Giannouli Stavroula, Voulgarelis Michael
2nd Department of Internal Medicine, Medical School, National University of Athens, Athens, Greece.
Expert Rev Clin Immunol. 2014 Dec;10(12):1679-88. doi: 10.1586/1744666X.2014.970181. Epub 2014 Oct 13.
Primary myelodysplastic syndromes (MDS) are heterogeneous clonal hemopoietic disorders clinically presented with a varying degree of peripheral cytopenias and an increased probability of leukemic evolution. A distinct subset of MDS patients manifests overt autoimmune-inflammatory manifestations, the underlying pathogenesis and prognostic significance of which still remain controversial. In this review we attempt to analyze clinical aspects of MDS-related rheumatoid disease, and discuss pathophysiologic associations between autoimmunity and distorted BM function in preleukemic states in light of recent findings, in vivo and in vitro. We further explore the potential of recent biological and molecular advances to forward therapeutic targeting against both autoimmune and malignant process.
原发性骨髓增生异常综合征(MDS)是一组异质性克隆性造血疾病,临床表现为不同程度的外周血细胞减少以及白血病进展概率增加。MDS患者中有一个独特的亚组表现出明显的自身免疫性炎症表现,其潜在的发病机制和预后意义仍存在争议。在本综述中,我们试图分析MDS相关类风湿性疾病的临床方面,并根据体内和体外的最新研究结果,探讨自身免疫与白血病前期骨髓功能异常之间的病理生理联系。我们还进一步探索了近期生物学和分子进展在针对自身免疫和恶性过程进行治疗靶向方面的潜力。