Sicherer S H, Cabana M D, Perlman E J, Lederman H M, Matsakis R R, Winkelstein J A
Eudowood Division of Allergy and Immunology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Pediatr Allergy Immunol. 1998 Feb;9(1):49-52. doi: 10.1111/j.1399-3038.1998.tb00301.x.
Thymoma has been associated with both humoral immunodeficiency and cellular immunodeficiency, but the latter association has never been described in the pediatric age group. We report a 15-year-old female with thymoma, recalcitrant oropharyngeal candidiasis, recurrent generalized cutaneous herpes simplex virus type 2 infection, recurrent pneumonia and myasthenia gravis. Pathology of the thymic lesion showed a 10x5x6 cm extensively hyalinized mass with residual regions of spindle cell predominant and lymphocyte-rich thymoma. There was no evidence of humoral immunodeficiency but there was clinical and laboratory evidence of cellular immunodeficiency with cutaneous anergy and absence of T cell proliferation to Candida antigen. Six weeks after the thymoma was resected, she was no longer anergic and Candida proliferation was normal, although she continued to experience infections. This is the first reported pediatric patient with an association of cellular immunodeficiency with thymoma.
胸腺瘤与体液免疫缺陷和细胞免疫缺陷均有关联,但后者的关联在儿童年龄组中从未被描述过。我们报告一名15岁女性,患有胸腺瘤、顽固性口咽念珠菌病、复发性全身性2型单纯疱疹病毒感染、复发性肺炎和重症肌无力。胸腺病变的病理显示一个10×5×6厘米的广泛透明变性肿块,残余区域以梭形细胞为主,富含淋巴细胞的胸腺瘤。没有体液免疫缺陷的证据,但有细胞免疫缺陷的临床和实验室证据,表现为皮肤无反应性以及对念珠菌抗原有T细胞增殖缺失。胸腺瘤切除六周后,她不再无反应,念珠菌增殖正常,尽管她仍继续经历感染。这是首例报道的细胞免疫缺陷与胸腺瘤相关的儿科患者。