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骨髓纤维化中自身免疫现象的患病率增加:与临床和形态学特征的关系,以及与免疫调节细胞因子模式的关系。

Increased prevalence of autoimmune phenomena in myelofibrosis: relationship with clinical and morphological characteristics, and with immunoregulatory cytokine patterns.

机构信息

U.O. Ematologia e Centro Trapianti di Midollo, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.

出版信息

Leuk Res. 2013 Nov;37(11):1509-15. doi: 10.1016/j.leukres.2013.09.001. Epub 2013 Sep 12.

DOI:10.1016/j.leukres.2013.09.001
PMID:24080022
Abstract

Autoimmune phenomena and cytokines were investigated in 100 patients with myelofibrosis (MF) and related to marrow fibrosis and clinical risk. Anti-erythrocyte antibodies by mitogen-stimulated direct antiglobulin test (MS-DAT) were positive in 45%, anti-platelets in 15% and organ/non organ-specific in 57% of cases, without clinically overt disease, and mostly in low-risk/intermediate-risk-1 and MF-0/MF-1. TGF-β and IL-8 were increased in MS-DAT positive cases, and IFN-γ in patients with serological autoantibodies. TGF-β and IL-17 were elevated in early clinical and morphological stages, while IL-8 increased in advanced stages. These data suggest that autoimmune phenomena and cytokine disregulation are particularly relevant in early MF.

摘要

研究了 100 例骨髓纤维化 (MF) 患者的自身免疫现象和细胞因子,并将其与骨髓纤维化和临床风险相关联。在无临床显性疾病的情况下,45%的病例通过丝裂原刺激直接抗球蛋白试验 (MS-DAT) 呈抗红细胞抗体阳性,15%的病例呈抗血小板抗体阳性,57%的病例呈器官/非器官特异性自身抗体阳性,且多发生于低危/中危-1 和 MF-0/MF-1。MS-DAT 阳性病例中 TGF-β 和 IL-8 增加,血清自身抗体患者中 IFN-γ 增加。在早期临床和形态学阶段,TGF-β 和 IL-17 升高,而在晚期阶段,IL-8 增加。这些数据表明,自身免疫现象和细胞因子失调在早期 MF 中尤为相关。

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