JAK1/2 Inhibition in Severe TAFRO Syndrome: A Case Report
Authors: Martin Killian, Sébastien Viel, Emilie Chalayer, Fabien Forest, Sylvain Grange, Pierre-Benoit Bonnefoy, Eric Oksenhendler, Pascal Cathébras, Stéphane Paul
Journal: Annals of Internal Medicine (2021)

LettersMay 2021JAK1/2 Inhibition in Severe TAFRO Syndrome: A Case ReportMartin Killian, MD, PhD, Sébastien Viel, PharmD, PhD, Emilie Chalayer, MD, PhD, Fabien Forest, MD, PhD, Sylvain Grange, MD, Pierre-Benoit Bonnefoy, MD, Eric Oksenhendler, MD, PhD, Pascal Cathébras, MD, and Stéphane Paul, PhDMartin Killian, MD, PhDCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, France, Sébastien Viel, PharmD, PhDCentre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France, Emilie Chalayer, MD, PhDInstitut de Cancérologie Lucien Neuwirth, Saint-Priest-en-Jarez, France, Fabien Forest, MD, PhDCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, France, Sylvain Grange, MDCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, France, Pierre-Benoit Bonnefoy, MDCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, France, Eric Oksenhendler, MD, PhDHôpital Saint-Louis, Paris, France, Pascal Cathébras, MDCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, France, and Stéphane Paul, PhDCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, FranceAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/L20-1051 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome is a rare inflammatory disorder of unknown cause (1). It is primarily regarded as a severe subtype of idiopathic multicentric Castleman disease (iMCD), referred to as iMCD-TAFRO. Others believe that TAFRO could be either a clinical entity overlapping with iMCD or a unique autoimmune or inflammatory disease that can be accompanied by an iMCD-like lymph node histopathology (2). Patients with iMCD-TAFRO have a different cytokine spectrum and unusual clinical manifestations (including thrombocytopenia, anasarca, and renal dysfunction) compared with patients with iMCD without TAFRO, referred to as iMCD not otherwise specified ...References1. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135:1353-64. [PMID: 32106302] doi:10.1182/blood.2019000931 CrossrefMedlineGoogle Scholar2. Masaki Y, Kawabata H, Takai K, et al. Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version. Int J Hematol. 2016;103:686-92. [PMID: 27084250] doi:10.1007/s12185-016-1979-1 CrossrefMedlineGoogle Scholar3. Fujimoto S, Sakai T, Kawabata H, et al. Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease. Am J Hematol. 2019;94:975-83. [PMID: 31222819] doi:10.1002/ajh.25554 CrossrefMedlineGoogle Scholar4. Arenas DJ, Floess K, Kobrin D, et al. Increased mTOR activation in idiopathic multicentric Castleman disease. Blood. 2020;135:1673-84. [PMID: 32206779] doi:10.1182/blood.2019002792 CrossrefMedlineGoogle Scholar5. Langan Pai R, Japp AS, Gonzalez M, et al. Type I IFN response associated with mTOR activation in the TAFRO subtype of idiopathic multicentric Castleman disease. JCI Insight. 7 May 2020. Accessed at https://insight.jci.org/articles/view/135031 on 20 May 2020. Google Scholar Author, Article, and Disclosure InformationAffiliations: Centre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, FranceCentre Hospitalier Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, FranceInstitut de Cancérologie Lucien Neuwirth, Saint-Priest-en-Jarez, FranceCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, FranceHôpital Saint-Louis, Paris, FranceCentre Hospitalier Universitaire de Saint-Etienne and Université Jean Monnet, Université de Lyon, Saint-Etienne, FranceGrant Support: This work was partly supported by a grant from the French Ministry of Health (PHRC-GIRCI AURA_16-006). The funding source had no role in the reporting of the study or the decision to publish the manuscript.Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=L20-1051.Corresponding Author: Martin Killian, MD, PhD, Department of Internal Medicine, Centre Hospitalier Universitaire de Saint-Etienne, 42055 Saint-Etienne Cedex 02, France; e-mail, martin.[email protected]fr.This article was published at Annals.org on 12 January 2021. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byTAFRO syndrome complicated by porto-sinusoidal vascular liver disease with portal hypertension: a case report May 2021Volume 174, Issue 5Page: 719-721KeywordsBone marrowCorticosteroid therapyDisclosureEdemaInterferonsKidneysLungsLymph nodesPlateletsPleural effusion ePublished: 12 January 2021 Issue Published: May 2021 Copyright & PermissionsCopyright © 2021 by American College of Physicians. 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