1st Department of Internal Medicine, Haematology Unit, National and Kapodistrian University of Athens, Laikon General Hospital, Athens 11527, Greece.
Virol J. 2012 Jan 3;9:1. doi: 10.1186/1743-422X-9-1.
Myelodysplastic syndromes (MDS) are often accompanied by autoimmune phenomena. The underlying mechanisms for these associations remain uncertain, although T cell activation seems to be important. Human T-lymphotropic virus (HTLV-1) has been detected in patients with myelodysplastic syndromes, mostly in regions of the world which are endemic for the virus, and where association of HTLV-1 with rheumatological manifestation is not rare. We present here the case of a 58 year old man who presented with cytopenias, leukocytoclastic vasculitis of the skin and glomerulopathy, and was diagnosed as MDS (refractory anemia with excess blasts - RAEB 1). The patient also tested positive for HTLV-1 by PCR. After 8 monthly cycles of 5-azacytidine he achieved a complete hematologic remission. Following treatment, a second PCR for HTLV-1 was carried out and found to be negative. This is the first report in the literature of a HTLV-1-positive MDS with severe autoimmune manifestations, which was treated with the hypomethylating factor 5-azacitidine, achieving cytogenetic remission with concomitant resolution of the autoimmune manifestations, as well as HTLV-1-PCR negativity. HTLV-1-PCR negativity may be due to either immune mediated clearance of the virus, or a potential antiretroviral effect of 5-azacytidine. 5-azacytidine is known for its antiretroviral effects, although there is no proof of its activity against HTLV-1 infection in vivo.
骨髓增生异常综合征（MDS）常伴有自身免疫现象。这些关联的潜在机制尚不确定，尽管 T 细胞激活似乎很重要。人类 T 淋巴细胞病毒（HTLV-1）已在骨髓增生异常综合征患者中检测到，主要在病毒流行的世界区域，并且 HTLV-1 与风湿表现的关联并不罕见。我们在此介绍一位 58 岁男性患者，他表现为血细胞减少、皮肤白细胞碎裂性血管炎和肾小球病，并被诊断为 MDS（难治性贫血伴原始细胞增多-RAEB 1）。该患者还通过 PCR 检测出 HTLV-1 阳性。经过 8 个 5-氮杂胞苷周期的治疗后，他达到了完全血液学缓解。治疗后，进行了第二次 HTLV-1 的 PCR 检测，结果为阴性。这是文献中首例 HTLV-1 阳性 MDS 伴严重自身免疫表现的病例，采用低甲基化因子 5-氮杂胞苷治疗，取得了细胞遗传学缓解，同时自身免疫表现也得到了缓解，以及 HTLV-1-PCR 阴性。HTLV-1-PCR 阴性可能是由于病毒的免疫清除，或 5-氮杂胞苷的潜在抗逆转录病毒作用。5-氮杂胞苷以其抗逆转录病毒作用而闻名，尽管没有证据表明其在体内对 HTLV-1 感染具有活性。
