Successful Treatment of the Postpartum Atypical Hemolytic Uremic Syndrome With Eculizumab
Authors: Stefan Zschiedrich, Eric P. Prager, E. Wolfgang Kuehn
Journal: Annals of Internal Medicine (2013)

LettersJuly 2, 2013Successful Treatment of the Postpartum Atypical Hemolytic Uremic Syndrome With EculizumabStefan Zschiedrich, MD, Eric P. Prager, MD, and E. Wolfgang Kuehn, MDStefan Zschiedrich, MDFrom University Hospital Freiburg, Freiburg, Germany., Eric P. Prager, MDFrom University Hospital Freiburg, Freiburg, Germany., and E. Wolfgang Kuehn, MDFrom University Hospital Freiburg, Freiburg, Germany.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-159-1-201307020-00023 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Thrombocytopenia with intravascular hemolysis is a serious complication of pregnancy. When it occurs in the second and third trimesters, it is usually associated with near-absent levels of the ADAMTS13 protease that cleaves von Willebrand factor, a condition that is typical of idiopathic thrombotic thrombocytopenic purpura (1). During pregnancy, approximately 50% of cases of thrombotic thrombocytopenic purpura result in perinatal fetal loss and maternal mortality is also substantial, although plasma exchange has reduced maternal mortality from 58% before 1980 to 9% after 1996 (1). In contrast, when thrombocytopenia with intravascular hemolysis occurs in the postpartum period, ADAMTS13 activity is normal, ...References1. Martin JN, Bailey AP, Rehberg JF, Owens MT, Keiser SD, and May WL. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955–2006. Am J Obstet Gynecol. 2008;199:98-104. [PMID: 18456236] CrossrefMedlineGoogle Scholar2. Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L, et al. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21:859-67. [PMID: 20203157] CrossrefMedlineGoogle Scholar3. Zuber J, Le Quintrec M, Krid S, Bertoye C, Gueutin V, Lahoche A, et al. French Study Group for Atypical HUS. Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant. 2012;12:3337-54. [PMID: 22958221] CrossrefMedlineGoogle Scholar4. Nürnberger J, Philipp T, Witzke O, Opazo Saez A, Vester U, Baba HA, et al. Eculizumab for atypical hemolytic-uremic syndrome [Letter]. N Engl J Med. 2009;360:542-4. [PMID: 19179328] CrossrefMedlineGoogle Scholar5. Menne J, Nitschke M, Stingele R, Abu-Tair M, Beneke J, Bramstedt J, et al. EHEC-HUS consortium. Validation of treatment strategies for enterohaemorrhagic Escherichia coli O104:H4 induced haemolytic uraemic syndrome: case-control study. BMJ. 2012;345:4565. [PMID: 22815429] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From University Hospital Freiburg, Freiburg, Germany.Disclosures: Other (money to institution): Alexion Pharmaceuticals. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics July 2, 2013Volume 159, Issue 1Page: 76KeywordsBlood plasmaComplement activationCreatinineDeliriumEscherichia coliFibrinogenHemoglobinHemolytic-uremic syndromeHemorrhageHypertensionMaternal mortalityMedical dialysisMutationPlateletsPregnancyProteasesProteinsRenal failureResearch laboratoriesThrombotic thrombocytopenic purpura ePublished: 2 July 2013 Issue Published: July 2, 2013 Copyright & PermissionsCopyright © 2013 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...