Mremi Alex, Ndale Eliasa, Stephen Leonard, Mkwizu Elifuraha, Kilonzo Kajiru
Department of Pathology, Kilimanjaro Christian Medical Centre, Moshi, Tanzania.
Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania.
SAGE Open Med Case Rep. 2023 May 23;11:2050313X231175720. doi: 10.1177/2050313X231175720. eCollection 2023.
Castleman's disease is a rare lympho-proliferative disease entity characterized by variable clinical presentations, distinctive histological manifestations, and prognosis. Its incidence and etiology are unclear. An interplay of HIV and human herpesvirus-8 has been implicated. Although its localized variety is benign, other types can be multifocal with adverse systemic manifestations. Human herpesvirus-8 Castleman's disease affects mainly HIV-positive individuals; however, individuals who are immunocompromised from other causes can also be affected, thus necessitating investigations for HIV. Herein, we report two patients presenting with long-standing lymphadenopathy. Histopathology, immunohistochemical testing and clinico-pathological correlation confirmed the diagnosis of Castleman's disease. The patients were successfully treated with surgery and/or rituximab. They were symptoms free in the subsequent follow-up visits. A brief review of the literature is also provided.
卡斯特尔曼病是一种罕见的淋巴增殖性疾病实体,其临床症状多样,组织学表现独特,预后情况不一。其发病率和病因尚不清楚。人们认为人类免疫缺陷病毒(HIV)与人类疱疹病毒8型之间存在相互作用。尽管其局限性类型为良性,但其他类型可能为多灶性,伴有不良的全身表现。人类疱疹病毒8型卡斯特尔曼病主要影响HIV阳性个体;然而,其他原因导致免疫功能低下的个体也可能受到影响,因此有必要对HIV进行检测。在此,我们报告两名长期存在淋巴结病的患者。组织病理学、免疫组化检测及临床病理相关性分析确诊为卡斯特尔曼病。这两名患者通过手术和/或利妥昔单抗治疗成功。在随后的随访中,他们均无症状。本文还对相关文献进行了简要综述。