多参数流式细胞术在儿童骨髓增生异常综合征诊断和预后评估中的应用价值

目的 评估多参数流式细胞术（MFC）及流式细胞术积分系统（FCSS）在儿童MDS诊断和预后评估中的应用价值。方法 回顾性分析42例儿童MDS初诊患者的临床资料，采用MFC分析MDS患儿骨髓各细胞群的表型及比例，并对FCSS评分与儿童MDS分型的关系、FCSS评分与国际预后积分系统（IPSS）评分的关系、FCSS评分与修订国际预后积分系统（IPSS-R）评分的关系进行相关分析。结果 异常髓系原始细胞增多者共20例（48%），19例（45%）淋系/髓系比> 1，淋系抗原在髓系细胞异常跨系表达者14例（33%），CD13/CD16分化抗原异常者8例（19%），CD56异常表达者5例（12%），3例（7%）粒系侧散射角（SSC）减弱或增强，3例（7%）有核红细胞CD36表达减弱，2例（5%）有核红细胞CD71表达减弱，髓系CD33表达缺失、粒系CD11b表达减弱或缺失、单核细胞系CD56及CD14表达缺失者各1例（2%）。FCSS评分低危组、中危组、高危组的中位总体生存时间以及无事件生存时间的差异均有统计学意义（P < 0.05）；3个组的2年总体生存率以低危组最高，中危组与高危组间的差异无统计学意义（P > 0.05）；3个组的2年无事件生存率依次95%、60%、46%，差异有统计学意义（P < 0.05）。FCSS评分与儿童MDS分型以及IPSS、IPSS-R的评分均呈正相关（P < 0.05）。结论 MFC及FCSS可以协助儿童MDS的诊断和预后评估。

Value of multiparameter flow cytometry in the diagnosis and prognostic evaluation of childhood myelodysplastic syndrome

Objective To investigate the value of multiparameter flow cytometry (MFC) and flow cytometric scoring system (FCSS) in the diagnosis and prognostic evaluation of childhood myelodysplastic syndrome (MDS). Methods A retrospective analysis was performed for the clinical data of 42 children who were diagnosed with MDS. MFC was performed to investigate the phenotype and proportion of each lineage of bone marrow cells. The correlations of FCSS score with MDS type, International Prognostic Scoring System (IPSS) score, and revised IPSS (IPSS-R) score were analyzed. Results Of all the 42 children, 20 (48%) had an increase in abnormal marrow blasts, 19 (45%) had a lymphoid/myeloid ratio of > 1, 14 (33%) had abnormal cross-lineage expression of lymphoid antigens in myeloid cells, 8 (19%) had abnormal CD13/CD16 differentiation antigens, 5 (12%) had abnormal expression of CD56, 3 (7%) had reduced or increased side scatter of granulocytes, 3 (7%) had reduced expression of CD36 in nucleated red blood cells, 2 (5%) had reduced expression of CD71 in nucleated red blood cells, 1 (2%) had absent expression of CD33 in myeloid cells, 1 (2%) had reduced or absent expression of CD11b in granulocytes, and 1 (2%) had absent expression of CD56 and CD14 in monocytes. There were significant differences in the median overall survival time and event-free survival time among the low-, medium-, and high-risk FCSS groups (P < 0.05). Among the low-, medium-, and high-risk FCSS groups,the low-risk FCSS group had the highest 2-year overall survival rate, while there was no significant difference between the medium-and high-risk FCSS groups (P > 0.05). The three groups had a 2-year event-free survival rate of 95%, 60%, and 46% respectively (P < 0.05). FCSS score was positively correlated with MDS type, IPSS score, and IPSS-R score (P < 0.05). Conclusions MFC and FCSS help with the diagnosis and prognostic evaluation of childhood MDS.