A Rare Case of Thrombotic Thrombocytopenic Purpura Presenting With Pancreatitis. Issue 6 (22nd October 2015)

• Record Type: Journal Article
• Authors: Gurevitz, Stacy A.; Zorinsky, David
• Is Part Of: Laboratory medicine. Volume 42:Issue 6(2011)
• Page Start: 329
• Page End: 331
• Publication Date: 2015-10-22
• Subjects: thrombotic thrombocytopenic purpura -- pancreatitis -- ADAMTS13 -- von Willebrand factor -- microangiopathic hemolytic anemia -- schistocytes -- spherocytes; Clinical chemistry -- Periodicals; Pathology -- Periodicals; Medical technology -- Periodicals; Chemistry, Clinical; Pathology; Medical Laboratory Science; Clinical chemistry; Medical technology; Pathology; Electronic journals; Periodicals; Periodicals; 616
• DOI: 10.1309/LM2AS2HVKC6GGVPQ
• Languages: English
• ISSNs: 0007-5027

Abstract

Abstract : Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by platelet consumption in thromboses, leading to a microangiopathic hemolytic anemia (MAHA). Thrombotic thrombocytopenic purpura involves a decrease in the activity of a metalloproteinase, ADAMTS13, which is critical in regulation of the coagulation cascade. This occurs in several conditions, and therefore, TTP is associated with a number of diseases. The decreased activity of ADAMTS13 leads to occlusive platelet thrombi, which can result in a variety of tissue manifestations. Patients normally present with nonspecific symptoms, such as fever and petechia. The classic pentad, although now extraordinarily uncommon, includes thrombocytopenia, MAHA, varying degrees of renal failure, fever, and nonlocalizable neurologic symptoms. We present a rare case in which pancreatitis was the presenting condition in a patient with acute idiopathic TTP. We discuss the pathogenesis and etiologies of these 2 diseases.