Zhang Xiaohui, Sokol Lubomir, Bennett John M, Moscinski Lynn C, List Alan, Zhang Ling
Department of Hematopathology and Laboratory Medicine, United States.
Department of Malignant Hematology, H. Lee Moffitt Cancer Center & Research Institute, Tampa, FL, United States.
Leuk Res. 2016 Apr;43:18-23. doi: 10.1016/j.leukres.2016.02.006. Epub 2016 Feb 17.
Inflammatory and immune dysregulation are crucial in the initiation and development of myelodysplastic syndromes (MDS). It is noted that clonal T-cell large granular lymphocyte (T-LGL) proliferation associated with MDS is not uncommon. However, clinicopathological features, and prognostic and predictive value of presence of T-LGL proliferation in MDS patients is not very clear. This study compared 35 MDS patients with T-LGL proliferation with 36 MDS patients without T-LGL proliferation and summarized clinicopathologic features, including peripheral blood LGL cell counts, immunophenotype, T cell receptor gene rearrangement, bone marrow hematopoietic status, and adjuvant immunosuppressive therapy. The peripheral blood CD3+/CD57+ cell counts were significantly different (p<0.01) between the two groups. Notably, on examination of the bone marrow, MDS patients with T-LGL proliferation showed more frequent hypocellularity and/or lineage hypoplasia, particularly erythroid hypoplasia. On survival analysis, no overall difference was noted between MDS patients with T-LGL proliferation and those without T-LGL proliferation, and between the patients who received therapy for LGL and those who did not receive adjuvant therapy for LGL in the same risk group. In conclusion, T-LGL proliferation present in MDS patients can be associated with bone marrow hypocellularity and lineage hypoplasia. Although immunosuppressive therapy to eliminate T-LGL cells is potentially beneficial to the MDS patients with associated T-LGL proliferation, there is no overall survival benefit to the patients who received such treatment.
炎症和免疫失调在骨髓增生异常综合征（MDS）的发生和发展中起着关键作用。值得注意的是，与MDS相关的克隆性T细胞大颗粒淋巴细胞（T-LGL）增殖并不少见。然而，MDS患者中T-LGL增殖的临床病理特征以及预后和预测价值尚不清楚。本研究比较了35例有T-LGL增殖的MDS患者和36例无T-LGL增殖的MDS患者，并总结了临床病理特征，包括外周血LGL细胞计数、免疫表型、T细胞受体基因重排、骨髓造血状态以及辅助免疫抑制治疗。两组之间外周血CD3+/CD57+细胞计数有显著差异（p<0.01）。值得注意的是，在骨髓检查中，有T-LGL增殖的MDS患者显示出更频繁的细胞减少和/或谱系发育不全，尤其是红系发育不全。生存分析显示，有T-LGL增殖的MDS患者与无T-LGL增殖的患者之间，以及在同一风险组中接受LGL治疗的患者与未接受LGL辅助治疗的患者之间，总体上没有差异。总之，MDS患者中存在的T-LGL增殖可能与骨髓细胞减少和谱系发育不全有关。虽然消除T-LGL细胞的免疫抑制治疗可能对伴有T-LGL增殖的MDS患者有益，但接受此类治疗的患者总体生存并无获益。
