与慢性粒单核细胞白血病相关的自身免疫性和炎性疾病：26例病例系列及文献综述
Autoimmune and inflammatory diseases associated with chronic myelomonocytic leukemia: A series of 26 cases and literature review.
作者信息
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
机构信息
DHUi2B, Service de Médecine Interne, Hôpital Saint Antoine, APHP, Université Paris 6, 75012 Paris, France.
DHUi2B, Service de Médecine Interne, Hôpital Saint Antoine, APHP, Université Paris 6, 75012 Paris, France.
出版信息
Leuk Res. 2016 Aug;47:136-41. doi: 10.1016/j.leukres.2016.05.013. Epub 2016 Jun 2.
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.
我们希望描述与慢性粒单核细胞白血病（CMML）相关的自身免疫性和炎性疾病（SAIDs）的特征、治疗及预后，并开展了一项法国多中心回顾性研究及文献综述。我们纳入了26例CMML患者（中位年龄75岁，54%为女性），80%为CMML-1型。75%的病例中动态国际预后评分系统（CPSS）分数较低（0或1）。54%的SAIDs为系统性血管炎。31%的病例中两种疾病同时诊断，12例（46%）中CMML在SAIDs之前被诊断。SAIDs的一线治疗主要为类固醇，缓解率为85%。40%的病例需要二线治疗。6例患者接受了去甲基化药物治疗，SAIDs的缓解率为66%。一项文献综述发现49例与CMML相关的SAIDs病例，其中29%的病例SAIDs为系统性血管炎。因此，血管炎是与CMML相关的最常见的SAIDs。在对类固醇产生初始反应后，复发和类固醇依赖很常见。在这种情况下，去甲基化药物可能是有意义的。
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