Dias Ana Luisa Abrahão, da Silva Raquel Gomes, Cunha Fernanda Gonçalves Pereira, Morcillo André Moreno, Lorand-Metze Irene, Vilela Maria Marluce Dos Santos, Riccetto Adriana Gut Lopes
Pediatric Allergy and Immunology/Center of Investigation in Pediatrics (CIPED), Faculty of Medical Sciences, State University of Campinas - Unicamp, Sao Paulo, Brazil.
Laboratory of Cell Markers - Hematology/Hemotherapy Center, Faculty of Medical Sciences, State University of Campinas - Unicamp, Sao Paulo, Brazil.
APMIS. 2019 Apr;127(4):228-235. doi: 10.1111/apm.12932.
Our aim was to evaluate the cost-effectiveness of a minimal lymphocyte subset quantification (LSQ) by flow cytometry as the first screening in children with clinically suspected primary immunodeficiency (PID). Two hundred sixty-eight Brazilian patients (0-21 years old) were studied. They were divided by clinical and phenotypical features into those fulfilling criteria for PID (PID phenotype) according to the 2017 International Union of Immunological Societies (IUIS) classification and those not fulfilling these criteria (non-PID phenotype). We evaluated how many patients had values below the 10th percentile for five lymphocyte subsets in peripheral blood, (suggestive of PID) according to reference values for Brazil, Italy and USA. Three lymphocyte subsets (T CD3/CD4, B CD19 and NK CD16/CD56) had p-value < 0.05 and Odds Ratio (OR) indicating a risk at least two times higher for the diagnosis of a PID phenotype. The application of Kappa coefficient (k) on Brazilian vs Italian and Brazilian vs US data sets resulted in k compatible with strong or excellent level of agreement between the three classification systems. The authors conclude that a number of CD3 /CD4 , CD19 and CD16 /CD56 (NK) cells in peripheral blood <10th percentile represented a significant risk for the diagnosis of PID in this cohort. Natural killer (NK) deficiency is quite rare and has a very specific clinical profile. So, the analysis of these cells could be requested only in some cases, saving even more costs. The minimal immunophenotyping, with quantification of T CD4 , CD19 and in some cases CD16 /CD56 cells, may be a useful tool for the first screening of PID, saving costs, especially in developing countries.
我们的目的是评估通过流式细胞术进行最小淋巴细胞亚群定量分析（LSQ）作为临床疑似原发性免疫缺陷病（PID）患儿首次筛查方法的成本效益。我们研究了268名巴西患者（0至21岁）。根据2017年国际免疫学会联盟（IUIS）的分类，依据临床和表型特征，将他们分为符合PID标准的患者（PID表型）和不符合这些标准的患者（非PID表型）。我们根据巴西、意大利和美国的参考值，评估了外周血中五个淋巴细胞亚群数值低于第10百分位数的患者数量（提示PID）。三个淋巴细胞亚群（T CD3/CD4、B CD19和NK CD16/CD56）的p值<0.05且比值比（OR）表明诊断为PID表型的风险至少高出两倍。对巴西与意大利以及巴西与美国数据集应用kappa系数（k），结果显示k值表明这三种分类系统之间具有强或极好的一致性水平。作者得出结论，外周血中CD3 /CD4、CD19和CD16 /CD56（NK）细胞数量低于第10百分位数表明该队列中PID诊断存在显著风险。自然杀伤（NK）细胞缺乏相当罕见且具有非常特殊的临床特征。因此，仅在某些情况下才需要分析这些细胞，从而进一步节省成本。对T CD4、CD19以及某些情况下的CD16 /CD56细胞进行定量的最小免疫表型分析，可能是首次筛查PID的有用工具，可节省成本，尤其是在发展中国家。
