1. Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and literature review.
- Author
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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, and Fain, Olivier
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AUTOIMMUNE diseases , *RETROSPECTIVE studies , *INFLAMMATION treatment , *CHRONIC myeloid leukemia , *STEROID drugs , *DIAGNOSIS , *THERAPEUTICS ,MEDICAL literature reviews - 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. [ABSTRACT FROM AUTHOR]
- Published
- 2016
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