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      Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study.

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          Abstract

          Among the syndromes characterised by thrombotic microangiopathy, thrombotic thrombocytopenic purpura is distinguished by a severe deficiency in the ADAMTS13 enzyme. Patients with this disorder need urgent treatment with plasma exchange. Because ADAMTS13 activity testing typically requires prolonged turnaround times and might be unavailable in resource-poor settings, a method to rapidly assess the likelihood of severe ADAMTS13 deficiency is needed.

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          Author and article information

          Journal
          Lancet Haematol
          The Lancet. Haematology
          Elsevier BV
          2352-3026
          2352-3026
          Mar 01 2017
          Affiliations
          [1 ] Division of Hematology, Massachusetts General Hospital, Boston, MA, USA; Blood Transfusion Service, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA. Electronic address: pkbendapudi@partners.org.
          [2 ] Center for Clinical Investigation, Brigham and Women's Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [3 ] Division of Hematology and Oncology, University of Alabama at Birmingham, Birmingham, AL, USA.
          [4 ] Department of Pathology, University of Alabama at Birmingham, Birmingham, AL, USA.
          [5 ] Department of Medicine, Veterans Affairs Eastern Colorado Health Care System, Denver, CO, USA.
          [6 ] Department of Medicine, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [7 ] Division of Hematology and Oncology, Beth Israel Deaconess Medical Center, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [8 ] Division of Hematology, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [9 ] Division of Hematology, Brigham and Women's Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [10 ] Department of Pathology, Brigham and Women's Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [11 ] Department of Pathology, Beth Israel Deaconess Medical Center, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [12 ] Blood Transfusion Service, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          [13 ] Division of Hematology, Massachusetts General Hospital, Boston, MA, USA; Blood Transfusion Service, Massachusetts General Hospital, Boston, MA, USA; Harvard Medical School, Boston, MA, USA.
          Article
          S2352-3026(17)30026-1
          10.1016/S2352-3026(17)30026-1
          28259520

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