Large-vessel arteritis and myelodysplastic syndrome: report of two cases

European Journal of Haematology
M SteurerJ Gruber

Abstract

Myelodysplastic syndrome (MDS) is frequently associated with autoimmune diseases such as polymyalgia, arthritis, and rarely, with systemic vasculitis. The pathogenesis of these autoimmune complications remains unknown, but there is increasing evidence of profound immune dysregulation in MDS. In the few cases reported so far, vasculitides associated with MDS affected mainly cutaneous vessels. Here we describe two cases of acute large-vessel vasculitis in association with MDS. The first patient is a 67-yr-old male presenting with a massive large-vessel arteritis as primary manifestation of refractory anemia with excess of blasts type 1 (RAEB-1). The second patient is a 60-yr-old male, who presented with acute thoracic aortitis after a 2-yr history of refractory anemia with ringed sideroblasts (RARS). Both patients received immunosuppressive treatment with steroids, leading to rapid improvement of systemic inflammatory symptoms, vessel wall injury and peripheral blood counts. Whereas the first patient displayed sustained favorable hematologic responses under long-term steroid therapy, there was a rapid transformation into secondary acute myeloid leukemia in the second patient. We conclude that large-vessel vasculitis should be add...Continue Reading

References

Jun 1, 1992·Seminars in Arthritis and Rheumatism·S W George, E D Newman
Mar 1, 1991·The American Journal of Medicine·C PomeroyG A Filice
Mar 1, 1990·British Journal of Haematology·A R GreenD P Bentley
May 1, 1986·British Journal of Haematology·G J MuftiJ A Copplestone
Aug 1, 1985·Journal of Clinical Pathology·T EconomopoulosS Raptis
Mar 1, 1985·British Journal of Haematology·G J MuftiD Machin
Oct 1, 1994·Leukemia & Lymphoma·T FelzmannH Ludwig
Jan 1, 1993·Leukemia & Lymphoma·J A SavigeJ C Duggan
Jan 1, 1997·Arthritis and Rheumatism·B PhilippeO Hermine
Feb 13, 2001·Postgraduate Medical Journal·F F LopezF J Schiffman
Oct 9, 2001·Seminars in Arthritis and Rheumatism·M A HamidouJ Y Grolleau
Nov 5, 2002·The American Journal of Pathology·Wei Ma KrupaCornelia M Weyand
Jan 21, 2003·Leukemia & Lymphoma·Muhammad Wasif SaifSteven D Gore
Sep 19, 2003·Annals of Internal Medicine·Cornelia M Weyand, Jörg J Goronzy

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Citations

May 12, 2005·Blood Reviews·Daniel V T Catenacci, Gary J Schiller
Jan 7, 2014·Internal Medicine·Takayuki KatsuyamaHirofumi Makino
Apr 2, 2013·Autoimmunity Reviews·C BeliznaN Ifrah
Jul 24, 2012·Current Rheumatology Reports·Amir AghaJoanne Valeriano-Marcet
Jan 10, 2012·Leukemia & Lymphoma·Shaun FlemingJohn F Seymour
May 23, 2013·Leukemia Research·Omar Al UstwaniMeir Wetzler
Apr 2, 2011·Clinical Lymphoma, Myeloma & Leukemia·Matan J CohenNeta Goldschmidt
Apr 27, 2011·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Jin Kyun ParkAlan N Baer
Apr 28, 2017·Hematological Oncology·Claudio Fozza
Mar 5, 2009·British Journal of Cancer·L A AndersonE A Engels
Mar 17, 2018·BMJ Case Reports·Shudan WangJohannes Nowatzky
Oct 14, 2014·Expert Review of Clinical Immunology·Stavroula Giannouli, Michael Voulgarelis
Jun 21, 2017·Case Reports in Hematology·Sylwia SasinowskaHala Eid
Oct 27, 2015·BMC Research Notes·Shabneez HussainShahid Ahmed Sami
Mar 17, 2017·Journal of Medical Case Reports·Dinusha ChandratillekeLucinda J Berglund
Dec 16, 2019·Autoimmunity Reviews·Anne Laure RoupieUNKNOWN On behalf MINHEMON (French Network of dysimmune disorders associated with hemopathies)

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