探讨Castleman病和TAFRO综合征的临床多样性:日本淋巴结分布模式的多中心研究

IF 10.1 1区 医学 Q1 HEMATOLOGY
Mizuna Otsuka, Tomohiro Koga, Remi Sumiyoshi, Shoichi Fukui, Yuko Kaneko, Takayuki Shimizu, Atsushi Katsube, Shingo Yano, Yasufumi Masaki, Makoto Ide, Hajime Yoshifuji, Masayasu Kitano, Yasuharu Sato, Naoki Sawa, Hiroaki Niiro, Naoya Nakamura, David C. Fajgenbaum, Frits van Rhee, Atsushi Kawakami
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引用次数: 0

摘要

被诊断为Castleman病(CD)和TAFRO综合征(以血小板减少、水肿、发热、骨髓纤维化和器官肿大为特征)的个体表现出广泛的临床症状,包括不同类型的淋巴结肿大、全身炎症和器官功能受损。有些患者可能同时出现CD和TAFRO综合征。在日本多个中心进行的一项回顾性研究检查了321例病例,以确定肿胀淋巴结的数量和位置是否可以预测这些疾病的临床进展和强度。有趣的是,该研究显示TAFRO综合征患者在所有淋巴结区域计数范围内均表现出淋巴结病变。此外,没有特定的临床模式与CD患者受影响的淋巴结区域的数量相关,无论他们是否也患有TAFRO综合征。这些结果增强了我们对CD和TAFRO综合征临床变异性的理解,表明全面的临床评估,而不是仅仅依赖于淋巴结计数,对有效管理这些疾病至关重要。需要进一步的研究来建立可靠的诊断标志物,并在诊断时预测疾病的严重程度,最终改善患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Exploring the Clinical Diversity of Castleman Disease and TAFRO Syndrome: A Japanese Multicenter Study on Lymph Node Distribution Patterns

Exploring the Clinical Diversity of Castleman Disease and TAFRO Syndrome: A Japanese Multicenter Study on Lymph Node Distribution Patterns

Individuals diagnosed with Castleman disease (CD) and TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, bone marrow fibrosis, and organomegaly) displays a wide range of clinical symptoms, including varying patterns of lymph node enlargement, systemic inflammation, and impaired organ function. Some patients may present with both CD and TAFRO syndrome concurrently. A retrospective study conducted across multiple centers in Japan examined 321 cases to determine if the quantity and position of swollen lymph nodes could forecast the clinical progression and intensity of these conditions. Interestingly, the study revealed that patients with TAFRO syndrome exhibited lymphadenopathy across all ranges of lymph node region counts. Moreover, no specific clinical patterns were associated with the number of affected lymph node regions in CD patients, regardless of whether they also had TAFRO syndrome. These results enhance our understanding of the clinical variability in CD and TAFRO syndrome, suggesting that a comprehensive clinical evaluation, rather than relying solely on lymph node count, is crucial for effectively managing these conditions. Additional studies are required to establish reliable diagnostic markers and to predict disease severity at the time of diagnosis, ultimately improving patient outcomes.

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来源期刊
CiteScore
15.70
自引率
3.90%
发文量
363
审稿时长
3-6 weeks
期刊介绍: The American Journal of Hematology offers extensive coverage of experimental and clinical aspects of blood diseases in humans and animal models. The journal publishes original contributions in both non-malignant and malignant hematological diseases, encompassing clinical and basic studies in areas such as hemostasis, thrombosis, immunology, blood banking, and stem cell biology. Clinical translational reports highlighting innovative therapeutic approaches for the diagnosis and treatment of hematological diseases are actively encouraged.The American Journal of Hematology features regular original laboratory and clinical research articles, brief research reports, critical reviews, images in hematology, as well as letters and correspondence.
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