Zhou Qian-Yun
Department of Critical Care Medicine, Peking University Shougang Hospital, Beijing 100144, China.
World J Clin Cases. 2022 Feb 16;10(5):1536-1547. doi: 10.12998/wjcc.v10.i5.1536.
Castleman disease (CD) and TAFRO syndrome are very rare in clinical practice. Most clinicians, especially non-hematological clinicians, do not know enough about the two diseases, so it often leads to misdiagnosis or missed diagnosis.
To explore the clinical features and diagnosis of CD and TAFRO syndrome.
We retrospectively collected the clinical and laboratory data of 39 patients who were diagnosed with CD from a single medical center.
Clinical classification identified 18 patients (46.15%) with unicentric Castleman disease (UCD) and 21 patients (53.85%) with multicentric Castleman disease (MCD), the latter is further divided into 13 patients (33.33%) with idiopathic multicentric Castleman disease-not otherwise specified (iMCD-NOS) and 8 patients (20.51%) with TAFRO syndrome. UCD and iMCD are significantly different in clinical manifestations, treatment, and prognosis. However, a few patients with MCD were diagnosed as UCD in their early stage. There was a correlation between two of Thrombocytopenia, anasarca and elevated creatinine, which were important components of TAFRO syndrome. In UCD group, the pathologies of lymph modes were mostly hyaline vascular type (13/18, 72.22%), however plasma cell type or mixed type could also appear. In iMCD-NOS group and TAFRO syndrome group, the pathologies of lymph mode shown polarity of plasma cell type and hyaline vascular type respectively. Compared with patients with TAFRO syndrome, patients with iMCD-NOS were diagnosed more difficultly.
The clinical and pathological types of CD are not completely separate, there is an intermediate situation or mixed characteristics between two ends of clinical and pathological types. The clinical manifestations of patients with CD are determined by their pathological type. TAFRO syndrome is a special subtype of iMCD with unique clinical manifestations.
Castleman病（CD）和TAFRO综合征在临床实践中非常罕见。大多数临床医生，尤其是非血液科临床医生，对这两种疾病了解不足，因此常导致误诊或漏诊。
探讨CD和TAFRO综合征的临床特征及诊断方法。
我们回顾性收集了来自单一医疗中心的39例诊断为CD患者的临床和实验室数据。
临床分类显示，18例（46.15%）为单中心Castleman病（UCD），21例（53.85%）为多中心Castleman病（MCD），后者进一步分为13例（33.33%）特发性多中心Castleman病-未另行指定（iMCD-NOS）和8例（20.51%）TAFRO综合征。UCD和iMCD在临床表现、治疗及预后方面有显著差异。然而，少数MCD患者在早期被诊断为UCD。血小板减少、全身水肿和肌酐升高这TAFRO综合征的重要组成部分中的两项之间存在相关性。在UCD组中，淋巴结病理大多为透明血管型（13/18，72.22%），但也可能出现浆细胞型或混合型。在iMCD-NOS组和TAFRO综合征组中，淋巴结病理分别表现为浆细胞型和透明血管型的极性。与TAFRO综合征患者相比，iMCD-NOS患者诊断更困难。
CD的临床和病理类型并非完全分开，在临床和病理类型的两端之间存在中间情况或混合特征。CD患者的临床表现由其病理类型决定。TAFRO综合征是iMCD的一种特殊亚型，具有独特的临床表现。
