Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?
Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?
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DOI：
10.1002/ajh.25554
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发表时间：
2019-09-01
影响因子：
12.8
通讯作者：
Masaki, Yasufumi
中科院分区：
文献类型：
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作者：
Fujimoto, Shino;Sakai, Tomoyuki;Masaki, Yasufumi
Castleman病（CD）是一种罕见的淋巴细胞增生性疾病，可以是单中心或多中心。多中心性CD （MCD）进一步细分为人类疱疹病毒8型相关、POEMS综合征相关和特发性（iMCD）。TAFRO综合征是一种新发现的病因不明的疾病，以血小板减少、贫血、发热、网状蛋白性骨髓纤维化、肾功能障碍和器官肿大为特征。TAFRO综合征有时被认为是iMCD的一个亚型（TAFRO-iMCD），而没有TAFRO综合征的iMCD被认为是“非其他特异性”（iMCD- nos）。然而，一部分TAFRO综合征患者在没有淋巴结活检的情况下被诊断（TAFRO综合征未证实iMCD； taafro -w/op-iMCD）。为了明确iMCD-NOS、tafroo - imcd和tafroo -w/op-iMCD的临床特征，我们回顾性分析了从建立TAFRO综合征概念的多中心协作回顾性研究数据库中提取的220例患者。其中iMCD-NOS 87例，TAFRO-iMCD 63例，TAFRO-w/op-iMCD 19例。三组患者均表现为贫血、低白蛋白血症、血清c反应蛋白和白细胞介素-6水平升高。tafroo - imcd组和tafroo -w/opiMCD组在临床、实验室和预后特征上没有显著差异。然而，iMCD-NOS组表现为多克隆高γ -球蛋白血症。iMCD-NOS组和taro组患者5年生存率分别为100%和66.5%（后者在前几个月明显下降）。iMCD-NOS和TAFRO-iMCD分别表现为典型的浆细胞和混合型组织学。因此，iMCD可分为两种不同的亚型，iMCD- nos和tafo -iMCD。因此，tafroo - imcd和tafroo -w/op-iMCD可被视为同一实体，需要及时诊断和重症监护。
Castleman disease (CD) is a rare lymphoproliferative disorder that can be unicentric or multicentric. Multicentric CD (MCD) is further subdivided into human herpesvirus type-8-associated, POEMS syndrome-associated, and idiopathic (iMCD). TAFRO syndrome is a newly identified disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. The TAFRO syndrome is sometimes regarded as a subtype of iMCD (TAFRO-iMCD), whereas iMCD without TAFRO syndrome is considered "not otherwise specified" (iMCD-NOS). However, a proportion of patients with TAFRO syndrome have been diagnosed without lymph node biopsies (TAFRO syndrome without proven iMCD; TAFRO-w/op-iMCD). To clarify the clinical features of iMCD-NOS, TAFRO-iMCD, and TAFRO-w/op-iMCD, we retrospectively analyzed 220 patients extracted from the database of the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO Syndrome. The patients included 87 with iMCD-NOS, 63 with TAFRO-iMCD, and 19 with TAFRO-w/op-iMCD. Patients in all three groups exhibited anemia, hypoalbuminemia, and elevated serum C-reactive protein and interleukin-6 levels. No significant differences in clinical, laboratory, and prognostic features were noted between the TAFRO-iMCD, and TAFRO-w/opiMCD groups. However, the iMCD-NOS group exhibited polyclonal hyper-gamma-globulinemia. The five-year survival rates of patients in the iMCD-NOS and TAFRO-involved groups were 100% and 66.5%, respectively (dropping markedly during the first few months in the latter). The iMCD-NOS and the TAFRO-iMCD samples typically showed plasma cell and mixed-type histologies, respectively. Thus, iMCD can be classified into two distinct subtypes, iMCD-NOS and TAFRO-iMCD. As such, TAFRO-iMCD and TAFRO-w/op-iMCD may be considered the same entity, requiring prompt diagnosis and intensive care.