Clinical features and treatment of 7 Chinese TAFRO syndromes from 96 de novo Castleman diseases: a 10-year retrospective study
Clinical features and treatment of 7 Chinese TAFRO syndromes from 96 de novo Castleman diseases: a 10-year retrospective study
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96 种新发 Castleman 病中的 7 种中国 TAFRO 综合征的临床特征和治疗：一项 10 年回顾性研究
DOI：
10.1007/s00432-019-03120-w
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发表时间：
2020-01
影响因子：
3.6
通讯作者：
Jin Jie
中科院分区：
文献类型：
--
作者：
Zhang Yi;Suo Shan-Shan;Yang Han-Jin;Zhou Xin-Ping;You Liang-Shun;Yu Wen-Juan;Wang Zhao-Ming;Jin Jie
背景Castleman病（CD）是一种罕见的多克隆淋巴组织增生性疾病，病因不明. TAFRO综合征目前被认为是CD的一种特殊亚型，对临床医生来说仍然是一个巨大的挑战。方法为了阐明TAFRO综合征在中国的临床特征和管理，我们回顾性分析了2008年至2017年在我中心诊断和治疗的96例HIV阴性CD患者（52例单中心CD和44例多中心CD）。结果7例TAFRO综合征患者中，男3例，女4例，中位年龄53岁。主要症状为血小板减少（7/7）、全身水肿（7/7）、发热（4/7）、肾功能不全（7/7）、脏器肿大（6/7）。1例患者接受皮质类固醇单药治疗，1例患者接受RD（利妥昔单抗，地塞米松）治疗，5例患者接受CHOP/COP样化疗作为一线治疗，其中2例患者联合利妥昔单抗治疗。4例患者因进行性肾衰竭需要血液透析或CRRT。TAFRO综合征的结局明显差于其他类型的CD。虽然3例患者早期治疗后好转，4例患者死于疾病进展，只有1例患者达到完全解决后，改变来那度胺为基础的regime.ConclusionsThis研究表明，TAFRO综合征是更严重的，有更多的全身症状比其他iMCD，大多数情况下，需要积极治疗，他们的预后差。以来那度胺为基础的治疗方案有望成为治疗TAFRO综合征的新方法。
BackgroundCastleman disease (CD) is a rare polyclonal lymphoproliferative disorder with unknown etiology. TAFRO syndrome is now regarded as a specific subtype of CD, and is still a huge challenge for clinicians.MethodsTo clarify the clinical features and management of TAFRO syndrome in China, we retrospectively analyzed 96 patients with HIV-negative CD (52 with unicentric CD and 44 with multicentric CD), who were diagnosed and treated at our center between 2008 and 2017. Specially, we systematically reviewed the 7 TAFRO syndrome cases based on the 2015 criteria proposed by Masaki.ResultsAmong the 7 cases, there were 3 men and 4 women, and the median age was 53 years. The main symptoms included thrombocytopenia (7/7), anasarca (7/7), fever (4/7), renal dysfunction (7/7), and organomegaly (6/7). One patient was treated with corticosteroid monotherapy, one received RD (Rituximab, dexamethasone), and 5 received CHOP/COP like chemotherapy as first-line treatment, 2 of the 5 combined with Rituximab. Four patients needed hemodialysis or CRRT because of progressive renal failure. The outcome for TAFRO syndrome was significantly worse compared to other types of CD. Although 3 patients improved after early treatment, 4 patients died due to disease progression, and only one patient achieved complete resolution of all the symptoms after changing to lenalidomide based regimen.ConclusionsThis study reveals that TAFRO syndrome is more severe and has more systemic symptoms than other iMCD, most cases need active treatment, and their prognoses are poor. Lenalidomide based regimen may be as a promising new therapy for TAFRO syndrome.
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DOI：
10.1038/s41572-021-00317-7
发表时间：
2021-11-25
期刊：
Nature reviews. Disease primers
影响因子：
--
作者：
通讯作者：
--
影响因子：
6.5
作者：
Oksenhendler, Eric;Boutboul, David;Galicier, Lionel
通讯作者：
Galicier, Lionel
影响因子：
2.1
作者：
Fujiwara, Shiho;Mochinaga, Hiromi;Nosaka, Kisato
通讯作者：
Nosaka, Kisato
影响因子：
1.2
作者：
Kojima, Masaru;Nakamura, Naoya;Nakamura, Shigeo
通讯作者：
Nakamura, Shigeo
影响因子：
--
作者：
P. Szturz;Z. Adam;Z. Řehák;R. Koukalová;L. Křen;M. Moulis;M. Krejci;J. Mayer
通讯作者：
P. Szturz;Z. Adam;Z. Řehák;R. Koukalová;L. Křen;M. Moulis;M. Krejci;J. Mayer