HIV-negative HHV8-positive multicentric Castleman's disease coexistent with atypical Kaposi's sarcoma

Anna Scattone, Giacomo Loseto, Biagina Gisella Mennuni, Valentina Mastrandrea, Rosalba Buquicchio, Caterina Foti, Francesco Alfredo Zito, Raffaele Filotico
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Abstract

The co-occurence of multicentric Castleman's disease (MCD) and Kaposi's sarcoma (KS) represents a rare clinical entity, mostly observed in individuals infected with the human immunodeficiency virus (HIV). Human herpesvirus 8 (HHV-8) is attributed a crucial etiological role in both conditions. This study presents the case of a 75-year-old woman who manifested an angiomatous lesion on the right thigh and erythematous firm plaques on the trunk and limbs, accompanied by asthenia, weight loss, and recurrent febrile episodes. Serological markers for HIV yielded negative results. Contrast-enhanced computed tomography (CT) and fluorine-18 fluorodeoxyglucose positron emission tomography (18 F FDG PET/CT) revealed multiple enlarged and intensely hypermetabolic lymph nodes in the supraclavicular, cervical, thoracic, and abdominopelvic regions. Subsequent excisional biopsy and immunohistochemical analysis confirmed the diagnosis of HIV-negative HHV8-positive MCD coexisting with KS.

Abstract Image

hiv阴性hhv8阳性多中心Castleman病与非典型卡波西肉瘤共存
多中心Castleman病(MCD)和卡波西肉瘤(KS)的共同发生是一种罕见的临床实体,主要见于感染人类免疫缺陷病毒(HIV)的个体。人类疱疹病毒8 (HHV-8)在这两种疾病中都起着至关重要的病原学作用。本研究报告了一位75岁的女性,她表现为右大腿血管瘤病变,躯干和四肢红斑性硬化斑块,伴有虚弱,体重减轻和反复发热发作。HIV的血清学标记结果为阴性。对比增强计算机断层扫描(CT)和氟-18氟脱氧葡萄糖正电子发射断层扫描(18 F FDG PET/CT)显示锁骨上、颈椎、胸椎和腹骨盆区域有多个肿大且高度代谢的淋巴结。随后的切除活检和免疫组织化学分析证实了hiv阴性hhv8阳性MCD与KS共存的诊断。
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