Orange Jordan S, Geha Raif S, Bonilla Francisco A
Division of Immunology, Children's Hospital, Enders Building, 300 Longwood Avenue, Boston, MA 02115, USA.
J Pediatr. 2003 Aug;143(2):243-9. doi: 10.1067/S0022-3476(03)00305-6.
To assess for immunodeficiency in patients with hypogammaglobulinemia in the setting of draining acute chylothorax.
Humoral and cellular immunity was evaluated in 8 patients with chylothorax. Chylous fluid was also analyzed to document cellular losses. Data regarding clinical course and immunologic characteristics were reviewed retrospectively.
All patients had hypogammaglobulinemia (IgG=179+/-35 mg/dL) as well as lymphopenia (985+/-636 cells/mm(3)). T cells were decreased and natural killer cells increased in peripheral blood. The converse was found in chylous fluid. The ratio of CD3+/CD45RA+ naive: CD3+/CD45RO+ memory T cells was greater in chyle than in peripheral blood. In vitro proliferative responses to antigens and mitogens were similar to control subjects, and previously immunized patients maintained evidence of protective vaccine-specific humoral immunity. To treat hypogammaglobulinemia, patients received intravenous immunoglobulin (IVIG) to maintain IgG within normal range; 6 of 8 patients had serious infections before receiving IVIG compared with 4 of 8 patients during the period of IVIG administration.
Draining chylothorax resulted in IgG and lymphocyte depletion with preferential retention of memory T cells and natural killer cells in the circulation. Overall, protective-specific antibody levels and T cell function were maintained. IVIG administration did not lead to discernible protection from infectious complications in this small group of patients.
评估在急性乳糜胸引流情况下低丙种球蛋白血症患者的免疫缺陷情况。
对8例乳糜胸患者的体液免疫和细胞免疫进行评估。对乳糜液也进行分析以记录细胞损失情况。回顾性分析有关临床病程和免疫特征的数据。
所有患者均有低丙种球蛋白血症(IgG = 179±35mg/dL)以及淋巴细胞减少(985±636个细胞/mm³)。外周血中T细胞减少而自然杀伤细胞增加。在乳糜液中情况则相反。乳糜中CD3⁺/CD45RA⁺初始T细胞与CD3⁺/CD45RO⁺记忆T细胞的比例高于外周血。对抗原和丝裂原的体外增殖反应与对照受试者相似,且既往免疫的患者维持有保护性疫苗特异性体液免疫的证据。为治疗低丙种球蛋白血症,患者接受静脉注射免疫球蛋白(IVIG)以使IgG维持在正常范围内;8例患者中有6例在接受IVIG前发生严重感染,而在IVIG给药期间8例患者中有4例发生严重感染。
急性乳糜胸引流导致IgG和淋巴细胞耗竭,循环中记忆T细胞和自然杀伤细胞优先保留。总体而言,保护性特异性抗体水平和T细胞功能得以维持。在这一小群患者中,IVIG给药并未带来明显的针对感染并发症的保护作用。