Fajgenbaum David C, Uldrick Thomas S, Bagg Adam, Frank Dale, Wu David, Srkalovic Gordan, Simpson David, Liu Amy Y, Menke David, Chandrakasan Shanmuganathan, Lechowicz Mary Jo, Wong Raymond S M, Pierson Sheila, Paessler Michele, Rossi Jean-François, Ide Makoto, Ruth Jason, Croglio Michael, Suarez Alexander, Krymskaya Vera, Chadburn Amy, Colleoni Gisele, Nasta Sunita, Jayanthan Raj, Nabel Christopher S, Casper Corey, Dispenzieri Angela, Fosså Alexander, Kelleher Dermot, Kurzrock Razelle, Voorhees Peter, Dogan Ahmet, Yoshizaki Kazuyuki, van Rhee Frits, Oksenhendler Eric, Jaffe Elaine S, Elenitoba-Johnson Kojo S J, Lim Megan S
Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.
National Cancer Institute, National Institutes of Health, Bethesda, MD.
Blood. 2017 Mar 23;129(12):1646-1657. doi: 10.1182/blood-2016-10-746933. Epub 2017 Jan 13.
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 of MCD and 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 11 Minor Criteria with at least 1 laboratory abnormality, and exclusion of infectious, malignant, and autoimmune disorders that can mimic iMCD. Characteristic histopathologic features may include a constellation of regressed or hyperplastic germinal centers, follicular dendritic cell prominence, hypervascularization, and polytypic plasmacytosis. Laboratory and clinical Minor Criteria include elevated C-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.
人疱疹病毒8型（HHV - 8）阴性的特发性多中心Castleman病（iMCD）是一种罕见且危及生命的疾病，其特征包括全身炎症症状、多克隆淋巴细胞增殖、血细胞减少以及由细胞因子风暴（常涉及白细胞介素-6）引起的多器官系统功能障碍。iMCD占所有Castleman病病例的三分之一至二分之一，可发生于任何年龄的个体。准确诊断具有挑战性，因为目前尚无标准诊断标准或诊断生物标志物，且与恶性、自身免疫和感染性疾病存在显著重叠。一个由来自8个国家的34名儿科和成人病理学及临床专家组成的国际工作组，其中包括2名也是iMCD患者 的医生，被召集起来制定iMCD诊断标准。该工作组回顾了244例病例的数据，召开了两次会议，并在15个月（2015年6月至2016年9月）内完善了标准。拟议的共识标准要求同时具备主要标准（特征性淋巴结组织病理学和多中心淋巴结病）、11项次要标准中的至少2项且至少有1项实验室异常，以及排除可模仿iMCD的感染性、恶性和自身免疫性疾病。特征性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、血管增生和多型性浆细胞增多。实验室和临床次要标准包括C反应蛋白或红细胞沉降率升高、贫血、血小板减少或血小板增多、低白蛋白血症、肾功能不全或蛋白尿、多克隆高球蛋白血症。iMCD共识诊断标准将有助于实现一致的诊断、恰当的治疗以及合作研究。
