Thrombotic thrombocytopenic purpura associated with severe acute pancreatitis in a context of decreased ADAMTS13 activity: a case report

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystemic microvascular disease defined by the association of hemolytic anemia, thrombocytopenia, acute renal failure, fever, and neurological disorders. The pathophysiology has recently been elucidated by the discovery of a von Willebrand factor-cleaving protease (ADAMTS13) deficiency involved in platelet aggregation and ischemia. The association between TTP and acute pancreatitis (AP) has rarely been reported, described either as a cause or a consequence. The role of ADAMTS13 during AP is still unknown. We describe the case of a 41-year-old woman who developed a TTP, with decreased ADAMTS13 activity, associated with severe AP. Published cases of thrombotic microangiopathy associated with AP are reviewed. The pathophysiology, management, prognostic factors, and rationale for treatment are discussed. AP should be sought in patients with TTP presenting with abdominal pain. On the other hand, TTP should be considered in patients with AP and thrombocytopenia.

Long‐term remission of recurrent thrombotic thrombocytopenic purpura (TTP) after Rituximab in children and young adults

“…Low VWCP activity seems to predict poor outcome in these patients . In most reported cases, TTP occurred in a median of 3 (1–15) days after acute pancreatitis suggesting that it may be a trigger for TTP, like surgery, infection or pregnancy . In all reported patients, remission was achieved with PEX .…”

“…Patient 7 suffered from TTP associated with pancreatitis. Autoimmune pancreatitis is the reason for 6% of chronic pancreatitis . Low VWCP activity seems to predict poor outcome in these patients .…”

Silent ischemic pancreatitis following urosepsis

“…Shock, vascular occlusion in mesenteric vessels, cardiac arrest and cardiovascular surgery may lead acute ischemic pancreatitis (Gullo et al, 1996;Sakorafas et al, 2000;Piton et al, 2008;Drissi et al, 2009;Hackert et al, 2009;Piton et al, 2010). Ischemic AP frequency is estimated between 5% to 8% in literature (Warshaw and O'Hara, 1978;Sakorafas et al, 2000).…”