Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report.
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.
Piton
Gaël
G
Intensive Care Unit, Besancon University Hospital, France. piton.gael@wanadoo.fr
Navellou
Jean Christophe
JC
Morati
Pierre
P
Patry
Cyrille
C
Carbonnel
Franck
F
Capellier
Gilles
G
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
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human