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TAFRO 综合征伴或不伴淋巴结病的病理生理学差异比较。

Comparison of pathophysiological differences in TAFRO syndrome with or without lymphadenopathy.

机构信息

Division of Hematology, Niigata University of Pharmacy and Medical and Life Sciences, Faculty of Medical Technology, Niigata City, Niigata, Japan.

出版信息

J Clin Exp Hematop. 2024 Sep 28;64(3):208-215. doi: 10.3960/jslrt.24026. Epub 2024 Jul 31.

DOI:10.3960/jslrt.24026
PMID:39085127
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11528249/
Abstract

In diagnosing TAFRO syndrome, lymph node histology often may not be evaluated due to inapparent lymphadenopathy. In this study, we analyzed the differences in the pathophysiology of TAFRO syndrome with or without lymphadenopathy. We used an anonymous questionnaire to survey 70 hematologists at 50 hospitals in the Kanto Koshinetsu area of Japan from February to April 2020. We received 31 responses and collected 26 cases with TAFRO syndrome. Compared to cases with or without lymph node biopsy, clinical features and laboratory test findings in both groups were not significantly different, except for stronger renal insufficiency found in those without biopsy. It was also revealed that clinical features and laboratory test findings had no significant differences between the cases with and without lymphadenopathy. However, renal insufficiency was more pronounced in those without lymphadenopathy. There were no significant differences in pathophysiology between cases with or without lymphadenopathy in the group that did not undergo lymph node biopsy. In the treatment strategies, no significant differences were found dependent on lymphadenopathy. This study shows that lymphadenopathy in TAFRO syndrome may be secondary to inflammation and unrelated to the underlying disease.

摘要

在诊断 TAFRO 综合征时,由于淋巴结病不明显,通常可能不会评估淋巴结组织病理学。在这项研究中,我们分析了有无淋巴结病的 TAFRO 综合征的病理生理学差异。我们于 2020 年 2 月至 4 月期间,使用匿名问卷对日本关东甲信越地区的 50 家医院的 70 名血液科医生进行了调查,共收到 31 份回复,并收集了 26 例 TAFRO 综合征。与有或无淋巴结活检的病例相比,两组的临床特征和实验室检查结果无明显差异,但无活检组的肾功能不全更为严重。此外,结果还表明,有或无淋巴结病的病例之间的临床特征和实验室检查结果无明显差异。然而,无淋巴结病的病例肾功能不全更为明显。未行淋巴结活检的病例中,有无淋巴结病组的病理生理学无明显差异。在治疗策略方面,也未发现与淋巴结病相关的显著差异。本研究表明,TAFRO 综合征中的淋巴结病可能是炎症引起的,与潜在疾病无关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c310/11528249/ecff18da47cd/jslrt-64-208-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c310/11528249/ecff18da47cd/jslrt-64-208-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c310/11528249/ecff18da47cd/jslrt-64-208-g001.jpg

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本文引用的文献

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Ann Hematol. 2022 Mar;101(3):485-490. doi: 10.1007/s00277-022-04762-6. Epub 2022 Jan 19.
2
Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease.特发性多中心 Castleman 病伴血小板减少、浮肿、发热、网状纤维纤维化、肾功能不全和器官肿大(TAFRO)临床亚型的验证性国际定义。
Am J Hematol. 2021 Oct 1;96(10):1241-1252. doi: 10.1002/ajh.26292. Epub 2021 Jul 28.
3
2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome.
2019年TAFRO综合征更新的诊断标准和疾病严重程度分类。
Int J Hematol. 2020 Jan;111(1):155-158. doi: 10.1007/s12185-019-02780-1. Epub 2019 Nov 28.
4
Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan.日本多中心和单中心Castleman病及TAFRO综合征的流行病学分析
J Clin Exp Hematop. 2019 Dec 22;59(4):175-178. doi: 10.3960/jslrt.19021. Epub 2019 Nov 8.
5
Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?TAFRO 综合征是否为特发性多中心 Castleman 病的一个亚型?
Am J Hematol. 2019 Sep;94(9):975-983. doi: 10.1002/ajh.25554. Epub 2019 Jun 21.
6
Proposal of new clinical diagnostic criteria for POEMS syndrome.POEMS 综合征新的临床诊断标准的建议。
J Neurol Neurosurg Psychiatry. 2019 Feb;90(2):133-137. doi: 10.1136/jnnp-2018-318514. Epub 2018 Oct 2.
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The clinicopathological comparison among nodal cases of idiopathic multicentric Castleman disease with and without TAFRO syndrome.特发性多中心 Castleman 病伴与不伴 TAFRO 综合征的淋巴结病例的临床病理比较。
Hum Pathol. 2018 Jul;77:130-138. doi: 10.1016/j.humpath.2018.04.001. Epub 2018 Apr 21.
8
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[Proposed diagnostic criteria, disease severity classification, and treatment strategy for a novel disorder; TAFRO syndrome].[一种新型疾病;TAFRO综合征的拟诊标准、疾病严重程度分类及治疗策略]
Rinsho Ketsueki. 2016;57(10):2029-2037. doi: 10.11406/rinketsu.57.2029.
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