Sikora Mariusz, Dąbrowska-Leonik Nel, Buda Piotr, Wolska-Kuśnierz Beata, Jahnz-Różyk Karina, Pac Małgorzata, Więsik-Szewczyk Ewa
Department of Internal Medicine, Pneumonology, Allergology, Clinical Immunology and Rare Diseases, Military Institute of Medicine-National Research Institute, 04-141 Warsaw, Poland.
Department of Immunology, Children's Memorial Health Institute, 04-730 Warsaw, Poland.
J Clin Med. 2025 Apr 18;14(8):2799. doi: 10.3390/jcm14082799.
Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder with diverse clinical presentations, often posing significant diagnostic challenges. We report the case of a 20-year-old woman who first presented with fever of unknown origin (FUO) at the age of 14, followed by the development of abdominal lymphadenopathy. We conducted a comprehensive review of her clinical course, diagnostic workup, treatment response, and outcomes. Additionally, we performed a literature review of CD focusing on pathophysiology, classification, diagnostic approaches, and treatment strategies. Extensive investigations performed in the meantime excluded infectious and autoimmune causes. Histopathological analysis of the excised lymph nodes ruled out malignancy and confirmed idiopathic multicentric Castleman disease. Treatment with tocilizumab, an IL-6 receptor antagonist, resulted in rapid resolution of clinical symptoms, normalization of inflammatory markers, and sustained remission. With the final diagnosis established and treatment initiated, she was transitioned at the age of 18 from a pediatric immunology to an adult clinical immunology center. The presented case highlights the importance of considering iMCD in the differential diagnosis of FUO, especially in adolescents, and the efficacy of targeted therapies in managing this challenging disease. A multidisciplinary approach involving clinical, laboratory, imaging, and histopathological evaluation is essential for accurate diagnosis. IL-6 pathway inhibition represents an effective targeted therapy for iMCD, capable of inducing sustained remission in this challenging disease.
特发性多中心Castleman病（iMCD）是一种罕见的淋巴增殖性疾病，临床表现多样，常常带来重大的诊断挑战。我们报告了一例20岁女性病例，她14岁时首次出现不明原因发热（FUO），随后出现腹部淋巴结肿大。我们对她的临床病程、诊断检查、治疗反应及结果进行了全面回顾。此外，我们对Castleman病进行了文献综述，重点关注病理生理学、分类、诊断方法及治疗策略。在此期间进行的广泛检查排除了感染和自身免疫性病因。切除淋巴结的组织病理学分析排除了恶性肿瘤，确诊为特发性多中心Castleman病。使用IL-6受体拮抗剂托珠单抗治疗后，临床症状迅速缓解，炎症标志物恢复正常，病情持续缓解。随着最终诊断的确立和治疗的开始，她在18岁时从儿科免疫科转诊至成人临床免疫科。该病例突出了在FUO的鉴别诊断中，尤其是在青少年中考虑iMCD的重要性，以及靶向治疗在管理这种具有挑战性疾病方面的疗效。涉及临床、实验室、影像学和组织病理学评估的多学科方法对于准确诊断至关重要。抑制IL-6通路是治疗iMCD的一种有效靶向疗法，能够在这种具有挑战性的疾病中诱导持续缓解。
