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Atypical Hemolytic Uremic Syndrome (Ahus): Treating the Patient October 2013 Volume 11, Issue 10, Supplement 15 Atypical Hemolytic Uremic Syndrome (aHUS): Treating the Patient Plus: A Review of Case Studies Jeffrey Laurence, MD Professor of Medicine Division of Hematology and Medical Oncology New York Presbyterian Hospital and Weill Cornell Medical College New York, New York ON THE WEB: hematologyandoncology.net Indexed through the National Library of Medicine (PubMed/MEDLINE), PubMed Central (PMC), and EMBASE EDITORIAL ADVISORY BOARD Editor-in-Chief Howard A. Burris III, MD David S. Ettinger, MD Jeffrey C. Laurence, MD The Sarah Cannon The Sidney Kimmel New York Presbyterian Hospital Bruce D. Cheson, MD Cancer Center Comprehensive Cancer Center Weill Cornell Medical College Georgetown University Hospital at Johns Hopkins Lombardi Comprehensive Mitchell S. Cairo, MD John P. Leonard, MD Cancer Center New York Medical College Robert A. Figlin, MD Weill Medical College Cedars-Sinai Comprehensive of Cornell University Section Editors George P. Canellos, MD Cancer Center New York Presbyterian Hospital Dana-Farber Cancer Institute Oncology Harvard Medical School Charles Fuchs, MD, MPH Maurie Markman, MD Dana-Farber Cancer Institute Cancer Treatment Centers Clifford A. Hudis, MD Michael A. Carducci, MD of America Memorial Sloan-Kettering Cancer The Sidney Kimmel Daniel J. George, MD Center and Weill Medical College Comprehensive Cancer Center Duke University Medical Center John L. Marshall, MD of Cornell University at Johns Hopkins Georgetown University Andre Goy, MD Mark J. Ratain, MD Edward Chu, MD Hackensack University Kathy D. Miller, MD The University of Chicago University of Pittsburgh Medical Center Indiana University School of Medicine Hematologic Malignancies Bertrand Coiffier, MD William Gradishar, MD Hospices Civils de Lyon Northwestern University Ruth O’Regan, MD Susan O’Brien, MD Centre Hospitalier Lyon-Sud Winship Cancer Institute The University of Texas Richard J. Gralla, MD Emory University MD Anderson Cancer Center Jeffrey Crawford, MD North Shore University Hospital Duke University Long Island Jewish Medical Center Edith A. Perez, MD Hematology Medical Center Mayo Clinic, Jacksonville F. Anthony Greco, MD Craig M. Kessler, MD Myron S. Czuczman, MD The Sarah Cannon Cancer Center Marshall R. Posner, MD Georgetown University Roswell Park Cancer Institute Mount Sinai Medical Center Medical School SUNY at Buffalo, School of Medicine Stephanie A. Gregory, MD Lombardi Comprehensive Rush Medical College Paul G. Richardson, MD Cancer Center David C. 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College of Medicine ISSN: 1543-0790 Table of Contents Atypical Hemolytic Uremic Syndrome (aHUS): Treating the Patient Jeffrey Laurence, MD 4 Management of Atypical Hemolytic Uremic Syndrome (aHUS): A Review of Case Studies Jeffrey Laurence, MD 16 Clinical Advances in Hematology & Oncology Volume 11, Issue 10, Supplement 15 October 2013 3 REVIEW ARTICLE Atypical Hemolytic Uremic Syndrome (aHUS): Treating the Patient Jeffrey Laurence, MD Professor of Medicine Division of Hematology and Medical Oncology New York Presbyterian Hospital and Weill Cornell Medical College New York, New York Abstract: The 3 major thrombotic microangiopathies (TMAs) are thrombotic thrombocytopenic purpura (TTP), atypical hemolytic uremic syndrome (aHUS), and Shiga toxin–producing Escherichia coli (STEC)-HUS. These conditions are often clinically identical, but they require different treatment. The TMAs can be distinguished based on factors such as presenting platelet count and serum creatinine level, assessment of ADAMTS13 activity and inhibitor assays, and evaluation for STEC if diarrhea is present. aHUS is a chronic, genetic disorder that is based, in most cases, on an inability to regulate the alterna- tive complement pathway. In the absence of appropriate therapy, up to 50% of aHUS patients progress to end-stage renal disease within a year, and 25% die during the acute phase. Patients who present with clinical and laboratory evidence of a TMA should begin immediate treatment with plasma therapy, but there is no role for plasma infusion or plasma exchange in the long-term management of aHUS. Eculizumab is a potent inhibitor of the terminal complement pathway. It was recently approved by the US Food and Drug Administration for the treatment of patients with aHUS to inhibit complement- mediated thrombotic microangiopathy, including adverse effects on renal function. Eculizumab should be initiated as early as possible after making the diagnosis of aHUS, in order to attempt optimal recovery of renal function and prevent an ongoing TMA with extrarenal manifestations. Introduction is approximately 2 to 4 per million, and This review, the second of a 2-part STEC-HUS is recognized primarily in series on aHUS, briefly summarizes One year ago, I wrote a primer for distin- outbreaks of variable expression. Yet the how the different TMAs are diagnosed, guishing among the 3 major thrombotic high morbidity and mortality associated and then describes advances in the microangiopathies (TMAs): thrombotic with untreated TTP and inappropriately treatment of aHUS. thrombocytopenic purpura (TTP), treated aHUS, and the divergent treat- atypical hemolytic uremic syndrome ments they require, mandate 3 steps: Distinguishing Among the (aHUS), and Shiga toxin–producing • First, recognition of the existence of TMAs: Review of Current Escherichia coli (STEC)-HUS.1 These a TMA. Diagnostic Criteria conditions are often clinically identical. • Second, the ability to distinguish A further impediment to reaching an between aHUS and TTP. Initiation of appropriate treatment for accurate diagnosis is that they are ultra- • Third, rapid institution of appropri- aHUS requires an accurate diagnosis. rare. The incidence of TTP and aHUS ate therapy. Up to 50% of aHUS patients progress Disclaimer Funding for this supplement has been provided by Alexion Pharmaceuticals, Inc. Support of this supplement does not imply the supporter’s agreement with the views expressed herein. Every effort has been made to ensure that drug usage and other information are presented accurately; however, the ultimate responsibility rests with the prescribing physician. Millennium Medical Publishing, Inc, and the participants shall not be held responsible for errors or for any consequences arising from the use of information contained herein. Readers are strongly urged to consult any relevant primary literature. No claims or endorsements are made for any drug or compound at present under clinical investigation. ©2013 Millennium Medical Publishing, Inc., 611 Broadway, Suite 310, New York, NY 10012. Printed in the USA. All rights reserved, including the right of reproduction, in whole or in part, in any form. 4 Clinical Advances in Hematology & Oncology Volume 11, Issue 10, Supplement 15 October 2013 ATYPICAL HEMOLYTIC UREMIC SYNDROME (AHUS): TREATING THE PATIENT to end-stage renal disease (ESRD) odds ratio for aHUS vs TTP is 23.4 in diarrhea, particularly bloody diarrhea, within a year, and 25% die during the the setting of a platelet count exceeding is a classic sign of STEC-HUS. How- acute phase in the absence of appro- 30,000/mm3.4 Near normal platelet ever, up to one-third of aHUS cases priate therapy.2 In this context, it is counts occur
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