Lessons from acute pancreatitis-induced thrombotic thrombocytopenic purpura

Acute pancreatitis is an inflammatory disease characterized by local tissue injury which can trigger a systemic inflammatory response. There is increasing evidence that endothelial dysfunction is one of the critical pathophysiologic manifestations in patients with severe form of acute pancreatitis. In keeping with this, there have been recent reports of a haematological disorder, thrombotic thrombocytopenic purpura (TTP), as being precipitated by acute pancreatitis. However, the patients who develop TTP, secondary to acute pancreatitis, do not always have the characteristic low levels of VonWillebrand multimer cleaving enzyme, ADAMTS-13 suggesting the involvement of other pathophysiological factors. On the contrary, the occurrence of acute pancreatitis in haemolytic diseases may suggest TTP as being a precipitating factor for the pancreatic inflammatory state. This review focuses on the association of these two conditions which have given insights into the role players and the pathogenic mechanisms leading to the development of either of these conditions.