Bonagura V R, Cunningham-Rundles S, Edwards B L, Ilowite N T, Wedgwood J F, Valacer D J
Department of Pediatrics, Schneider Children's Hospital, New Hyde Park, New York 11042.
Clin Immunol Immunopathol. 1989 May;51(2):216-31. doi: 10.1016/0090-1229(89)90021-4.
A toddler with common variable hypoimmunoglobulinemia (CVH), inflammatory bowel disease, and recurrent Pneumocystis carinii pneumonia (PCP) on intravenous gammaglobulin (IVIG) replacement was evaluated for a combined cellular immunodeficiency. He had a normal number of circulating T-cells, natural killer (NK) cells, T-cell subset percentages, and his peripheral blood mononuclear (PBM)-derived B-cell number was low. PBM mitogen blastogenesis and mixed lymphocyte reaction (MLR) were normal. MLR activated T-cells expressed class I and II MHC antigens, interleukin 2 (IL-2), and B-cell growth factor (IL-5)-related receptors. The patient's T-cells induced control B-cell maturation with pokeweed mitogen (PWM-PC), and did not suppress PWM-PC production by allogeneic PBM. Bone marrow (BM) CD19+ B-cell number varied between 10 and 44% of all PBM, and the BM B-cell-enriched fraction failed to differentiate to PWM-PC with autologous or allogeneic T-cell help. The NK activity assayed using K562 target cells was deficient, 9.2 x 7.7% (6.9-9.2%) pt, control 35.9 x 35.8% (16.3-67.2% +/- 12.8). In the presence of interferon-alpha, 800 U/ml, the patient's NK activity increased to 17.2 x 14.9% (12.6-17.2%), control 35.9 x 51.0% (36.5-72.3% +/- 12.0). The patient's cell-mediated lympholysis of HLA nonidentical, allogeneic stimulators was normal. Maintaining trough serum IgG levels above 500 mg/dl was required to suppress recurrent PCP. This functional NK deficiency may be relevant to the development of recurrent PCP in IVIG-treated CVH patients.
一名患有常见变异型低丙种球蛋白血症（CVH）、炎症性肠病且在接受静脉注射丙种球蛋白（IVIG）替代治疗时反复发生卡氏肺孢子虫肺炎（PCP）的幼儿接受了联合细胞免疫缺陷评估。他的循环T细胞、自然杀伤（NK）细胞数量、T细胞亚群百分比均正常，但其外周血单个核（PBM）来源的B细胞数量较低。PBM有丝分裂原刺激增殖反应和混合淋巴细胞反应（MLR）正常。MLR激活的T细胞表达I类和II类主要组织相容性复合体（MHC）抗原、白细胞介素2（IL-2）以及B细胞生长因子（IL-5）相关受体。患者的T细胞可通过美洲商陆有丝分裂原（PWM）诱导对照B细胞成熟（PWM-PC），但不能抑制同种异体PBM产生PWM-PC。骨髓（BM）中CD19+B细胞数量占所有PBM的10%至44%不等，且富含BM B细胞的部分在自体或同种异体T细胞的辅助下无法分化为PWM-PC。使用K562靶细胞检测的NK活性不足，患者为9.2×7.7%（6.9 - 9.2%），对照组为35.9×35.8%（16.3 - 67.2%±12.8）。在存在800 U/mlα干扰素的情况下，患者的NK活性增加至17.2×14.9%（12.6 - 17.2%），对照组为35.9×51.0%（36.5 - 72.3%±12.0）。患者对HLA不匹配的同种异体刺激物的细胞介导淋巴细胞溶解作用正常。为抑制PCP复发，需要将血清IgG谷值水平维持在500 mg/dl以上。这种功能性NK缺陷可能与IVIG治疗的CVH患者反复发生PCP有关。
