The full spectrum of Castleman disease: 273 patients studied over 20 years
The full spectrum of Castleman disease: 273 patients studied over 20 years
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DOI：
10.1111/bjh.15019
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发表时间：
2018-01-01
影响因子：
6.5
通讯作者：
Galicier, Lionel
中科院分区：
文献类型：
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作者：
Oksenhendler, Eric;Boutboul, David;Galicier, Lionel
自从1956年首次描述以来，Castleman病(CD)的谱系已经相当广泛。最近，一个国际协作工作组就CD的诊断标准和分类达成了共识。我们在此报告273例淋巴结组织病理学与CD一致的患者，并调查新建立的诊断标准。这些具有Castleman样组织病理学的患者中有20人被排除在分析之外，因为他们被诊断为排他性疾病(18人患有血液系统恶性肿瘤)。在253例患者中，57例为单中心CD(UCD)，169例为人类疱疹病毒8型(HHV-8+MCD)多中心CD，其中人类免疫缺陷病毒(HIV)感染140例，非HIV感染29例，HHV-8阴性/特发性多中心CD(IMCD)27例。2-(F-18)氟-2-脱氧-D-葡萄糖正电子发射断层扫描/计算机断层扫描在62例患者中可用于疾病的分期/分型和排除相关淋巴瘤。UCD主要与透明血管的组织病理学特征有关，多数患者无症状。在我们最初诊断为IMCD的27名患者中，26名符合新建立的诊断标准。IMCD和HHV-8+MCD患者表现相似的特征，包括发热、脾肿大、细胞减少和炎症症状。然而，在HHV-8+MCD中，这种疾病更具侵袭性，特别是在艾滋病毒感染的患者中。
The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Twenty of these patients with Castleman-like histopathology were removed from analyses, because they were diagnosed with an exclusionary disorder (18 with haematological malignancy). Among the 253 remaining patients, 57 were considered unicentric CD (UCD), 169 were multicentric CD associated with Human Herpesvirus 8 (HHV-8+MCD), including 140 patients with human immunodeficiency virus (HIV) infection and 29 patients without HIV infection, and 27 were HHV-8 negative/idiopathic multicentric CD (iMCD). 2-(F-18)fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography was useful in 62 patients for staging/classification of the disease and for excluding associated lymphoma. UCD was mainly associated with hyaline-vascular histopathological features, and most patients were asymptomatic. Of the 27 patients that we had originally diagnosed with iMCD, 26 met the newly established diagnostic criteria. Patients with iMCD and HHV-8+ MCD demonstrated similar characteristics, including fever, splenomegaly, cytopenia and inflammatory symptoms. However, the disease was more aggressive in HHV-8+ MCD, particularly in HIV-infected patients.