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感染HIV的儿科患者黏膜相关淋巴组织病变谱:6例临床病理研究

The spectrum of mucosa-associated lymphoid tissue lesions in pediatric patients infected with HIV: A clinicopathologic study of six cases.

作者信息

Joshi V V, Gagnon G A, Chadwick E G, Berard C W, McClain K L, Leach C T, Jenson H B, Murphy S B

机构信息

Department of Pathology and Laboratory Medicine, East Carolina University School of Medicine, Greenville, North Carolina, USA.

出版信息

Am J Clin Pathol. 1997 May;107(5):592-600. doi: 10.1093/ajcp/107.5.592.

DOI:10.1093/ajcp/107.5.592
PMID:9128273
Abstract

Mucosa-associated lymphoid tissue (MALT) lesions in nonimmunocompromised individuals include reactive lymphoid proliferations and both low- and high-grade lymphoid neoplasms. These lesions occur at extranodal mucosal sites, such as the gastrointestinal tract, bronchus, salivary gland, and other locations. The spectrum of MALT lesions in children with HIV infection had not been previously described. In this study, six cases that demonstrated the spectrum of MALT lesions in pediatric patients, aged 28 months to 23 years, who had HIV infection were described. Half the patients acquired the infection perinatally, and half acquired it by transfusion. Mucosal sites of involvement included the salivary gland (4 patients), bronchiolar mucosa (2 patients), and oropharyngeal mucosa (1 patient). One patient had lesions in lung and oropharynx sequentially; all others had involvement of solitary sites. The histologic diagnoses included myoepithelial sialadenitis (MESA), MESA with low-grade MALT lymphoma, typical low-grade MALT lymphoma, diffuse large cell lymphoma (DLCL), and atypical pulmonary lymphoid hyperplasia and lymphoid interstitial pneumonitis complex. The two cases of high-grade DLCL were confined to mucosal sites (tonsil and parotid); in one of these patients, a previous biopsy specimen showed a MALT lesion with low-grade features. In two cases, quantitation of the Epstein-Barr virus (EBV) genome by the polymerase chain reaction showed a very high copy number in peripheral blood mononuclear cells but a low copy number in the MALT lesion, which suggested that MALT lesions may not be directly associated with EBV infection. Two patients who had high-grade tumors (DLCL) were successfully treated with chemotherapy and radiation therapy. The remaining patients, all of whom had low-grade MALT lesions, received either corticosteroids or alpha-interferon or no specific therapy; in all patients, the lesions followed an indolent clinical course. Clinicians and pathologists should be alert to the possibility that MALT lesions, including MALT lymphomas, may be present in children who have AIDS.

摘要

非免疫功能低下个体的黏膜相关淋巴组织(MALT)病变包括反应性淋巴组织增生以及低级别和高级别淋巴肿瘤。这些病变发生于结外黏膜部位,如胃肠道、支气管、唾液腺及其他部位。此前尚未描述过HIV感染儿童的MALT病变谱。在本研究中,描述了6例年龄在28个月至23岁之间感染HIV的儿科患者的MALT病变谱。半数患者在围产期感染,半数通过输血感染。受累的黏膜部位包括唾液腺(4例患者)、细支气管黏膜(2例患者)和口咽黏膜(1例患者)。1例患者先后在肺部和口咽部出现病变;其他所有患者均为单一部位受累。组织学诊断包括肌上皮涎腺炎(MESA)、伴低级别MALT淋巴瘤的MESA、典型的低级别MALT淋巴瘤、弥漫大B细胞淋巴瘤(DLCL)以及非典型肺淋巴组织增生和淋巴间质肺炎复合体。2例高级别DLCL局限于黏膜部位(扁桃体和腮腺);其中1例患者之前的活检标本显示为具有低级别特征的MALT病变。在2例病例中,通过聚合酶链反应对爱泼斯坦-巴尔病毒(EBV)基因组进行定量分析显示,外周血单个核细胞中的拷贝数非常高,但MALT病变中的拷贝数低,这表明MALT病变可能与EBV感染无直接关联。2例患有高级别肿瘤(DLCL)的患者通过化疗和放疗成功治愈。其余患者均患有低级别MALT病变,接受了皮质类固醇或α干扰素治疗或未接受特定治疗;所有患者的病变临床过程均较为惰性。临床医生和病理医生应警惕艾滋病儿童可能存在MALT病变,包括MALT淋巴瘤。

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