成功治疗特发性多中心Castleman病伴TAFRO和肾脏受累：病例报告和文献综述
Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.
摘要(中文译文)
特发性多中心Castleman病（iMCD）是一种临床表现异质性的罕见血液系统疾病，症状从轻微到严重甚至危及生命不等。iMCD的一种严重亚型以血小板减少、全身性水肿（包括胸腔积液和腹水）、发热、网状纤维骨髓纤维化或肾功能不全以及器官肿大为特征（iMCD-TAFRO）。由于iMCD-TAFRO起病迅速、肾功能损害严重且死亡风险高，早期识别和治疗至关重要。然而，由于缺乏诊断性生物标志物，且其症状与多种淋巴增殖性疾病、感染性疾病和风湿性疾病相重叠，iMCD-TAFRO的诊断可能较为困难。尽管iMCD-TAFRO的确切病因尚不明确，但白细胞介素（IL）-6已被确定为关键的病理驱动因子，IL-6拮抗剂司妥昔单抗被推荐为一线治疗。虽然许多iMCD-TAFRO患者存在肾功能异常并需要肾脏科诊疗，但描述该病肾脏组织病理学和肾脏受累情况的证据有限。本文综述了该疾病的状态及最新进展，重点关注肾脏组织病理学和iMCD-TAFRO肾脏损伤的机制。我们还呈现并讨论了一例iMCD-TAFRO合并急性肾衰竭患者的病例报告。我们的目的是引起对iMCD-TAFRO肾脏受累谱系的关注，并提高对这一罕见疾病早期识别和适当治疗重要性的认识。
Abstract
Idiopathic multicentric Castleman disease (iMCD) is a rare hematological disease of heterogenous presentation, with symptoms ranging from mild to severe and life-threatening. A severe subtype of iMCD is characterized by thrombocytopenia, anasarca (including pleural effusion and ascites), fever, reticulin myelofibrosis or renal dysfunction, and organomegaly (iMCD-TAFRO). Early identification and treatment of iMCD-TAFRO is crucial because of its rapid onset, severe renal dysfunction and high mortality risk. However, diagnosing iMCD-TAFRO can be difficult due to a lack of diagnostic biomarkers and symptoms that overlap with a variety of lymphoproliferative, infectious, and rheumatological diseases. Although the exact etiology of iMCD-TAFRO is unknown, interleukin (IL)-6 has been identified as a critical pathological driver, and the IL-6 antagonist siltuximab is recommended as first-line treatment. While many patients with iMCD-TAFRO have abnormal kidney function and will require nephrology care, evidence describing kidney histopathology and kidney involvement in this condition is limited. Herein, we review the disease state and recent advances with a focus on renal histopathology and the mechanisms of kidney injury in iMCD-TAFRO. We also present and discuss a case report of a patient with iMCD-TAFRO and acute kidney failure. Our aim is to draw attention to the spectrum of kidney involvement in iMCD-TAFRO, as well as raise awareness to the importance of early recognition and appropriate treatment of this rare condition.
如何引用
AMA
Ahmed I Kamal, Hima Doppalapudi, Thien Ho, Salem Vilayet, Vishu Pasham, Milos Budisavljevic, et al. Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.. Renal failure. 2026; doi:10.1080/0886022X.2026.2671463.
APA
Ahmed I Kamal, Hima Doppalapudi, Thien Ho, Salem Vilayet, Vishu Pasham, Milos Budisavljevic, et al (2026). Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.. Renal failure. https://doi.org/10.1080/0886022X.2026.2671463
GB/T 7714
Ahmed I Kamal, Hima Doppalapudi, Thien Ho, Salem Vilayet, Vishu Pasham, Milos Budisavljevic, et al. Successful treatment of idiopathic multicentric Castleman disease with TAFRO and kidney involvement: case report and literature review.[J]. Renal failure, 2026 doi:10.1080/0886022X.2026.2671463.
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