Thrombotic Thrombocytopenic Purpura Triggered by Inflammatory Pseudotumor: A Report of a Rare Case

Abstract 4660 Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by microangiopathic anemia, thrombocytopenia and neurological or kidney damage. TTP occurs due to the deficiency of ADAMTS13, mainly because of autoantibodies against the enzyme. Treatment requires combination of plasmapheresis and steroids. Underline diseases must be ruled out and treated when present. Although well established in the literature, diagnosis and clinical management can be difficult because of the rarity of the disease. Case report: J.C.S., male, 41 years old, previously healthy. In March 2012 he developed malaise, abdominal pain, jaundice, fever, headache and hematuria, being initially treated as having leptospirosis. During the clinical course, he presented renal failure requiring dialysis, transient ischemic attack and thrombocytopenia. Tests showed microangiopathic hemolytic anemia, thrombocytopenia ( Conclusion: In this report, we showed a case of TTP caused by a primary and localized inflammatory pseudotumor. This tumor probably triggered the formation of autoantibodies against ADAMTS 13, leading to the picture of TTP. The evidence that the patient had sustained clinical remission and normalization of ADAMTS 13 only after tumor removal supports this hypothesis. Many cases of TTP triggered by systemic infectious, autoimmune diseases or metastatic neoplasia have been reported before. However, of our knowledge, there are no published cases of TTP secondary to a localized and benign disease so far. This report highlights the importance of a comprehensive etiological investigation in cases of TTP, the role of plasmapheresis as adjuvant therapy and the need for specific treatment of the underlying disease. Disclosures: No relevant conflicts of interest to declare.