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TTP: A COMPLICATION OF POST-ERCP PANCREATITIS
M Veerasamy1, B Brinker2, R Meisner2
1Internal Medicine Residency; 2Grand Rapids, Michigan, USA
INTRODUCTION: Thrombotic thrombocytopenic purpura (TTP) is a rare syndrome traditionally characterized by the clinical pentad of thrombocytopenia, microangiopathic hemolytic anemia, neurologic changes, renal dysfunction, and fever. It causes thrombosis in the microvasculature of several organs, producing diverse manifestations including acute pancreatitis (AP). We report a patient who developed TTP after presenting with post-Endoscopic Retrograde Cholangio-pancreatography (ERCP) pancreatitis suggesting AP to be the cause, rather than a consequence, of TTP.
Case description: A 28 years old Caucasian female presented with abdominal pain and was found to have cholelithiasis. She had laparoscopic cholecystectomy and the intraoperative cholangiogram showed a filling defect in the ampulla of Vater. ERCP and sphincterotomy was done She developed pancreatitis the following day and she was treated conservatively. Two days later, she developed anemia, thrombocytopenia, fever, renal failure and mental status changes. A peripheral smear showed 3+ schistocytes and fibrinogen levels were normal. TTP was diagnosed and she improved with daily plasmaphresis. No relapses occurred.
DISCUSSION: PubMed search with key words “pancreatitis” and “TTP” revealed 27 citations, including many reports of TTP presenting as pancreatitis. Only a few articles implicate pancreatitis as a cause of TTP. There were no reports similar to our patient.
While the pathophysiologic mechanism remains unclear, investigators speculate that the release of proteolytic enzymes from AP into the systemic circulation causes modification of von Willebrand factor, that enables spontaneous platelet membrane receptor binding, and results in intravascular platelet aggregation and TTP. There is one report of recurrence of TTP associated with relapsing episodes of pancreatitis that suggests that a cause-and-effect relationship exists.
SIGNIFICANCE: Our case appears to be the first report of a patient with post-ERCP pancreatitis who developed TTP. Thus, TTP may constitute a hematological complication of AP.