INTRODUCTION: ADAMTS13 deficiency and hyperferritinemia (HF) are characteristic features of thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) respectively. Congenital thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman syndrome, is associated with an inherited deficiency of ADAMTS13, a von Willebrand factor-cleaving protease. Also, the majority of mice showed one or more submucosal colonic thrombi. In case of TTP suspicion, the measurement of plasma ADAMTS13 activity should be performed in first intention. We present a case of Capnocytophaga bacteremia in an asplenic patient with atypical findings of severe ADAMTS13 deficiency and hyperferritinemia. Several symptoms indicate an individual may have thrombotic thrombocytopenic purpura. Thrombotic thrombocytopenic purpura is diagnosed through numerous blood tests that reveal specific antibodies and activity levels of ADAMTS13. Congenital ADAMTS13 deficiency, also referred to as Upshaw-Schulman syndrome, is an autosomal-recessive disorder that is associated with ADAMTS13 activity levels below the level of detection of activity assays (ie, >10% for the assay used by LabCorp). ADAMTS13-/- mice had increased VWF release, with accumulation at inflamed colonic sites. However, patients with severe deficiency had a lower platelet count and either normal or minimally impaired renal function at presentation compared to those with an ADAMTS13 activity >10%. Conversely, patients with other non-TTP conditions may have a severe ADAMTS13 deficiency (< or =10%). However, the disease is associated with a deficiency of an enzyme involved in blood clotting called the von Willebrand factor cleaving protease (also called ADAMTS13). Mechanisms producing ADAMTS13 deficiency. ADAMTS13 deficiency worsened colitis and propagated intestinal inflammation, most likely through increased platelet-leukocyte recruitment by VWF. Treatment involves the use of steroid medications and plasma-like substances to replace the missing enzyme. While all patients with severe deficiency were diagnosed with autoimmune TTP, those without severe deficiency frequently had disseminated intravas- The measured ADAMTS13 activity may not reflect the true in vivo biological ADAMTS13 activity. Congenital thrombotic thrombocytopenic purpura (TTP), also known as Upshaw-Schulman syndrome, is associated with an inherited deficiency of ADAMTS13, a von Willebrand factor-cleaving protease. ADAMTS13 deficiency (activity ≤10%, N = 68) were more likely to be young, female and without a history of cancer treatment or transplantation. Severe deficiency of ADAMTS-13 (activity <5-10%) may be acquired or congenital, and is a relatively specific finding in patients with a clinical diagnosis of thrombotic thrombocytopenic purpura (TTP). If ADAMTS13 activity is lower than 10%, both assays for ADAMTS13 circulating inhibitor and anti-ADAMTS13 immunoglobulin (Ig)G are performed in second intention to search for an acquired severe ADAMTS13 deficiency. Not all patients with a clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura (TTP) have a severe ADAMTS13 deficiency. Neither neurological symptoms at presentation nor fever distinguished between severe deficiency and an ADAMTS13 activity >10%. The most frequent is that caused by circulating anti-ADAMTS13 autoantibodies that neutralize enzymatic activity , and/or accelerate protease removal from the circulation , , which produces the condition known as acquired TTP, accounting for 70-80% of … Two mechanisms leading to deficient ADAMTS13 activity have been identified in TTP patients.
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