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A Close Association of Autoimmune-Mediated Processes and Autoimmune Disorders with Chronic Myelomonocytic Leukemia: Observation from a Single Institution

机译:自身免疫介导的过程和自身免疫性疾病与慢性粒单核细胞白血病的密切联系:从单个机构的观察。

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Chronic myelomonocytic leukemia (CMML), a clonal hematopoietic stem cell disease, may be linked to immune-mediated processes and/or autoimmune disorders (AID), although the exact pathogens are still elusive. We retrospectively analyzed 123 CMML patients in our institution. Twenty-four CMML patients (19.5%) had at least one immune-mediated disorder, most commonly idiopathic thrombocytopenic purpura, gout and psoriasis. Four of these 24 patients (15%) had more than one AID. We found that, in contrast to the general population with a prevalence rate of 3.2-5.2%, newly diagnosed CMML patients demonstrated a high prevalence and variety of immune-mediated processes and/or AID. When we compared the results with those of myelodysplastic syndromes published in the literature, the prevalence of AID in these two groups of patients is similar. Our results also showed that the presence of cytogenetic abnormalities was less in CMML patients with AID (6 of 21; 28.6%) than in those without AID (37 of 94; 39.4%), although there was no statistical significance (p = 0.334). A multicenter large cohort study of CMML with AID is recommended to illustrate the molecular relationship between the two distinct groups. (C) 2014 S. Karger AG, Basel
机译:慢性粒细胞单核细胞白血病(CMML)是一种克隆的造血干细胞疾病,尽管确切的病原体仍然难以捉摸,但可能与免疫介导的过程和/或自身免疫性疾病(AID)有关。我们回顾性分析了我们机构中的123例CMML患者。 24名CMML患者(19.5%)患有至少一种免疫介导的疾病,最常见的是特发性血小板减少性紫癜,痛风和牛皮癣。这24名患者中有4名(15%)的AID超过一​​种。我们发现,与一般人群3.2-5.2%的患病率相比,新诊断的CMML患者表现出很高的患病率,并且免疫介导的过程和/或AID多种多样。当我们将结果与文献中发表的骨髓增生异常综合征的结果进行比较时,这两组患者中AID的患病率相似。我们的研究结果还表明,尽管没有统计学意义(p = 0.334),但有AID的CMML患者(21例中的6例; 28.6%)比没有AID的CMML患者(37例中的94例; 39.4%)更少。建议对AML进行CMML的多中心大型队列研究,以说明两个不同组之间的分子关系。 (C)2014 S.Karger AG,巴塞尔

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