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Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports

Hematology-Oncology Section, Department of Medicine, College of Medicine (KKS, JTD, JNG); Department of Biostatistics and Epidemiology, College of Public Health, The University of Oklahoma Health Science Center Oklahoma City, OK, USA (SKV, JNG); Division of Hematology/Oncology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA (HCK, BK); Department of Hematology and Central Hematology Laboratory, University Hospital, Berne, Switzerland (BL, JAKH)

Correspondence: James N. George, M.D., The University of Oklahoma Health Sciences Center, Hematology-Oncology Section, P.O. Box 26901, Oklahoma City, OK 73190 USA. E-mail: james-george{at}ouhsc.edu

Background and Objectives: The thrombotic thrombocytopenic purpura-hemolytic uremic syndromes (TTP-HUS) have diverse etiologies, clinical manifestations, and risk factors, but the events that may trigger acute episodes are often unclear. We describe the occurrence of TTP-HUS following pancreatitis and consider whether pancreatitis may be a triggering event for acute episodes of TTP-HUS.

Design and Methods: We report on three patients from the Oklahoma Registry and two patients from Northwestern University who had an acute episode of TTP-HUS following pancreatitis. A systematic review of published case reports was performed to identify additional patients who had TTP-HUS following pancreatitis.

Results: In each of our five patients there was an apparent etiology of alcoholism or common bile duct obstruction for the pancreatitis and no evidence of TTP-HUS when the pancreatitis was diagnosed. Two patients had severe ADAMTS13 deficiency with an inhibitor; in one of these patients TTP-HUS recurred following a subsequent recurrent episode of pancreatitis. The systematic review identified 16 additional patients who had TTP-HUS following pancreatitis; recurrent TTP-HUS occurred in three of these patients following a subsequent episode of recurrent pancreatitis. In all 21 patients, the interval between the diagnosis of pancreatitis and TTP-HUS was short (1–13 days; median, 3 days). The three Oklahoma patients represent approximately 1% of the 356 patients in the Registry.

Interpretation and Conclusions: These observations suggest that in some patients pancreatitis, a disorder that results in an intense systemic inflammatory response, may be a triggering event for acute episodes of TTP-HUS.

Key words: pancreatitis, thrombotic thrombocytopenic purpura, TTP, hemolytic uremic syndrome, HUS.