title: Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report.
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.
authors.affiliation: Intensive Care Unit, Besancon University Hospital, France. piton.gael@wanadoo.fr
authors.forename: Gaël
authors.initials: G
authors.lastname: Piton
authors.forename: Jean Christophe
authors.initials: JC
authors.lastname: Navellou
authors.forename: Pierre
authors.initials: P
authors.lastname: Morati
authors.forename: Cyrille
authors.initials: C
authors.lastname: Patry
authors.forename: Franck
authors.initials: F
authors.lastname: Carbonnel
authors.forename: Gilles
authors.initials: G
authors.lastname: Capellier
mesh_terms: D051722:ADAM Proteins / Q000172:deficiency*; D000071120:ADAMTS13 Protein; D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D005260:Female; D006801:Humans; D008875:Middle Aged; D010195:Pancreatitis / Q000097:blood / Q000150:complications*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000150:complications*; D055815:Young Adult
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human