Autologous Hematopoietic Stem Cell Transplantation for Susac Syndrome
Authors: Jose G. Ruiz de Morales, Miguel Cordero-Coma, Montserrat Rovira, Sara Calleja-Antolin, Gerard Espinosa, Natalia de las Heras
Journal: Annals of Internal Medicine (2020)

Letters18 August 2020Autologous Hematopoietic Stem Cell Transplantation for Susac SyndromeJose G. Ruiz de Morales, MD, PhD, Miguel Cordero-Coma, MD, PhD, Montserrat Rovira, MD, PhD, Sara Calleja-Antolin, MD, Gerard Espinosa, MD, PhD, and Natalia de las Heras, MDJose G. Ruiz de Morales, MD, PhDComplejo Hospitalario Universitario de León and Instituto de Biomedicina, University of León, León, Spain (J.G.R., M.C.), Miguel Cordero-Coma, MD, PhDComplejo Hospitalario Universitario de León and Instituto de Biomedicina, University of León, León, Spain (J.G.R., M.C.), Montserrat Rovira, MD, PhDStem Cell Transplantation Unit, IDIBAPS, Hospital Clinic, University of Barcelona, Barcelona, Spain (M.R.), Sara Calleja-Antolin, MDComplejo Hospitalario Universitario de León, León, Spain (S.C., N.D.), Gerard Espinosa, MD, PhDHospital Clinic, University of Barcelona, Barcelona, Spain (G.E.), and Natalia de las Heras, MDComplejo Hospitalario Universitario de León, León, Spain (S.C., N.D.)Author, Article, and Disclosure Informationhttps://doi.org/10.7326/L20-0055 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Susac syndrome is a rare, immune-mediated, ischemic vasculopathy affecting the brain, eyes, and ears. It varies greatly in severity, ranging from mild disease that is fully treatable with standard immunotherapy to severe, refractory, debilitating disease (1, 2).Objective: To alert physicians that autologous hematopoietic stem cell transplantation might successfully treat otherwise refractory Susac syndrome.Case Report: We took care of a woman with a history of epilepsy starting at age 9 months who was treated with carbamazepine until age 10 years. She also had a history of migraine with aura. At age 20 years, she was admitted for several ...References1. Rennebohm R, Susac JO, Egan RA, et al. Susac's syndrome—update. J Neurol Sci. 2010;299:86-91. [PMID: 20855088] doi:10.1016/j.jns.2010.08.032 CrossrefMedlineGoogle Scholar2. Rennebohm RM, Asdaghi N, Srivastava S, et al. Guidelines for treatment of Susac syndrome—an update. Int J Stroke. 2018:1747493017751737. [PMID: 29319463] doi:10.1177/1747493017751737 CrossrefMedlineGoogle Scholar3. Swart JF, Delemarre EM, van Wijk F, et al. Haematopoietic stem cell transplantation for autoimmune diseases. Nat Rev Rheumatol. 2017;13:244-256. [PMID: 28228650] doi:10.1038/nrrheum.2017.7 CrossrefMedlineGoogle Scholar4. Cohen JA, Baldassari LE, Atkins HL, et al. Autologous hematopoietic cell transplantation for treatment-refractory relapsing multiple sclerosis: position statement from the American Society for Blood and Marrow Transplantation. Biol Blood Marrow Transplant. 2019;25:845-854. [PMID: 30794930] doi:10.1016/j.bbmt.2019.02.014 CrossrefMedlineGoogle Scholar5. Gross CC, Meyer C, Bhatia U, et al. CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 2019;10:5779. [PMID: 31852955] doi:10.1038/s41467-019-13593-5 CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: Complejo Hospitalario Universitario de León and Instituto de Biomedicina, University of León, León, Spain (J.G.R., M.C.)Stem Cell Transplantation Unit, IDIBAPS, Hospital Clinic, University of Barcelona, Barcelona, Spain (M.R.)Complejo Hospitalario Universitario de León, León, Spain (S.C., N.D.)Hospital Clinic, University of Barcelona, Barcelona, Spain (G.E.)Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=L20-0055.Corresponding Author: Jose G. Ruiz de Morales, MD, PhD, Complejo Hospitalario Universitario de León, Altos de Nava S/N, 24080-León, Spain; e-mail, [email protected]es.This article was published at Annals.org on 7 July 2020. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited bySusac Syndrome: Description of a Single-Centre Case SeriesSusac syndrome: A scoping reviewActualités dans le syndrome de Susac 18 August 2020Volume 173, Issue 4Page: 315-317KeywordsArteriesColony stimulating factorCyclophosphamideDisclosureHeadachesHematopoietic stem cell transplantationIntravenous immunoglobulinLesionsMultiple sclerosisVisual acuity ePublished: 7 July 2020 Issue Published: 18 August 2020 Copyright & PermissionsCopyright © 2020 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...