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维斯科特-奥尔德里奇综合征。转移因子治疗的结果。

The Wiskott-Aldrich syndrome. Results of transfer factor therapy.

作者信息

Spitler L E, Levin A S, Stites D P, Fudenberg H H, Pirofsky B, August C S, Stiehm E R, Hitzig W H, Gatti R A

出版信息

J Clin Invest. 1972 Dec;51(12):3216-24. doi: 10.1172/JCI107148.

Abstract

12 patients with Wiskott-Aldrich syndrome were treated with therapeutic doses of transfer factor in an attempt to induce cellular immunity. Clinical improvement was noted after transfer factor therapy in 7 of the 12 patients treated. Because this disease has a variable course and temporary spontaneous improvement can occur, the observed improvement cannot necessarily be attributed to the transfer factor. However, in two patients repeated remissions consistently followed transfer factor administration on repeated occasions. This included freedom from infections, regression of splenomegaly, and clearing of eczema. An unexpected finding was a decrease in bleeding in 3 of the 10 patients who had bleeding. Conversion of skin reactivity was obtained in all seven patients who clinically seemed to respond to transfer factor. In vitro studies performed after the administration of transfer factor demonstrated that the lymphocytes of the patients now produced migration inhibitory factor in response to appropriate test antigens, but did not undergo increased radioactive thymidine incorporation in response to the same antigens. A defect in the monocyte IgG receptors has been found in certain patients with the disease, and the current study shows that all patients with defective monocyte IgG receptors responded to transfer factor, whereas only one patient with normal receptors showed any response. This test may thus prove to be useful in predicting the results of transfer factor therapy in patients with Wiskott-Aldrich syndrome, although evaluation of a larger series of patients will be necessary to confirm this point. We conclude that cellular immunity can be induced, that there appears to be clinical benefit in certain patients with Wiskott-Aldrich syndrome by the use of transfer factor, and that this mode of therapy warrents trial in these patients and others with defects of cellular immunity.

摘要

12例威斯科特-奥尔德里奇综合征患者接受了治疗剂量的转移因子治疗,试图诱导细胞免疫。在接受治疗的12例患者中,7例在转移因子治疗后出现临床改善。由于这种疾病病程多变,可能会出现暂时的自发改善,所以观察到的改善不一定归因于转移因子。然而,有2例患者在多次重复给予转移因子后持续出现缓解。这包括无感染、脾肿大消退和湿疹消退。一个意外发现是,10例有出血症状的患者中有3例出血情况有所减轻。临床上似乎对转移因子有反应的所有7例患者均出现了皮肤反应性的转变。给予转移因子后进行的体外研究表明,患者的淋巴细胞现在能对适当的试验抗原产生迁移抑制因子,但对相同抗原的放射性胸腺嘧啶核苷掺入并未增加。在某些患有该疾病的患者中发现了单核细胞IgG受体缺陷,目前的研究表明,所有单核细胞IgG受体缺陷的患者对转移因子均有反应,而只有1例受体正常的患者有任何反应。因此,尽管需要评估更多患者系列来证实这一点,但该试验可能被证明对预测威斯科特-奥尔德里奇综合征患者转移因子治疗的结果有用。我们得出结论,细胞免疫可以被诱导,使用转移因子对某些威斯科特-奥尔德里奇综合征患者似乎有临床益处,并且这种治疗方式值得在这些患者以及其他细胞免疫缺陷患者中进行试验。

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