title: Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and literature review.
abstract: We wanted to describe the characteristics, treatment and outcome of autoimmune and inflammatory diseases (SAIDs) associated with chronic myelomonocytic leukemia (CMML), and conducted a French multicenter retrospective study and a literature review. We included 26 cases of CMML (median age 75 years, 54% female), 80% with CMML-1. CPSS score was low (0 or 1) in 75% of cases. SAIDS was systemic vasculitis in 54%. Diagnosis of the 2 diseases was concomitant in 31% cases, and CMML was diagnosed before SAIDs in 12 cases (46%). First line treatment for SAIDs consisted mostly of steroid, with 85% of response. Second-line treatment was needed in 40% cases. Six patients received hypomethylating agents, with 66% response on SAIDs. A literature review found 49 cases of CMML-associated SAIDs, in whom SAIDs was systemic vasculitis in 29% cases. Hence, vasculitis is the most frequent SAIDs associated with CMML. After initial response to steroids, recurrence and steroid-dependence were frequent. Hypomethylating agents may be interesting in this context.
authors.affiliation: DHUi2B, Service de Médecine Interne, Hôpital Saint Antoine, APHP, Université Paris 6, 75012 Paris, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Grignano
authors.affiliation: DHUi2B, Service de Médecine Interne, Hôpital Saint Antoine, APHP, Université Paris 6, 75012 Paris, France. Electronic address: arsene.mekinian@sat.aphp.fr.
authors.forename: Arsene
authors.initials: A
authors.lastname: Mekinian
authors.affiliation: Service d'hématologie clinique, Université Paris 13, AP-HP, Avicenne, 93000, Bobigny, France.
authors.forename: Thorsten
authors.initials: T
authors.lastname: Braun
authors.affiliation: Service de médecine interne, CHU Dupuytren, Limoges, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Liozon
authors.affiliation: Service de médecine interne, Université Nantes, CHU de Nantes, Nantes, France.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.affiliation: Service de médecine interne, Université Rennes 1, Hôpital Universitaire de Rennes, Rennes, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Decaux
authors.affiliation: Service de médecine interne, Université Paris 5, AP-HP, CHU Cochin, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Service de médecine interne, Université Versailles-Saint Quentin en Yvelines, Hôpital Foch, Suresnes, France.
authors.forename: Jean Emmanuel
authors.initials: JE
authors.lastname: Kahn
authors.affiliation: Service de médecine interne, Université Versailles-Saint Quentin en Yvelines, Hôpital Foch, Suresnes, France.
authors.forename: Yoland
authors.initials: Y
authors.lastname: Schoindre
authors.affiliation: Service d'hématologie clinique, Université Paris 5, AP-HP, Hôpital Necker, Paris, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Rossignol
authors.affiliation: Service de maladies infectieuses, Université Paris 5, AP-HP, Hôpital Necker, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Lortholary
authors.affiliation: Service de médecine interne, CHU Tours, Tours, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Lioger
authors.affiliation: Service d'hématologie clinique, Université Paris 5, AP-HP, Hôpital Necker, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.affiliation: Service d'hématologie clinique, CHU Grenoble, Grenoble, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Park
authors.affiliation: Service d'hématologie clinique, Université Paris 7, AP-HP, CHU Saint-Louis, Paris, France.
authors.forename: Lionel
authors.initials: L
authors.lastname: Ades
authors.affiliation: eXYSTAT, Statistics, Malakoff, France.
authors.forename: François
authors.initials: F
authors.lastname: Montestruc
authors.affiliation: DHUi2B, Service de Médecine Interne, Hôpital Saint Antoine, APHP, Université Paris 6, 75012 Paris, France.
authors.forename: Laure
authors.initials: L
authors.lastname: Ricard
authors.affiliation: Service d'hématologie clinique, Université Paris 13, AP-HP, Avicenne, 93000, Bobigny, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Gardin
authors.affiliation: Service d'hématologie clinique, Université Paris 7, AP-HP, CHU Saint-Louis, Paris, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Fenaux
authors.affiliation: DHUi2B, Service de Médecine Interne, Hôpital Saint Antoine, APHP, Université Paris 6, 75012 Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Fain
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000188:drug therapy; D005260:Female; D005602:France; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007249:Inflammation / Q000188:drug therapy; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications*; D008297:Male; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use; D011795:Surveys and Questionnaires; D014657:Vasculitis
chemical_list: D007166:Immunosuppressive Agents; D013256:Steroids
keywords: Autoimmune diseases; Chronic myelomonocytic leukemia; Hypomethylating agents; Immunosuppressive agents; Outcome; Vasculitis