Information aIntensive Care Unit bDigestive Surgery Unit cGastroenterology and Nutrition Unit, Besancon University Hospital, France Correspondence to Dr Gaël Piton, Service de Réanimation Médicale, Hôpital Jean Minjoz, Boulevard Fleming, 25000 Besançon, France Tel: +33 38 166 8254; e-mail: [email protected] Received 6 December 2007 Accepted 4 March 2008 European Journal of Gastroenterology & Hepatology: December 2008 - Volume 20 - Issue 12 - p 1226-1230 doi: 10.1097/MEG.0b013e3282ffd9e6 Buy Metrics Abstract Thrombotic thrombocytopenic purpura (TTP) is a severe multisystemic microvascular disease defined by the association of hemolytic anemia, thrombocytopenia, acute renal failure, fever, and neurological disorders. The pathophysiology has recently been elucidated by the discovery of a von Willebrand factor-cleaving protease (ADAMTS13) deficiency involved in platelet aggregation and ischemia. The association between TTP and acute pancreatitis (AP) has rarely been reported, described either as a cause or a consequence. The role of ADAMTS13 during AP is still unknown. We describe the case of a 41-year-old woman who developed a TTP, with decreased ADAMTS13 activity, associated with severe AP. Published cases of thrombotic microangiopathy associated with AP are reviewed. The pathophysiology, management, prognostic factors, and rationale for treatment are discussed. AP should be sought in patients with TTP presenting with abdominal pain. On the other hand, TTP should be considered in patients with AP and thrombocytopenia. © 2008 Lippincott Williams & Wilkins, Inc.