Plasmapheresis for the treatment of acute pancreatitis induced by hemophagocytic syndrome related to hypertriglyceridemia.

Histiocytic cytophagic panniculitis is an unusual form of hemophagocytic syndrome related to T-cell lymphoma that can be responsible for hypertriglyceridemia. The elevation of serum triglycerides, usually in the setting of familial lipidemia or during uncontrolled diabetes mellitus, is a well-recognized cause of pancreatitis. We report here on the treatment by plasmapheresis of a patient suffering from a T-cell lymphoma-related panniculitis who presented with an acute pancreatitis induced by hypertriglyceridemia. The Ranson scoring system at day 2 was increased and predicted a poor course while clinical features worsened. The patient was treated with one plasmapheresis that allowed a dramatic (89%) decrease in the triglycerides level. The acute pancreatitis resolved and the patient was discharged from the intensive care unit at day 5 with lipids and pancreatic enzyme levels within normal range. To our knowledge, this is the first case of acute pancreatitis induced by hypertriglyceridemia in the setting of an uncontrolled cytophagic histiocytic panniculitis successfully treated by plasmapheresis. Copyright 2003 Wiley-Liss, Inc.