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TAFRO 综合征仅用泼尼松治疗有效:评估白介素-6 的变化。

TAFRO Syndrome That Responded to Prednisolone-only Treatment: Evaluating Changes in IL-6.

机构信息

Division of Gastroenterology, Department of Medicine, Kurume University School of Medicine, Japan.

Department of Pathology, Kurume University School of Medicine, Japan.

出版信息

Intern Med. 2022 Oct 1;61(19):2967-2972. doi: 10.2169/internalmedicine.9160-21. Epub 2022 Feb 26.

DOI:10.2169/internalmedicine.9160-21
PMID:35228431
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9593160/
Abstract

Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is a systemic inflammatory disorder characterized by the above-mentioned symptoms. Because of the similarity in phenotypes between TAFRO syndrome and decompensated liver cirrhosis, an accurate diagnosis is often difficult. We herein report a 62-year-old Japanese patient with TAFRO syndrome who was misdiagnosed with intractable ascites associated with liver cirrhosis. Improvement of symptoms after treatment with prednisolone was associated with interleukin-6 rather than C-reactive protein. The pathogenesis of TAFRO syndrome, which has similar clinical manifestations to liver cirrhosis, remains unclear, and our findings may help elucidate the concept of this condition.

摘要

血小板减少症、全身性水肿、发热、网状纤维纤维化和器官肿大(TAFRO)综合征是一种以上述症状为特征的系统性炎症性疾病。由于 TAFRO 综合征和失代偿性肝硬化在表型上的相似性,准确的诊断往往很困难。本文报告了一例 62 岁的日本 TAFRO 综合征患者,该患者被误诊为肝硬化伴难治性腹水。泼尼松龙治疗后症状改善与白细胞介素-6而非 C 反应蛋白相关。TAFRO 综合征的发病机制与肝硬化具有相似的临床表现,但尚不清楚,我们的发现可能有助于阐明这种情况的概念。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ea9/9593160/54d79ded9dcc/1349-7235-61-2967-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ea9/9593160/52480cbd09b6/1349-7235-61-2967-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ea9/9593160/54d79ded9dcc/1349-7235-61-2967-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ea9/9593160/52480cbd09b6/1349-7235-61-2967-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ea9/9593160/54d79ded9dcc/1349-7235-61-2967-g002.jpg

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Am J Hematol. 2021 Oct 1;96(10):1241-1252. doi: 10.1002/ajh.26292. Epub 2021 Jul 28.
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