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广泛淋巴结病患者的卡波西肉瘤是否预示着多中心 Castleman 病?

Does Kaposi's sarcoma predict multicentric Castleman disease in the presence of generalized lymphadenopathy?

机构信息

Staten Island University Hospital, 475 Seaview Ave, Staten Island, NY 10305, USA.

出版信息

Med Oncol. 2012 Jun;29(2):1109-13. doi: 10.1007/s12032-011-9886-z. Epub 2011 Apr 7.

Abstract

Multisystemic Castleman disease (MCD) can be associated with HHV8 infection, which involves the mantle zone of follicles. This condition results in an increase number of HHV8-positive (HHV8+) plasmablasts that multiply and amalgamate to form plasmablastic B-cell lymphoma. All previously reported cases of HIV-positive patients with MCD were coinfected with HHV8. Twelve cases of HIV- MCD that are HHV8+ were encountered in the literature, three of them developed lymphoma, and none of those cases were reported to have Kaposi's sarcoma (KS). We report a unique case of HIV-negative elderly woman with preexisting KS that presented to the hospital with recurring nausea, vomiting, and fever, assessment revealed diffuse lymphadenopathy. Axillary lymph node biopsy showed HHV8+ MCD with foci of microlymphoma. Despite the treatment with high-dose steroids, she developed multisystem failure that lead to her death.

摘要

多系统 Castleman 病(MCD)可与 HHV8 感染相关,其涉及滤泡套区。这种情况会导致大量 HHV8 阳性(HHV8+)浆母细胞的增加,这些浆母细胞增殖并融合形成浆母细胞性 B 细胞淋巴瘤。所有先前报道的 HIV 阳性 MCD 患者均合并 HHV8 感染。文献中报道了 12 例 HIV-MCD 患者 HHV8 阳性,其中 3 例发展为淋巴瘤,且均未报告卡波西肉瘤(KS)。我们报告了一例独特的 HIV 阴性老年女性病例,该患者患有先前存在的 KS,因反复恶心、呕吐和发热就诊,评估发现弥漫性淋巴结病。腋窝淋巴结活检显示 HHV8+MCD 伴微淋巴瘤灶。尽管接受了大剂量类固醇治疗,但她仍出现多系统衰竭,最终导致死亡。

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