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疣状表皮发育不良:对源自家庭接触者的可透析白细胞提取物(转移因子)治疗的反应

Epidermodysplasia verruciformis: response to therapy with dialyzable leukocyte extract (transfer factor) derived from household contacts.

作者信息

Vasily D B, Miller O F, Fudenberg H H, Goust J M, Wilson G B

出版信息

J Clin Lab Immunol. 1984 May;14(1):49-57.

PMID:6086930
Abstract

Dialyzable leukocyte extracts (DLE) have been used to treat a variety of antigen selective, and broad spectrum immunodeficiency diseases with sometimes encouraging results. We describe here the clinical and laboratory responses to DLE therapy of 2 patients with epidermodysplasia verruciformis (EV), a chronic cutaneous infection with a variety of human papilloma viruses. One patient with longstanding (30 yr) disease and no improvement to previous therapy showed gradual yet definite resolution of extensive verrucae planae, plaque, tinea-versicolor-like, and tumor lesions scattered over his entire integument. Cessation of DLE therapy for a short time resulted in recurrence of partially regressed lesions and also in the development of new tumors in this patient. The second patient, a grandson of the first patient, with minimal disease showed no progression of the disease during DLE prophylaxis. A third subject (brother of patient number 2) received no DLE and served as a control. All 3 subjects demonstrated severely depressed levels of suppressor T cells, a defect in cell-mediated immunity that has not been hitherto reported in patients with EV. Finally, evidence is presented for a possible X-linked recessive mode of inheritance for susceptibility to EV.

摘要

可透析白细胞提取物(DLE)已被用于治疗多种抗原选择性和广谱免疫缺陷疾病,有时取得了令人鼓舞的结果。我们在此描述了2例疣状表皮发育不良(EV)患者对DLE治疗的临床和实验室反应,EV是一种由多种人乳头瘤病毒引起的慢性皮肤感染。一名患有长期(30年)疾病且先前治疗无改善的患者,其广泛分布于全身皮肤的扁平疣、斑块、花斑癣样病变和肿瘤性病变逐渐且明确地消退。在该患者中,短期停止DLE治疗导致部分消退的病变复发,并且还出现了新的肿瘤。第二名患者是第一名患者的孙子,病情较轻,在接受DLE预防治疗期间疾病未进展。第三名受试者(患者2的兄弟)未接受DLE治疗,作为对照。所有3名受试者均表现出抑制性T细胞水平严重降低,这是一种细胞介导免疫缺陷,此前在EV患者中尚未见报道。最后,有证据表明EV易感性可能存在X连锁隐性遗传模式。

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