Fiorilli M, Sirianni M C, Pandolfi F, Quinti I, Tosti U, Aiuti F, Goldstein G
Clin Exp Immunol. 1981 Aug;45(2):344-51.
The case of an 18-month-old child, affected by malnutrition, severe interstitial pneumonia and immunological abnormalities is reported. Since the age of 10 months, the infant suffered from severe recurrent infections and failure to thrive. Immunological studies revealed a striking decrease of T lymphocytes and of natural killer (NK) function. Serum immunoglobulins, salivary IgA, natural isohaemagglutinins, Fc-IgG receptor-bearing cells and suppressor T lymphocytes were absent, together with an impaired de novo DNA synthesis after PHA, Con A, PWM and Cowan I strain from Staphylococcus aureus stimulation. In vitro incubation of the patient's lymphocytes with TP-5, a thymopoietin-derived synthetic pentapeptide, resulted in improvement of sheep rosettes, Fc-IgG receptor-positive lymphocytes and NK activity. The child was therefore treated with TP-5 for 8 months and his clinical condition improved as well as the number of T cells, Fc-IgG-positive lymphocytes and NK function. However, humoral immunity remained persistently depressed. We suggest that this child could be classified as affected by 'late-onset severe combined immunodeficiency'. In vitro assays with thymic hormones or synthetic drugs that mimic the action of thymic hormones should be performed and this therapy could be applied in the treatment of some of these heterogeneous syndromes, especially when an immunological reconstitution with bone marrow or fetal graft cannot be attempted.
报告了一名18个月大儿童的病例,该儿童患有营养不良、严重间质性肺炎和免疫异常。自10个月大起,该婴儿就遭受严重反复感染且发育不良。免疫学研究显示T淋巴细胞和自然杀伤(NK)功能显著下降。血清免疫球蛋白、唾液IgA、天然血型凝集素、携带Fc-IgG受体的细胞和抑制性T淋巴细胞均缺失,同时在PHA、Con A、PWM和金黄色葡萄球菌考恩I菌株刺激后,从头DNA合成受损。用胸腺生成素衍生的合成五肽TP-5对患者淋巴细胞进行体外培养,导致羊红细胞花环形成、Fc-IgG受体阳性淋巴细胞和NK活性得到改善。因此,该儿童接受了8个月的TP-5治疗,其临床状况以及T细胞数量、Fc-IgG阳性淋巴细胞和NK功能均有所改善。然而,体液免疫仍持续受到抑制。我们建议该儿童可被归类为患有“迟发性严重联合免疫缺陷”。应进行胸腺激素或模拟胸腺激素作用的合成药物的体外试验,这种疗法可应用于治疗其中一些异质性综合征,尤其是在无法尝试进行骨髓或胎儿移植进行免疫重建时。