International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease
International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease
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DOI：
10.1182/blood-2016-10-746933
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发表时间：
2017-03-23
期刊：
影响因子：
20.3
通讯作者：
Lim, Megan S.
中科院分区：
文献类型：
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作者：
Fajgenbaum, David C.;Uldrick, Thomas S.;Lim, Megan S.
人类疱疹病毒-8 （HHV-8）阴性，特发性多中心Castleman病（iMCD）是一种罕见的危及生命的疾病，涉及全身炎症症状，多克隆淋巴细胞增生，细胞减少和多器官系统功能障碍，由细胞因子风暴引起，通常包括白细胞介素-6。iMCD占所有mcd病例的三分之一到一半，可发生在任何年龄的个体中。准确诊断具有挑战性，因为目前没有标准的诊断标准或诊断生物标志物，并且与恶性、自身免疫性和感染性疾病有显著重叠。召集了一个国际工作组，由来自8个国家的34名儿童和成人疾病及相关疾病的病理学和临床专家组成，其中包括2名同时也是儿童疾病的医生，以制定儿童疾病诊断标准。工作组审查了244例病例的数据，在15个月（2015年6月至2016年9月）期间达到了两次并完善了标准。建议的共识标准要求主要标准（特征性淋巴结组织病理学和多中心淋巴结病），11个次要标准中至少2个伴有至少1个实验室异常，并排除可模拟iMCD的感染性、恶性和自身免疫性疾病。特征性的组织病理学特征可能包括一系列退化或增生的生发中心、滤泡树突状细胞突出、血管增生和多型浆细胞增多症。实验室和临床轻微标准包括c反应蛋白升高或红细胞沉降率、贫血、血小板减少或血小板增多、低白蛋白血症、肾功能障碍或蛋白尿、多克隆性高γ球蛋白血症、体质症状、肝脾肿大、积液或水肿、爆发性樱桃血管瘤病或紫色丘疹、淋巴细胞间质性肺炎。iMCD共识诊断标准将促进一致的诊断、适当的治疗和合作研究。
Human herpesvirus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction caused by a cytokine storm often including interleukin-6. iMCD accounts for one third to one half of all cases ofMCDand can occur in individuals of any age. Accurate diagnosis is challenging, because no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders. An international working group comprising 34 pediatric and adult pathology and clinical experts in iMCD and related disorders from 8 countries, including 2 physicians that are also iMCD patients, was convened to establish iMCD diagnostic criteria. The working group reviewed data from 244 cases, met twice, and refined criteria over 15 months (June 2015 to September 2016). The proposed consensus criteria require both Major Criteria (characteristic lymph node histopathology and multicentric lymphadenopathy), at least 2 of 11Minor Criteria with at least 1 laboratory abnormality, and exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. Characteristic histopathologic featuresmay include a constellation of regressed or hyperplastic germinal centers, follicular dendritic cell prominence, hypervascularization, and polytypic plasmacytosis. Laboratory and clinical Minor Criteria include elevatedC-reactive protein or erythrocyte sedimentation rate, anemia, thrombocytopenia or thrombocytosis, hypoalbuminemia, renal dysfunction or proteinuria, polyclonal hypergammaglobulinemia, constitutional symptoms, hepatosplenomegaly, effusions or edema, eruptive cherry hemangiomatosis or violaceous papules, and lymphocytic interstitial pneumonitis. iMCD consensus diagnostic criteria will facilitate consistent diagnosis, appropriate treatment, and collaborative research.