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卡斯特尔曼病与卡波西肉瘤:文献综述及病例系列

Castleman Disease and Kaposi Sarcoma: A Review of the Literature and a Case Series.

作者信息

Denaro Nerina, Brambilla Lucia, Scarfì Federica, Tourlaki Athanasia, Muscatello Antonio, Solinas Cinzia, Rampi Nicolò, Bandera Alessandra, Garrone Ornella

机构信息

Oncology Unit, Fondazione Ca' Granda-Policlinico di Milano, 20122 Milan, Italy.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

出版信息

J Clin Med. 2025 Sep 18;14(18):6563. doi: 10.3390/jcm14186563.

Abstract

Castleman disease and Kaposi sarcoma (KS) are both associated with infection by human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). This virus plays a critical role in the pathogenesis of both conditions, particularly in immunocompromised individuals, such as those with HIV/AIDS. Multicentric Castleman disease (MCD) generally presents with systemic inflammatory symptoms, lymphadenopathy, and organ dysfunction, while Kaposi sarcoma typically appears as vascular tumors on the skin, with occasional involvement of mucous membranes or internal organs. We present four clinical cases, with concurrent KS and MCD, treated with chemotherapy and rituximab, with a satisfactory response. We highlighted the essential role of prompt investigation of systemic or inflammatory manifestations (fever, vital parameter alterations such as palpitation, high breath frequency, edema, and kidney impairment) as underlined in our case series, which might underscore possible complications. Multiorgan failure, opportunistic infections, or rapid clinical deterioration might occur if the diagnosis is not adequately assessed. Therefore, this paper emphasizes the importance of timely diagnosis, as it enables the prompt initiation of appropriate antiviral, immunomodulatory, or oncologic therapies-interventions that can significantly improve outcomes and may be life-saving in advanced or aggressive disease presentations.

摘要

卡斯特曼病和卡波西肉瘤(KS)均与人类疱疹病毒8(HHV - 8)感染有关,HHV - 8也被称为卡波西肉瘤相关疱疹病毒(KSHV)。该病毒在这两种疾病的发病机制中都起着关键作用,尤其是在免疫功能低下的个体中,如患有艾滋病毒/艾滋病的患者。多中心卡斯特曼病(MCD)通常表现为全身炎症症状、淋巴结病和器官功能障碍,而卡波西肉瘤通常表现为皮肤上的血管肿瘤,偶尔累及黏膜或内脏器官。我们报告了4例同时患有KS和MCD的临床病例,采用化疗和利妥昔单抗治疗,取得了满意的疗效。我们强调了及时调查全身或炎症表现(发热、生命体征改变,如心悸、呼吸频率加快、水肿和肾功能损害)的重要作用,正如我们病例系列中所强调的,这可能会突出潜在的并发症。如果诊断评估不充分,可能会发生多器官功能衰竭、机会性感染或临床快速恶化。因此,本文强调了及时诊断的重要性,因为它能够迅速启动适当的抗病毒、免疫调节或肿瘤治疗——这些干预措施可以显著改善预后,对于晚期或侵袭性疾病表现可能是救命的。

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