Plasma Exchange for Hemolytic Crisis in Wilson Disease
Authors: Akira Matsumura, Hideyuki Hiraishi, Akira Terano
Journal: Annals of Internal Medicine (1999)

Letters7 December 1999Plasma Exchange for Hemolytic Crisis in Wilson DiseaseAkira Matsumura, MD, Hideyuki Hiraishi, MD, and Akira Terano, MDAkira Matsumura, MDDokkyo University School of Medicine; Tochigi 321-0293, Japan (Matsumura)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Hiraishi)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Terano), Hideyuki Hiraishi, MDDokkyo University School of Medicine; Tochigi 321-0293, Japan (Matsumura)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Hiraishi)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Terano), and Akira Terano, MDDokkyo University School of Medicine; Tochigi 321-0293, Japan (Matsumura)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Hiraishi)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Terano)Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-131-11-199912070-00018 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:Half of patients with Wilson disease, a disorder of copper metabolism with two mutant ATP7B genes (1), present with hepatic disturbance (2). Acute hepatic failure tends to be fulminant when it is associated with hemolysis (3); patients can survive for only days or weeks unless transplantation is performed (2). We describe a patient with Wilson disease whose hemolysis was treated with plasma exchange.A 17-year-old boy was hospitalized because of fatigue. He was alert and slightly jaundiced. Laboratory findings included a hemoglobin level of 11.9 g/dL, an albumin level of 3.1 g/dL, an aspartate aminotransferase level of ...References1. Bull PC, Thomas GR, Rommens JM, Forbes JR, Cox DW. The Wilson disease gene is a putative copper transporting P-type ATPase similar to the Menkes gene. Nat Genet. 1993;5:327-37. CrossrefMedlineGoogle Scholar2. McCullough AJ, Fleming CR, Thistle JL, Baldus WP, Ludwig J, McCall JT, et al . Diagnosis of Wilson's disease presenting as fulminant hepatic failure. Gastroenterology. 1983;84:161-7. CrossrefMedlineGoogle Scholar3. Bellary S, Hassanein T, Van Thiel DH. Liver transplantation for Wilson's disease. J Hepatol. 1995;23:373-81. CrossrefMedlineGoogle Scholar4. Stremmel W, Meyerrose KW, Niederau C, Hefter H, Kreuzpaintner G, Strohmeyer G. Wilson disease: clinical presentation, treatment, and survival. Ann Intern Med. 1991;115:720-6. LinkGoogle Scholar5. Kiss JE, Berman D, Van Thiel D. Effective removal of copper by plasma exchange in fulminant Wilson's disease. Transfusion. 1998;38:327-31. CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAuthors: Akira Matsumura, MD; Hideyuki Hiraishi, MD; Akira Terano, MDAffiliations: Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Matsumura)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Hiraishi)Dokkyo University School of Medicine; Tochigi 321-0293, Japan (Terano) PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byEmergencies in paediatric hepatologyDiagnosis and management of fulminant Wilson's disease: a single center's experienceHematopoietic Abnormalities and HemostasisWilson's DiseasePlasmapheresis for hemolytic crisis and impending acute liver failure in Wilson diseaseTherapeutic plasmapheresis as a bridge to liver transplantation in fulminant Wilson diseaseSuccessful Plasma Exchange Treatment In Hemolytic Crisis Of Wilson'S Disease Preventing Liver TransplantationHematopoietic Abnormalities and HemostasisOxidant Stress and Haemolysis of the Human ErythrocyteRole of Intensive PEX in a Patient with Fulminant Hepatic Failure due to Wilson's Disease (WD) in Preparation for Orthotopic Liver Transplantation (OLT) 7 December 1999Volume 131, Issue 11Page: 866KeywordsAcute liver failureBilirubinBlood plasmaHemoglobinPhosphatasesProthrombin timeResearch laboratoriesSerum glutamic oxaloacetic transaminaseTransplantationUltrasound imaging ePublished: 15 August 2000 Issue Published: 7 December 1999 Copyright & PermissionsCopyright © 1999 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...