Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are uncommon diseases that frequently overlap their features, together having an incidence of three to seven cases per million. Pancreatitis may be, in some cases, part of both clinical presentations. The onset of pancreatitis followed by manifestations of these diseases has few reports in the literature.
We report the case of a 27-year-old man who presented to the emergency department of our hospital with 2 days of epigastric pain associated with nausea and vomiting, mild jaundice and moderate dehydration. He had no fever and his physical examination was unremarkable except for a light tenderness of the epigastric area and abdominal distention. An initial amylase level of 612 U/l, lipase of 2678 U/l together with a leukocyte count of 12,000 were the only altered laboratory data. The abdominal sonography showed a heterogeneous pancreas, with a slight increase in its size, and a lightly distended gallbladder without any signs of gallstones. We also performed an abdominal CT, where there was an undefined and irregular contour of the pancreas, increased density of adjacent plans, a few liquid collections within and bilateral pleural effusion. A diagnosis of pancreatitis was made and 2 days after his admission the patient had complete clinical relapse and was about to start an oral diet again. Nevertheless his laboratory data showed progressive thrombocytopenia with a fall from 333,000 to 9000 platelets and a rise on LDH from 363 to 5711, together with a progressive loss of renal function (peak creatinine, 3.8 mg/dl). He became oliguric but had no need for renal replacement therapy; he also presented light drowsiness and confusion. After the confirmation of erythrocyte fragmentation in a peripheral blood smear, we established a diagnosis of thrombotic thrombocytopenic purpura and started plasmapheresis daily for 6 days until his platelets reached 150,000. After that he had four more sessions on alternate days and was discharged from hospital with a platelet count of 269,000, without any neurologic or renal impairment. He has been followed in the hematologic department of our hospital and has so far maintained remission (3 months).