A Rapidly Progressive Case of Hemorrhagic Pancreatitis in a Patient with Polycythemia Vera, or Not?

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Introduction: Acute pancreatitis and polycythemia vera are risk factors for mesenteric vein thrombosis. Rates of significant thrombosis and hemorrhage are reported as approximately 25% for either in patients with PCV. The case describes the difficult dilemma that occurs when clinical presentation includes both thrombo-hemorrhagic complications simultaneously. Case: A 62-year-old woman with a history of polycythemia vera was admitted for severe upper abdominal pain, nausea, vomiting and CT findings consistent with acute pancreatitis and small pelvic hematoma. She had no other risk factors for pancreatitis such as gallstones, alcohol use, hypertriglyceridemia, drugs, or autoimmune phenomena. Admission laboratory evaluation was notable for white blood cell count of 50,000, platelet count of 2.4 million, and a normal lactate. Interestingly, amylase and lipase were also normal as well as CT abdomen/pelvis obtained 3 days prior. Conservative management for pancreatitis was initiated. Unfortunately, the patient deteriorated over the next 2 days, with worsening pain and peritoneal signs. A repeat CT abdomen/pelvis showed a peripancreatic hematoma compressing the portal vein, progressive pelvic hematoma, superior mesenteric vein thrombus, and new ileus. She was taken emergently to the operating room for evacuation of peripancreatic hematoma, oversewing of bleeding vessels in right retroperitoneum, and packing of abdomen with temporary closure. Plasma exchange (PLEX) and hydroxyurea were initiated for cytoreduction of the dysfunctional platelet load in hopes of preventing further bleeding or thrombotic complications. Platelets improved to 1 million. Anticoagulation was held despite her prothrombotic state and mesenteric thrombosis in the setting of large retroperitoneal hematoma. Discussion: The diagnostic criteria for pancreatitis include two of the following: characteristic abdominal pain, elevated pancreatic enzymes, or imaging consistent with the diagnosis. Our patient met the criteria without an elevated amylase or lipase. However, upon review of the clinical picture and laparotomy findings, the peripancreatic changes were not consistent with acute pancreatitis. Instead, local hemorrhage and inflammatory changes related to the disease process of polycythemia vera were mimicking pancreatitis. In evaluating patients with presumed pancreatitis special attention should be paid to the overall clinical picture to avoid overdiagnosis. Similarly, early recognition of alternative diagnoses would allow prompt institution of appropriate therapy.