{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:09:55Z","timestamp":1772755795242,"version":"3.50.1"},"reference-count":17,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2014,5,20]],"date-time":"2014-05-20T00:00:00Z","timestamp":1400544000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Transfusion"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Since there are many disorders that can present with thrombotic microangiopathy (TMA), establishing a correct diagnosis is important to offer the most appropriate therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Report<\/jats:title><jats:p>A 26\u2010year\u2010old woman was transferred to our hospital with fragmentation hemolytic anemia, thrombocytopenia, and acute kidney failure. History revealed that she was recently diagnosed with adult\u2010onset Still's disease (<jats:styled-content style=\"fixed-case\">AOSD<\/jats:styled-content>) and received intraocular injections of bevacizumab to treat acute retinal artery occlusion. At our hospital, she underwent extensive investigations and was treated with high\u2010dose steroids, hemodialysis, and therapeutic plasma exchange. For recurrent disease, she received a single dose of eculizumab.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The patient's <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>13 activity was normal and she had evidence of complement activation. Genetic testing identified a benign polymorphism in the <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>3 gene. Pathophysiology of TMA in <jats:styled-content style=\"fixed-case\">AOSD<\/jats:styled-content> is briefly discussed and an overview of the literature is presented.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Work\u2010up of a new fragmentation hemolytic anemia and thrombocytopenia should include careful review of past history, including medications, as well as relevant laboratory investigations with aim to establish a correct diagnosis. Occasionally, the correct diagnosis is not the obvious one and there could be multiple contributors to the pathogenesis. Establishing diagnosis is important for counseling patient on disease prognosis and to guide treatment.<\/jats:p><\/jats:sec>","DOI":"10.1111\/trf.12708","type":"journal-article","created":{"date-parts":[[2014,5,21]],"date-time":"2014-05-21T03:07:07Z","timestamp":1400641627000},"page":"2983-2987","source":"Crossref","is-referenced-by-count":8,"title":["Thrombotic microangiopathy in a patient with adult\u2010onset <scp>S<\/scp>till's disease"],"prefix":"10.1111","volume":"54","author":[{"given":"Shail","family":"Rawal","sequence":"first","affiliation":[{"name":"Department of Medicine University of Toronto  Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yael","family":"Einbinder","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Hypertension Meir Medical Center  Kfar Saba 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