Aydintug Yavuz Sinan, Bayar Gurkan Rasit, Ozkan Aydin, Gunhan Omer, Musabak Ugur
Department of Oral and Maxillofacial Surgery, Gulhane Military Medical Academy, Ankara, Turkey.
Department of Pathology, Gulhane Military Medical Academy, Ankara, Turkey.
J Dent Sci. 2016 Mar;11(1):103-106. doi: 10.1016/j.jds.2012.10.005. Epub 2012 Dec 17.
Thymomas with immunodeficiency (formerly Good's syndrome) are a rare acquired disease of combined T- and B-cell immunodeficiency accompanying a thymoma. Recurrent opportunistic infections associated with disorders of both humoral and cell-mediated immunity frequently accompany this rare primary, adult-onset immunodeficiency. This is a report of a case of a thymoma with immunodeficiency in a 65-year-old male patient who developed recurrent oral herpetic infections. He consulted us about recurrent vesiculo-ulcerative lesions on his tongue, lower lip, and buccal mucosa. Results of laboratory examinations indicated hypogammaglobulinemia accompanied by diminished B cells in the peripheral blood, which is consistent with the characteristic features of a thymoma with immunodeficiency. After a diagnosis confirming herpes simplex virus infection, systemic antiviral therapy was administered, which was effective for his vesiculo-ulcerative lesions at follow-up. When an intractable infection accompanied by a thymoma is encountered, increased awareness about the clinical and immunological profiles of this primary immunodeficiency may help in its early diagnosis, thereby preventing mortality.
伴免疫缺陷的胸腺瘤(既往称古德综合征)是一种罕见的获得性疾病,伴有胸腺瘤的T细胞和B细胞联合免疫缺陷。这种罕见的原发性成人起病免疫缺陷常伴有与体液免疫和细胞介导免疫紊乱相关的反复机会性感染。本文报告一例65岁男性伴免疫缺陷胸腺瘤患者,该患者反复发生口腔疱疹感染。他就舌部、下唇和颊黏膜反复出现的水疱性溃疡病变向我们咨询。实验室检查结果显示低丙种球蛋白血症,外周血B细胞减少,这与伴免疫缺陷胸腺瘤的特征相符。确诊为单纯疱疹病毒感染后,给予全身抗病毒治疗,随访时其水疱性溃疡病变有效。当遇到伴有胸腺瘤的难治性感染时,提高对这种原发性免疫缺陷的临床和免疫学特征的认识可能有助于早期诊断,从而预防死亡。