{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T03:01:24Z","timestamp":1761102084994},"reference-count":20,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,2,20]],"date-time":"2014-02-20T00:00:00Z","timestamp":1392854400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2014,6]]},"abstract":"Abstract<\/jats:title>Objectives<\/jats:title>Thrombotic thrombocytopenic purpura (TTP<\/jats:styled-content>) is a rare and devastating hematologic disorder frequently associated with multiple organ failure and sometimes death. This syndrome is mainly associated with severe deficiency of ADAMTS<\/jats:styled-content>13, a disintegrin and metalloprotease with thrombospondin (TSP<\/jats:styled-content>)\u20101 repeats, cleaving high molecular weight von Willebrand Factor (ULVWF<\/jats:styled-content>) multimers. Decreased plasma ADAMTS<\/jats:styled-content>13 activity results in the accumulation of ULVWF<\/jats:styled-content> multimers with consequent platelet activation. Recently, obesity has been considered as a potential independent risk factor for TTP<\/jats:styled-content>, but the reason of this association is still unknown.<\/jats:p><\/jats:sec>Methods and results<\/jats:title>We describe an unusual case of fatal recurrent TTP<\/jats:styled-content> in a morbid obese female with non\u2010alcoholic steatohepatitis (NASH<\/jats:styled-content>) and severe ADAMTS<\/jats:styled-content>13 activity deficiency due neither to an inhibitory autoantibody nor to a gene mutation.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>Visceral obesity is associated with non\u2010alcoholic fatty liver disease (NAFLD<\/jats:styled-content>) and NASH<\/jats:styled-content>: we hypothesized that these conditions can influence ADAMTS<\/jats:styled-content>13 antigen and activity. In fact, hepatic stellate cells (HSC<\/jats:styled-content>) are the main producers of ADAMTS<\/jats:styled-content>13, and a decrease in ADAMTS<\/jats:styled-content>13 activity has been reported in liver disease.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12273","type":"journal-article","created":{"date-parts":[[2014,1,27]],"date-time":"2014-01-27T11:24:07Z","timestamp":1390821847000},"page":"497-501","source":"Crossref","is-referenced-by-count":6,"title":["Defective ADAMTS<\/scp>13 synthesis as a possible consequence of NASH<\/scp> in an obese patient with recurrent thrombotic thrombocytopenic purpura"],"prefix":"10.1111","volume":"92","author":[{"given":"Anna Maria","family":"Lombardi","sequence":"first","affiliation":[{"name":"Internal Medicine Department of Medicine University of Padova Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberto","family":"Fabris","sequence":"additional","affiliation":[{"name":"Metabolic Diseases and Cardiovascular Risk 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