Castleman 病 (CD) 包括一组罕见且异质的血液疾病，包括单中心 (UCD) 和多中心 (MCD) 形式，后者进一步细分为 HHV8-MCD、POEMS-MCD 和特发性 MCD (iMCD)。然而，根据 Castleman 疾病协作网络指南，CD 的诊断只能通过临床医生和病理学家之间的合作来实现。我们应用这些临床和病理标准，并对 48 名诊断为意大利成人和儿童的患者进行回顾性队列实施克隆性测试具有 CD 样组织学特征的反应性淋巴结炎。我们在 60% (29/48) 的病例中确诊为 CD，包括 12 例 (41%) UCD 和 17 例 (59%)；5 例 HHV8-MCD，3 例 POEMS-MCD和九个 iMCD) MCD。其余 19 例（40%）多发性淋巴结肿大中，5 例（26%）属于自身免疫性疾病，1 例（5%）属于自身免疫性淋巴增殖性疾病，1 例（5%）属于 IgG4 相关疾病，11 例（83%）其中 1 例 (5%) 为反应性淋巴结炎，1 例 (5%) 为淋巴结边缘区淋巴瘤。我们的研究强调了采用多学科方法治疗具有 CD 样特征的反应性淋巴结炎的重要性，以便获得明确的诊断并选择适当的治疗方法。© 2023 作者。英国血液学杂志由英国血液学会和约翰·威利出版

Castleman disease (CD) comprises a group of rare and heterogeneous haematological disorders, including unicentric (UCD) and multicentric (MCD) forms, the latter further subdivided into HHV8-MCD, POEMS-MCD and idiopathic-MCD (iMCD). However, according to the Castleman Disease Collaborative Network guidelines, the diagnosis of CD can only be achieved through collaboration between clinicians and pathologists.We applied these clinical and pathological criteria and implement with clonality testing to a retrospective cohort of 48 adult and paediatric Italian patients diagnosed with reactive lymphadenitis with CD-like histological features.We confirmed the diagnosis of CD in 60% (29/48) of the cases, including 12 (41%) UCD and 17 (59%; five HHV8-MCD, three POEMS-MCD and nine iMCD) MCD. Of the remaining 19 cases (40%) with multiple lymphadenopathy, 5 (26%) were classified as autoimmune diseases, 1 (5%) as autoimmune lymphoproliferative disorder, 1 (5%) as IgG4-related disease, 11 (83%) as reactive lymphadenitis and 1 (5%) as nodal marginal zone lymphoma.Our study emphasizes the importance of the multidisciplinary approach to reactive lymphadenitis with CD-like features in order to achieve a definitive diagnosis and choose the appropriate treatment.© 2023 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.