Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and literature review

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Grignano, Eric | Mekinian, Arsene | Braun, Thorsten | Liozon, Eric | Hamidou, Mohamed | Decaux, Olivier | Puechal, 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

Edité par CCSD ; Elsevier -

International audience. 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. © 2016 Elsevier Ltd.

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