Case Presentation: A 59-year-old female with a history of type 2 diabetes, hypertension, dyslipidemia, bipolar disorder, and daily alcohol use, presented with acute onset epigastric abdominal pain radiating to the back, nausea and vomiting. Physical examination was notable only for epigastric tenderness. Pertinent labs include neutrophilic leukocytosis, elevated lipase (2353 units/L) and lactic acidosis (2.4 mmol/L). Computed tomography (CT) of the abdomen was notable for interstitial pancreatic edema and inflammatory changes, suggestive of acute pancreatitis. He was treated conservatively with bowel rest, intravenous hydration, and symptomatic treatment, with satisfactory symptomatic improvement. On hospital day 2, he developed fever, acute kidney injury stage II, thrombocytopenia, and hemolytic anemia. His platelets downtrended to a nadir of 42 K/mm3. A peripheral smear revealed schistocytes. Further imaging and cultures ruled out an infectious etiology. A negative serotonin release assay and elevated fibrinogen did not favor the diagnosis of disseminated intravascular coagulation or heparin induced thrombocytopenia. The diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was made; he was treated with four sessions of plasmapheresis, in addition to intravenous steroids and supportive blood transfusions, with subsequent normalization of platelet counts, and stabilization of renal function.
Discussion: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, fatal thrombotic microangiopathy with an estimated incidence of three cases per 1,000,000 adults per year. It can be triggered by various conditions such as sepsis, infections, and pregnancy. But, an inciting event is not usually identified in majority of the cases. In idiopathic acquired TTP, severe deficiency in ADAMTS13 protease results in accumulation of large vWF multimers. These multimers results in widespread formation of platelet microthrombi, with protean manifestations depending on the distribution of microthrombi. A high index of clinical suspicion should be maintained in a patient with hemolytic anemia and thrombocytopenia, despite the nature of presentation; timely diagnosis and treatment is paramount, given the high fatality rate. An ADAMTS13 activity assay confirms the diagnosis, but should not delay plasmapheresis, which is the mainstay of treatment.While pancreatic involvement in TTP is an established entity, acute pancreatitis as an inciting event of TTP is rare, with only a few reported cases in our literature review. Swisher et al, in his review of such cases, found a median interval of three days between the diagnosis of acute pancreatitis and TTP, corresponding to the timeline in this patient. Diffuse endothelial injury as a consequence of inflammatory cytokine release in association with the systemic inflammatory state observed with pancreatitis has been postulated to be the causative mechanism.
Conclusions: This case demonstrates the rare occurrence of TTP as a consequence of acute pancreatitis. Clinicians should consider TTP upon encountering thrombocytopenia and microangiopathic hemolytic anemia in systemic inflammatory states. Early initiation of plasmapheresis is key to the clinical outcome.
