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


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.


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.
Swisher
Karen K
KK
Hematology-Oncology Section, Department of Medicine, College of Medicine, The University of Oklahoma Health Science Center, Oklahoma City, OK 73190, USA.
Doan
John T
JT
Vesely
Sara K
SK
Kwaan
Hau C
HC
Kim
Benjamin
B
Lämmle
Bernhard
B
Kremer Hovinga
Johanna A
JA
George
James N
JN
D000328:Adult; D000437:Alcoholism / Q000150:complications; D002779:Cholestasis / Q000150:complications; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000150:complications* / Q000209:etiology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology*; D012008:Recurrence; D012042:Registries; D013997:Time Factors