Alcohol Induced Severe Pancreatitis Causing Thrombotic Thrombocytopenic Purpura

Introduction:
Thrombotic thrombocytopenic purpura (TTP) is a very rare complication of acute pancreatitis. Acute pancreatitis can develop from TTP, but TTP as a consequence of pancreatitis is extremely rare. Congenital TTP occurs due to the absence or severe deficiency of ADAMTS13 activity by mutations in the ADAMTS13 gene while an acquired form is thought to be caused by auto-antibodies to the same enzyme. Secondary TTP has also been known to occur in association with bone marrow transplant, malignancy, drugs and collagen disease. In rare occasions, acute pancreatitis can also lead to TTP. Here we present the case of a young female patient who developed TTP due to recurrent alcoholic pancreatitis.
Methods:
A 23-year-old female with past medical history significant for alcohol abuse presented with 5-6 episodes of non-bloody yellow emesis and progressively worsening severe right upper quadrant abdominal pain. Lipase was 1465 U/L. CT of the abdomen/pelvis was significant for pancreatic inflammation. On day 3, the patient appeared increasingly jaundiced with worsening symptoms. Findings were suspicious for microangiopathic hemolytic anemia as hemoglobin was decreased to 5.6 g/dl, platelets were significantly decreased to 19 K/uL, creatinine was elevated to 1.9, haptoglobin was Results:
Swisher et al., reported 5 patients and reviewed 16 cases from the literature, in which it is noticed that acute pancreatitis preceded clinical and laboratory signs of TTP by a median of 3‚Äâdays. The likely mechanism involves diffuse endothelial injury mediated by inflammatory cytokines that are released as part of the systemic inflammatory response to acute pancreatitis. Decrease in nitric oxide (NO) level predisposes the patient to thrombotic microangiopathy. In in vitro studies, it is proved that inflammatory cytokines stimulated endothelial cell release of ultra large vWF multimers and inhibited the cleavage of ultra large vWF by ADAMTS13. In acute pancreatitis, endothelial damage is found to be associated with a decrease in endothelial NO synthase production. In rat models, this decrease in NO synthase has been found to be an important mediator in the pathogenesis of acute pancreatitis.These studies strongly favor the lack of NO as being one of the significant contributors to AP-induced TTP rather than just the lack of ADAMTS-13.
Discussion:
In patients with acute pancreatitis, any sudden drop in hemoglobin or platelet count should raise the clinical suspicion of TTP, especially with evidence of schistocytes in peripheral blood smear. Diverse mechanisms apart from ADAMTS-13 deficiency may be involved. Prompt diagnosis is required to start early treatment with plasmapheresis which brings a favorable outcome.