Low-Grade Myelodysplastic Syndromes with Preserved CD34+B-Cell Precursors (CD34+Hematogones)
Low-Grade Myelodysplastic Syndromes with Preserved CD34+B-Cell Precursors (CD34+Hematogones)
复制标题
保留 CD34+B 细胞前体的低度骨髓增生异常综合征 (CD34+Hematogones)
DOI：
10.1002/cyto.b.21830
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发表时间：
2020-01-01
影响因子：
3.4
通讯作者：
Wang, Sa A.
中科院分区：
文献类型：
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作者：
Chen, Zhining;Ok, Chi Young;Wang, Sa A.
骨髓增生异常综合征 (MDS) 的骨髓中 B 细胞祖细胞（血细胞素）显着减少或完全缺失，这一发现被认为是 MDS 流式细胞术免疫表型分析的一个重要特征。我们在两年内连续收集的 160 名初治低度原发性 MDS 患者中研究了 CD34+ 血细胞占总 CD34+ 细胞的比例。在确认中位 CD34 + 血细胞素显着下降（1.08%、0% 至 67.86%）的同时，我们观察到一些 MDS 患者的 CD34 + 血细胞素存在不同程度的保留。使用 5% 截止值，总共 46 名 (29%) MDS 患者具有 >= 5% CD34 + 血红素，其中环状铁粒幼细胞 (RS) (MDS-RS) 的 MDS 患者比例显着过高（18/40, 45%，vs. 28/120, 23%，P = 0.015）。虽然我们没有观察到 MDS-RS 中的独特特征，但在大量无 RS 的 MDS 中明显不存在突变（37% vs. 14%，P = 0.013），包括 TP53 突变（0% vs.16.5%，P = 0.021）（如果存在 >= 5% CD34 + 血红素）。尽管随访时间较短（18.5 个月，0 至 151.0），但在 CD34 + 血素≥ 5% 的 MDS 中观察到更好的总生存率，无论是作为一个组（P = 0.008）还是在没有 RS 的患者中（P = 0.028）。在多变量分析中，血红素减少仍然是重大危险（P = 0.015）。总之，在超过四分之一的原发性低级别 MDS 中观察到保留的 CD34 + 血红素 (>= 5%)，这些病例以伴有 RS 的 MDS 或没有可检测到的突变的 MDS 为代表。这一发现是新的，这种现象可能部分归因于 CD34+ 祖细胞的 B 细胞分化的保留，并且与低度 MDS 患者更好的预后相关。 (c) 2019年国际临床细胞计数学会
B-cell progenitors (hematogones) are markedly decreased or completely absent in the bone marrows of myelodysplastic syndromes (MDS), and the finding is considered as an important feature in MDS flow cytometry immunophenotyping. We studied CD34+ hematogones as a proportion of total CD34+ cells in 160 treatment naive low grade primary MDS patients, consecutively collected over a two-year period. While confirming that the median CD34 + hematogones was significantly decreased (1.08%, 0%, to 67.86%), we observed variably preserved CD34 + hematogones in some MDS patients. Using a 5% cutoff, a total of 46 (29%) MDS patients had >= 5% CD34 + hematogones, significantly overrepresented by MDS with ring sideroblasts (RS) (MDS-RS) (18/40, 45%, vs. 28/120, 23%, P = 0.015). While we did not observe unique features among MDS-RS, mutations were noticeably absent in a significant number of MDS without RS (37% vs. 14%, P = 0.013), including TP53 mutations (0% vs.16.5%, P = 0.021) if >= 5% CD34 + hematogones were present. Although the follow up was short (18.5 months, 0 to 151.0), a better overall survival was observed in MDS with >= 5% CD34 + hematogones, either as a group (P = 0.008) or among patients with no RS (P = 0.028). In multivariate analysis, reduced hematogones remained to be significant hazard (P = 0.015). In summary, preserved CD34 + hematogones (>= 5%) are seen in over a quarter of primary low-grade MDS and these cases are overrepresented by MDS with RS or MDS with no detectable mutations. The finding is new, and this phenomenon may in part attribute to preservation of B-cell differentiation of CD34+ progenitors, and it is associated with a better prognosis in low grade MDS patients. (c) 2019 International Clinical Cytometry Society