ESPN 51th Annual Meeting

ESPN 2018


 
A RARE CAUSE OF ACQUIRED THROMBOTIC THROMBOCYTOPENIC PURPURA: ACUTE PANCREATITIS
NESLIHAN GÜNAY 1 AYŞE SEDA PINARBAŞI 1 İSMAIL DURSUN 1 SEFA ARMAĞAN GÖKÇELI 2 SIBEL YEL 1 MUAMMER HAKAN POYRAZOĞLU 1 RUHAN DÜŞÜNSEL 1

1- ERCIYES UNIVERSITY MEDICAL FACULTY, PEDIATRIC NEPHROLOGY, KAYSERI
2- ERCIYES UNIVERSITY MEDICAL FACULTY, PEDIATRICS DEPARTMENT, KAYSERI
 
Introduction:

 Thrombotic thrombocytopenic purpura (TTP) is a multisystemic disorder that caused by defect of large von Willebrand factor multimers cleaving protease ADAMTS13. TTP with acute pancreatitis has been rarely reported. Herein, we report a patient who presented with acute pancreatitis and acquired TTP and later developed pancreatic pseudocyst.

Material and methods:

 An 8-year-old girl presented with complaints of nausea, bilious vomiting for a week. On physical examination, paleness, dehydration, tachycardia and ascites in abdomen were detected. Her urine output was decreased during two days. Hemoglobin was 8.6 g /dl, platelet count 17000/mm3, reticulocyte percentage 3.6%, blood urea nitrogen 122.6 mg/dl, serum creatinine 4.6 mg/dl, amylase 728 U/L, lipase 952 U/L, LDH 1782 U/L, total bilirubin 1,24 mg/dL direct bilirubin 0.7mg/dl. Direct coombs were negative. Haptoglobulin level was low. ADAMTS 13 activity was 0.34% IU/mL(40-130%) and inhibitor level  was 20.7 U/mL (<12). The patient was considered as acquired TTP, with these findings. Plasmapheresis was performed, and steroid was given. In abdominal ultrasonography pancreas head was mildly edematous. In follow-up, the patients clinic and laboratory findings improved, and she was discharged.

Ten days after, she represented with abdominal pain. Her serum amylase and lipase values were increased again. In the repeated USG and computed tomography, a 46x37 mm pseudocyst appearance was observed at the pancreas head. Existing pseudocysts were drained by drainage catheter

Results:

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Conclusions:

 

The association between acute pancreatitis and TTP is unclear and it’s a rare condition. ADAMTS13 activity is reduced by inflammatory disease. An increase in inflammatory cytokines in patients with acute pancreatitis is considered as one of the causes of TTP. In cases who have TTP clinic with a significant high amylase and lipase, the possibility of acute pancreatitis should be consider as a cause of TTP, and should follow up for acute pancreatitis complication like pancreatic pseudocysts.