| Literature DB >> 27337291 |
Eric Grignano1, Arsene Mekinian2, Thorsten Braun3, Eric Liozon4, Mohamed Hamidou5, Olivier Decaux6, Xavier Puéchal7, Jean Emmanuel Kahn8, Yoland Schoindre8, Julien Rossignol9, Olivier Lortholary10, Bertrand Lioger11, Olivier Hermine9, Sophie Park12, Lionel Ades13, François Montestruc14, Laure Ricard1, Claude Gardin3, Pierre Fenaux13, Olivier Fain1.
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.Entities:
Keywords: Autoimmune diseases; Chronic myelomonocytic leukemia; Hypomethylating agents; Immunosuppressive agents; Outcome; Vasculitis
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Year: 2016 PMID： 27337291 DOI： 10.1016/j.leukres.2016.05.013
Source DB: PubMed Journal: Leuk Res ISSN： 0145-2126 Impact factor: 3.156