Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and literature review. (August 2016)

• Record Type: Journal Article
• Authors: Grignano, Eric; Mekinian, Arsene; Braun, Thorsten; Liozon, Eric; Hamidou, Mohamed; Decaux, Olivier; Puéchal, Xavier; Kahn, Jean Emmanuel; Schoindre, Yoland; Rossignol, Julien; Lortholary, Olivier; Lioger, Bertrand; Hermine, Olivier; Park, Sophie; Ades, Lionel; Montestruc, François; Ricard, Laure; Gardin, Claude; Fenaux, Pierre; Fain, Olivier
• Is Part Of: Leukemia research. Volume 47(2016:Aug.)
• Page Start: 136
• Page End: 141
• Publication Date: 2016-08
• Subjects: Chronic myelomonocytic leukemia -- Autoimmune diseases -- Vasculitis -- Immunosuppressive agents -- Hypomethylating agents -- Outcome; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2016.05.013
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: Vasculitis are the most frequent autoimmune diseases associated with CMML. CMML features are worse when associated to SAIDs. Hypomethylating agents may represent an alternative therapy after steroids. 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.