Moschcowitz Syndrome – A Rare Conundrum with Acute Pancreatitis – Case Report and Narrative Review of Literature

Abstract

Introduction: Moschcowitz syndrome or Thrombotic Thrombocytopenic Purpura (TTP) is a thrombogenic condition due to deficiency or dysfunction of A disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), which causes a myriad of clinical presentations. The association between acute pancreatitis and TTP is a conundrum as both can be etiology and effect of one another. We describe a clinical case and review the available literature to understand this conundrum better. Case: A 52-year-old male was admitted to our setting with acute pancreatitis and on further evaluation was found to have deranged renal function with anemia and thrombocytopenia. He had multiple seizures during his stay requiring mechanical ventilation. Based on clinical findings a diagnosis of TTP was suspected which was confirmed on relevant investigations. His course and management were a challenge for the team. Discussion: A thorough literature search was conducted to identify all published reports/case series on acute pancreatitis and TTP. Of 38 identified publications, 20 case reports were reviewed as a cohort of acute pancreatitis and TTP. All cases had acute pancreatitis before TTP, alcohol being the most common cause. Early diagnosis and initiation of plasma exchange improves prognosis. Conclusion: Acute pancreatitis is a sinister trigger for TTP. Vigilance, suspicion, and early diagnosis are crucial to manage these cases. Early plasma exchange reduces mortality and hospital stay.

Keywords

• thrombotic thrombocytopenic purpura
• ADAMTS13 deficiency
• acute pancreatitis
• plasmapheresis