Thrombotic Thrombocytopenic Purpura INDUCED PANCREATITIS– A Rare Complication

Abstract 4683

Thrombotic Thrombocytopenic Purpura (TTP) is a rare clinical disorder characterized by clinical combination of fever, microangiopathic hemolytic anemia, thrombocytopenia, renal failure and neurological changes.

Pancreatitis may occur as a result of TTP or may rarely trigger TTP. We present a case of acute Pancreatitis as a complication of TTP.

A 29 year-old African-American female with history of alcohol abuse presented with three days of nausea, vomiting, diarrhea, headache and fever. Vitals: BP-120/80mmHg, HR-75/min, Temp-98F, and RR-14/min. Examination was remarkable for lethargy and mild epigastric tenderness. Pertinent laboratory: Hgb-12.4g/dL (Day1) - 8.5g/dL (Day3), reticulocyte count-3.35, Platelets-13,000mm³, BUN/Cr-69/6.3 (mg/dL), LDH-1523U/L, indirect hyperbilrubinemia-3.6mg/dL, Amylase/Lipase-117/87(U/L), fibrinogen-462, D-dimer-15.39, PT/PTT-normal and Haptoglobulin<8mg/dL. Peripheral smear showed 3+ schistocytes. Imaging: CXR, CT Head and CT Abdomen were unremarkable. Work-up for various infections and autoimmune diseases was negative.

TTP was diagnosed and patient was admitted to ICU. Plasmapheresis was initiated with clinical improvement by day 3 with subsequent deterioration by day 7. On day 10, patient developed severe abdominal pain with increase in Amylase (145U/L) and lipase (281U/L). Repeat CT Abdomen showed acute pancreatitis. Day 13 - Rapid worsening in clinical status with death of the patient. Autopsy revealed micothrombi and infarcts in multiple organs including kidney, pancreas, and heart.

Acute Pancreatitis is a rare complication of TTP suggested to occur in 2% of cases of TTP but no case has ever been reported in the literature. There are cases that have been reported where Acute Pancreatitis have triggered TTP-HUS syndrome and chronic relapsing TTP. Idiopathic cases of TTP have a deficiency of ADAMS 13, a metalloprotease enzyme that cleaves von Willebrand factor (vWF) resulting in abnormal vWF multimers causing hyaline thrombi formation with end-organ damage due to ischemia. Pancreatic inflammation in TTP has been regarded as ischemic in nature, due to microthrombi of pancreatic small vessels. It is common to see the inflammation of pancreas in the histological specimens on autopsy, but very rare to develop pancreatitis with clinical symptoms, increase in pancreatic enzymes and CT findings of Pancreatitis. Prognosis of Patients with Acute Pancreatitis in TTP is guarded. Patient should be closely monitored in ICU setting aiming to control TTP by Plasmapheresis and Immunosuppressive therapy (Rituximab) in refractory cases.

Acute Pancreatitis is a rare complication of TTP and carries a worse prognosis.

In patients developing abdominal pain during the course of TTP, Acute Pancreatitis should always be considered in the differential diagnosis.