Characterization of Infants with Idiopathic Transient and Persistent T Cell Lymphopenia Identified by Newborn Screening—a Single-Center Experience in New York State,Journal of Clinical Immunology

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Characterization of Infants with Idiopathic Transient and Persistent T Cell Lymphopenia Identified by Newborn Screening—a Single-Center Experience in New York State
Journal of Clinical Immunology ( IF 7.2 ) Pub Date : 2021-01-07 , DOI: 10.1007/s10875-020-00957-6
Artemio M Jongco _{1,

2,

3,

4} , Robert Sporter _{5,

6} , Elise Hon ₁ , Omer Elshaigi ₁ , Shouling Zhang _{2,

6} , Foysal Daian ₁ , Emily Bae ₁ , Amanda Innamorato ₁ , Catherine Capo ₁ , Brianne Navetta-Modrov ₇ , David W Rosenthal _{1,

2,

3} , Vincent R Bonagura _{1,

2,

3,

8}

Affiliation

Purpose

Newborn screening (NBS) quantifies T cell receptor excision circles (TREC) and identifies infants with T cell lymphopenia (TCL). This study elucidates the demographics, laboratory characteristics, genetics, and clinical outcomes following live viral vaccine administration of term infants with transient or persistent idiopathic TCL.

Methods

A single-center retrospective analysis was performed from September 2010 through June 2018. Laboratory variables were compared with Mann-Whitney tests. Correlations between initial TREC levels and T cell counts were determined by Spearman tests.

Results

Twenty-two transient and 21 persistent TCL infants were identified. Males comprised 68% of the transient and 52% of the persistent TCL cohorts. Whites comprised 23% of the transient and 29% of the persistent cohorts. Median initial TREC levels did not differ (66 vs. 60 TRECs/μL of blood, P = 0.58). The transient cohort had higher median initial CD3⁺ (2135 vs. 1169 cells/μL, P < 0.001), CD4⁺ (1460 vs. 866 cells/μL, P < 0.001), and CD8⁺ (538 vs. 277 cells/μL, P < 0.001) counts. The median age of resolution for the transient cohort was 38 days. Genetic testing revealed 2 genes of interest which warrant further study and several variants of uncertain significance in immunology-related genes in the persistent cohort. 19 transient and 14 persistent subjects received the initial rotavirus and/or MMRV immunization. No adverse reactions to live viral vaccines were reported in either cohort.

Conclusion

Transient and persistent TCL infants differ by demographic, laboratory, and clinical characteristics. Select transient and persistent TCL patients may safely receive live attenuated viral vaccines, but larger confirmatory studies are needed.

中文翻译：

新生儿筛查确定的特发性短暂性和持续性 T 细胞淋巴细胞减少症婴儿的特征——纽约州的单中心经验

目的

新生儿筛查 (NBS) 量化 T 细胞受体切除环 (TREC) 并识别患有 T 细胞淋巴细胞减少症 (TCL) 的婴儿。本研究阐明了对患有短暂性或持续性特发性 TCL 的足月婴儿进行活病毒疫苗接种后的人口统计学、实验室特征、遗传学和临床结果。

方法

从 2010 年 9 月到 2018 年 6 月进行了单中心回顾性分析。将实验室变量与 Mann-Whitney 检验进行了比较。初始 TREC 水平和 T 细胞计数之间的相关性由 Spearman 测试确定。

结果

确定了 22 名短暂性和 21 名持续性 TCL 婴儿。男性占短暂性 TCL 队列的 68% 和持续性 TCL 队列的 52%。白人占短暂队列的 23% 和持续队列的 29%。中位初始 TREC 水平没有差异（66 对 60 TRECs/μL 血液，P = 0.58）。瞬态队列的初始 CD3 ⁺中位数较高（2135 对 1169 个细胞/μL，P < 0.001）、CD4 ⁺（1460 对 866 个细胞/μL，P < 0.001）和 CD8 ⁺（538 对 277 个细胞/μL ,磷 < 0.001) 计数。暂时性队列的中位消退年龄为 38 天。基因检测揭示了 2 个值得进一步研究的感兴趣的基因，以及持续队列中免疫学相关基因中的几个不确定意义的变体。19 名暂时性和 14 名持续性受试者接受了初始轮状病毒和/或 MMRV 免疫接种。两个队列均未报告对活病毒疫苗的不良反应。