Division of Allergy & Immunology, The Children's Hospital of Philadelphia, Pa.
Division of Cardiology, The Children's Hospital of Philadelphia, Pa.
J Allergy Clin Immunol Pract. 2016 May-Jun;4(3):491-6. doi: 10.1016/j.jaip.2015.11.034. Epub 2016 Feb 18.
Congenital cardiac anomalies are associated with immunologic perturbations. Surgical thymectomy, thoracic duct manipulation, and protein- losing enteropathy (PLE), a condition related to stressed Fontan hemodynamics, presumably contribute to low peripheral absolute lymphocyte counts (ALCs) and quantitative immunoglobulins. Clinical significance of lymphopenia and hypogammaglobulinemia in single-ventricle survivors requires additional study.
Although immunologic laboratory anomalies are common in this population, we hypothesize that clinically significant immunodeficiency requiring intervention is rarely required.
A retrospective chart review of the immunologic parameters of patients enrolled in the Single Ventricle Survivorship Program (SVSP) at the Children's Hospital of Philadelphia was performed.
The age range of the 178 SVSP patients was 3 to 26 years, with a median of 10.8 years. Most of the SVSP patients had some degree of lymphopenia. In the non-PLE group, the range of ALCs varied from 530 to 5322 cells/μL, with 17 patients without PLE maintaining an ALC of less than 1000 cells/μL. Among those with PLE, the median ALC and the IgG level were lower (672 cells/μL and 200 mg/dL, respectively) than in those without (1610 cells/μL and 868 mg/dL, respectively). Despite lymphopenia in the majority, few were severely clinically affected: 24% had delayed clearance of cutaneous viral infections, 63% had atopy, and 1 died of EBV-associated Hodgkin lymphoma. Immunoglobulin replacement was clinically indicated for 3 patients, 1 of whom had common variable immunodeficiency. Four patients with normal splenic function were treated with daily antibiotic prophylaxis.
Patients with repaired single-ventricle physiology often demonstrate T-cell lymphopenia and hypogammaglobulinemia. A significant portion of patients without PLE also have lymphopenia. The most common clinical manifestation was delayed clearance of cutaneous viral infections, but significant systemic opportunistic infections were not seen despite laboratory abnormalities and lack of antimicrobial prophylaxis.
先天性心脏异常与免疫紊乱有关。胸腺切除术、胸导管操作以及蛋白丢失性肠病（PLE）——一种与Fontan 血流动力学压力相关的疾病——可能导致外周血绝对淋巴细胞计数（ALC）和定量免疫球蛋白降低。单心室幸存者淋巴细胞减少和低丙种球蛋白血症的临床意义需要进一步研究。
尽管该人群中免疫实验室异常很常见，但我们假设需要干预的临床显著免疫缺陷很少见。
对费城儿童医院单心室存活者计划（SVSP）入组患者的免疫参数进行回顾性图表审查。
178 例 SVSP 患者的年龄范围为 3 至 26 岁，中位数为 10.8 岁。大多数 SVSP 患者存在一定程度的淋巴细胞减少。在非 PLE 组中，ALC 范围为 530 至 5322 个细胞/μL，17 例无 PLE 的患者 ALC 低于 1000 个细胞/μL。在有 PLE 的患者中，中位数 ALC 和 IgG 水平较低（分别为 672 个细胞/μL 和 200mg/dL），而无 PLE 的患者分别为 1610 个细胞/μL 和 868mg/dL。尽管大多数患者存在淋巴细胞减少，但很少有严重的临床影响：24%的患者有皮肤病毒感染清除延迟，63%的患者有特应性，1 例患者死于 EBV 相关霍奇金淋巴瘤。有 3 名患者临床需要免疫球蛋白替代治疗，其中 1 名患者为普通可变免疫缺陷。4 名脾功能正常的患者接受了每日抗生素预防治疗。
修复后的单心室生理患者常表现为 T 细胞淋巴细胞减少和低丙种球蛋白血症。无 PLE 的患者中有相当一部分也存在淋巴细胞减少。最常见的临床表现是皮肤病毒感染清除延迟，但尽管存在实验室异常和缺乏抗菌预防，仍未观察到严重的全身机会性感染。
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