Joshi V V, Oleske J M, Connor E M
Department of Pathology, Children's Hospital of New Jersey, New Jersey Medical School, Newark 07107.
Pediatr Pathol. 1990;10(1-2):155-65. doi: 10.3109/15513819009067104.
The lesions observed in biopsy and autopsy material from children with the acquired immunodeficiency syndrome (AIDS) can be divided into three pathogenetic categories: primary lesions related to infection by human immunodeficiency virus (HIV) (e.g., lymphoreticular system and brain); lesions due to the sequelae of HIV infection (e.g., opportunistic infections, pulmonary lymphoid lesions, etc.); and lesions of undetermined pathogenesis (e.g., renal lesions, cardiomyopathy, etc.). The role of morphologic studies in AIDS in understanding the pathogenesis of the various lesions and their clinical implications are discussed by describing the following examples among others. Study of the thymus enabled us to distinguish AIDS from some congenital immune deficiency syndromes. Thymic injury contributes to immunodeficiency in AIDS. Its apparent irreversibility will have to be considered in the long-term management of children with AIDS when specific effective therapy for HIV becomes available. Demonstration of HIV--like particles in the characteristic giant cells in the brain has been instrumental in the recognition of HIV encephalopathy. Biopsy is helpful in the rapid diagnosis of opportunistic infections (OIs). Autopsy study of OIs has shown involvement of clinically unsuspected organs, such as the adrenals. Characterization of the pulmonary lymphoid lesions led to their inclusion as a diagnostic criterion for AIDS in children. Progression of pulmonary lymphoid lesions to a lymphoproliferative disorder was demonstrated at autopsy. Recognition of lesions such as cardiomyopathy and arteriopathy at autopsy should alert clinicians to suspect these disorders during life.
在获得性免疫缺陷综合征(AIDS)患儿的活检和尸检材料中观察到的病变可分为三类致病类型:与人类免疫缺陷病毒(HIV)感染相关的原发性病变(如淋巴网状系统和脑);HIV感染后遗症所致病变(如机会性感染、肺淋巴样病变等);以及发病机制不明的病变(如肾脏病变、心肌病等)。本文通过列举以下例子等方式,讨论了形态学研究在AIDS中对于理解各种病变的发病机制及其临床意义所起的作用。对胸腺的研究使我们能够将AIDS与某些先天性免疫缺陷综合征区分开来。胸腺损伤导致AIDS患者免疫缺陷。当针对HIV的特效疗法出现时,在AIDS患儿的长期管理中必须考虑到其明显的不可逆性。在脑内特征性巨细胞中发现HIV样颗粒有助于识别HIV脑病。活检有助于快速诊断机会性感染(OIs)。对OIs的尸检研究显示,临床上未怀疑的器官(如肾上腺)也会受累。对肺淋巴样病变的特征描述使其被纳入儿童AIDS的诊断标准。尸检显示肺淋巴样病变进展为淋巴增殖性疾病。在尸检中识别出心肌病和动脉病等病变,应提醒临床医生在患儿生前怀疑这些疾病。