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      Thrombotic Microangiopathy Presenting with Intestinal Involvement Following Long-term Interferon-β1b Treatment for Multiple Sclerosis

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          Thrombotic microangiopathies (TMAs) are systemic microvascular occlusive disorders. The present report describes a patient with relapsing-remitting multiple sclerosis who had been treated with interferon (IFN)-β1b therapy for eight years and developed TMA. The patient presented with headache, thrombocytopenia, renal dysfunction, severe hypertension, posterior reversible encephalopathy syndrome, and gastrointestinal involvement. After discontinuation of the medication and initiation of antihypertensive treatment, the patient rapidly improved. This is the first report of TMA with gastrointestinal involvement (intestinal TMA) induced by IFN-β. The new onset of hypertension or headache requires careful attention in cases of long-term administration of IFN-β1b.

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          Most cited references 12

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          Syndromes of thrombotic microangiopathy.

          This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.
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            Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Seventh Special Issue.

            The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maintain this methodology and rigor to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Seventh Edition, like its predecessor, has consistently applied the category and grading system definitions in the fact sheets. The general layout and concept of a fact sheet that was used since the fourth edition has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. The Seventh Edition discusses 87 fact sheets (14 new fact sheets since the Sixth Edition) for therapeutic apheresis diseases and medical conditions, with 179 indications, which are separately graded and categorized within the listed fact sheets. Several diseases that are Category IV which have been described in detail in previous editions and do not have significant new evidence since the last publication are summarized in a separate table. The Seventh Edition of the JCA Special Issue serves as a key resource that guides the utilization of therapeutic apheresis in the treatment of human disease. J. Clin. Apheresis 31:149-162, 2016. © 2016 Wiley Periodicals, Inc.
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              Posterior reversible encephalopathy syndrome: prognostic utility of quantitative diffusion-weighted MR images.

              The recently described posterior reversible encephalopathy syndrome (PRES) classically consists of reversible vasogenic edema in the posterior circulation territories, although conversion to irreversible cytotoxic edema has been described. We hypothesized that the extent of edema has prognostic implications and that diffusion-weighted MR imaging (DWI) can help predict the progression to infarction. Twenty-two patients with PRES and 18 control subjects were examined with isotropic DWI. Nineteen regions of interest (ROIs) were systematically placed, and apparent diffusion coefficients (ADCs) were computed and correlated with T2 and DWI signal intensity in each ROI. T2 signal abnormalities were always present in territories of the posterior circulation. Anterior circulation structures were involved in 91% of patients. ADC values in areas of abnormal T2 signal were high. More extensive T2 signal abnormalities were seen in patients with a poor outcome than in patients who recovered. In six patients (27%), areas of high DWI signal intensity were seen with ADC values that were paradoxically normal, which we called pseudonormalized. Abnormal T2 signal intensity and high ADC values surrounded these areas. Follow-up images in two patients showed progression to infarction in pseudonormalized regions. Vasogenic edema in PRES involves predominantly the posterior circulation territories, but anterior circulation structures are also frequently involved. The extent of combined T2 and DWI signal abnormalities correlate with patient outcome. High DWI signal intensity and pseudonormalized ADC values are associated with cerebral infarction and may represent the earliest sign of nonreversibility as severe vasogenic edema progresses to cytotoxic edema.

                Author and article information

                Intern Med
                Intern. Med
                Internal Medicine
                The Japanese Society of Internal Medicine
                20 November 2017
                1 March 2018
                : 57
                : 5
                : 741-744
                [1 ]Department of Neurology, The Jikei University School of Medicine, Japan
                Author notes

                Correspondence to Dr. Shusaku Omoto, s-omoto@ 123456jikei.ac.jp

                Copyright © 2018 by The Japanese Society of Internal Medicine

                The Internal Medicine is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit ( https://creativecommons.org/licenses/by-nc-nd/4.0/).

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