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lüll Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports Swisher KK; Doan JT; Vesely SK; Kwaan HC; Kim B; Lammle B; Kremer Hovinga JA; George JNHaematologica 2007[Jul]; 92 (7): 936-43BACKGROUND 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.|Adult[MESH]|Alcoholism/complications[MESH]|Cholestasis/complications[MESH]|Female[MESH]|Hemolytic-Uremic Syndrome/*etiology[MESH]|Humans[MESH]|Male[MESH]|Middle Aged[MESH]|Pancreatitis/*complications/etiology[MESH]|Purpura, Thrombotic Thrombocytopenic/*etiology[MESH]|Recurrence[MESH]|Registries[MESH]|Time Factors[MESH] |