Boston University School of Medicine, Boston, MA, USA.
Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Curr Hematol Malig Rep. 2018 Aug;13(4):244-255. doi: 10.1007/s11899-018-0463-9.
Immune dysregulation is a defining feature of myelodysplastic syndromes (MDS). Recently, several studies have further defined the complex role of immune alterations within MDS. Herein, we will summarize some of these findings and discuss the therapeutic strategies currently in development.
Immune alterations in MDS are complex, heterogeneous, and intertwined with clonal hematopoiesis and stromal cell dysfunction. Inflammation in MDS proceeds as a vicious cycle, mediated in large part by secreted factors, which induce cell death and activate innate immune signaling. Therapeutic targeting of this variable immune dysregulation has led to modest responses thus far, but incorporation of the growing repertoire of immunotherapy brings new potential for improved outcomes. The immune milieu is variable across the spectrum of MDS subtypes, with a changing balance of inflammatory and suppressive cellular forces from low- to high-risk disease.
免疫失调是骨髓增生异常综合征（MDS）的一个特征。最近，多项研究进一步明确了 MDS 中免疫改变的复杂作用。在此，我们将总结其中的一些发现，并讨论目前正在开发的治疗策略。
MDS 中的免疫改变复杂且具有异质性，与克隆性造血和基质细胞功能障碍交织在一起。MDS 中的炎症呈恶性循环，很大程度上由分泌因子介导，这些因子诱导细胞死亡并激活先天免疫信号。针对这种可变免疫失调的治疗靶向迄今为止仅取得了适度的反应，但免疫疗法的应用范围不断扩大，为改善预后带来了新的潜力。免疫微环境在 MDS 各亚型中存在差异，从低危疾病到高危疾病，炎症和抑制性细胞力量的平衡不断变化。
