Watts R G, Kelly D R
Department of Pediatrics, University of Alabama, Birmingham 35233.
Med Pediatr Oncol. 1990;18(3):246-51. doi: 10.1002/mpo.2950180318.
An 8-year-old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.
一名8岁男性接受了包膜性胸腺瘤切除术。四个月后，他出现了播散性、致命性水痘。在终末期疾病期间对其免疫状态进行评估，发现低丙种球蛋白血症和淋巴细胞减少，符合古德综合征（胸腺瘤伴免疫缺陷）的诊断。这是首例报道的儿童古德综合征病例，也是首例与古德综合征相关的致命性水痘病例。本文讨论了这种罕见的儿科肿瘤与免疫缺陷的组合情况。尽管进行了特异性抗病毒治疗，但水痘在免疫功能低下的宿主中仍然是一种致命疾病。
