Division of Allergy, Immunology, and Rheumatology, University of South Florida, St Petersburg, Fla.
Department of Pediatrics Residency Training Program, University of South Florida, St Petersburg, Fla.
J Allergy Clin Immunol Pract. 2013 Nov-Dec;1(6):583-8. doi: 10.1016/j.jaip.2013.09.007. Epub 2013 Oct 31.
Measurement of T-cell receptor excision circles (TREC) in neonates has allowed for population-based screening of severe combined immunodeficiency and other disorders associated with T-cell lymphopenia. In addition to primary T-cell lymphopenic disorders, secondary causes of T-cell lymphopenia can be diagnosed with TREC analysis. We discuss the diagnostic evaluation of a patient with normal TREC analysis at birth that became abnormal after cardiac surgery. TREC analysis was performed by the Florida State Laboratory. Diagnostic evaluation and treatment were performed at All Children's Hospital, St Petersburg, Florida. We identified a 38-day-old female patient with thoracic duct injury, which caused chylothorax and chylous ascites diagnosed after an abnormal newborn screen. Chylothorax was secondary to thoracic duct injury after cardiac surgery and led to severe lymphopenia and hypogammaglobulinemia. Thoracic duct ligation led to improved lymphocyte counts and normalization of immunoglobulin levels. Secondary causes of lymphopenia are detected with TREC assay that lead to abnormal newborn screen results. Many secondary causes of lymphopenia can be acquired with normal initial newborn screens that become abnormal over time.
对 T 细胞受体切除环(TREC)的测量可用于对严重联合免疫缺陷和其他与 T 细胞淋巴细胞减少相关的疾病进行基于人群的筛查。除了原发性 T 细胞淋巴细胞减少症外,TREC 分析还可诊断继发性 T 细胞淋巴细胞减少症的原因。我们讨论了一名新生儿 TREC 分析正常但在心脏手术后变得异常的患者的诊断评估。TREC 分析由佛罗里达州立实验室进行。诊断评估和治疗在佛罗里达州圣彼得堡的 All Children's Hospital 进行。我们发现了一名 38 天大的女性患者,她因胸导管损伤导致乳糜胸和乳糜性腹水,在异常新生儿筛查后确诊。乳糜胸继发于心脏手术后的胸导管损伤,导致严重的淋巴细胞减少症和低丙种球蛋白血症。胸导管结扎导致淋巴细胞计数增加和免疫球蛋白水平正常化。TREC 检测可发现导致异常新生儿筛查结果的继发性淋巴细胞减少症的原因。许多继发性淋巴细胞减少症的原因可通过正常的初始新生儿筛查来发现,但随着时间的推移会变得异常。