Giannouli S, Tzoanopoulos D, Ritis K, Kartalis G, Moutsopoulos H M, Voulgarelis M
Department of Pathophysiology, Medical School, National University of Athens, Athens, Greece.
Ann Rheum Dis. 2004 May;63(5):578-82. doi: 10.1136/ard.2003.012948.
Patients with myelodysplasia may have autoimmune manifestations (AIM). Interferon regulatory factor-1 (IRF-1) is a transcription factor involved in interferon signalling, leukaemogenesis, and the development of the immune system.
To determine whether IRF-1 is implicated in the pathophysiology of AIM in myelodysplasia.
14 patients with myelodysplasia were studied, seven with AIM and seven without. Five patients with vasculitis and seven normal subjects served as controls. The expression of IRF-1 was studied in bone marrow mononuclear cells taken from patients and controls, using a relative quantitative reverse transcriptase polymerase chain reaction.
A 10-fold reduction in full length IRF-1 mRNA was detected in the myelodysplasia patients without AIM compared with the normal controls. In contrast, the group with AIM had increased IRF-1 transcripts, to a level almost equal to that observed in patients with vasculitis and normal controls.
Myelodysplasia patients without IRF-1 expression had a decreased incidence of AIM. Thus the absence of IRF-1 transcription factor appears to protect against the development of autoimmunity in myelodysplasia.
骨髓增生异常综合征患者可能有自身免疫表现（AIM）。干扰素调节因子-1（IRF-1）是一种参与干扰素信号传导、白血病发生及免疫系统发育的转录因子。
确定IRF-1是否与骨髓增生异常综合征中AIM的病理生理学有关。
研究了14例骨髓增生异常综合征患者，其中7例有AIM，7例无AIM。5例血管炎患者和7名正常受试者作为对照。采用相对定量逆转录聚合酶链反应，研究患者及对照骨髓单个核细胞中IRF-1的表达。
与正常对照相比，无AIM的骨髓增生异常综合征患者全长IRF-1 mRNA降低了10倍。相反，有AIM的组IRF-1转录本增加，几乎达到血管炎患者及正常对照的水平。
无IRF-1表达的骨髓增生异常综合征患者AIM发生率降低。因此，IRF-1转录因子的缺失似乎可预防骨髓增生异常综合征中自身免疫的发生。
