Castleman Disease and Kaposi Sarcoma: A Review of the Literature and a Case Series
Nerina Denaro, L. Brambilla, Federica Scarfì, Athanasia Tourlaki, Antonio Muscatello, Cinzia Solinas, Nicolò Rampi, Alessandra Bandera, Ornella Garrone
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.