Comparison of the clinical characteristics of TAFRO syndrome and idiopathic multicentric Castleman disease in general internal medicine: a 6-year retrospective study.

Published on Feb 1, 2020in Internal Medicine Journal1.677
· DOI :10.1111/IMJ.14404
Yoshito Nishimura5
Estimated H-index: 5
(Okayama University),
Yoshihisa Hanayama6
Estimated H-index: 6
(Okayama University)
+ 2 AuthorsFumio Otsuka3
Estimated H-index: 3
(Okayama University)
Sources
Abstract
BACKGROUND: Although thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome was first described as a variant of idiopathic multicentric Castleman disease, patients with TAFRO syndrome have more aggressive clinical features. Because these patients may present with fever of unknown origin, general physicians need to recognize its characteristic laboratory data and clinical features during hospitalization. AIMS: Herein, we described the features, symptoms, and characteristics of TAFRO syndrome and compared them to those of idiopathic Castleman disease. METHODS: This was a retrospective study of patients with histopathologically confirmed TAFRO syndrome and idiopathic multicentric Castleman disease who were diagnosed and managed between April 2012 and June 2018 in a Japanese university hospital General Medicine Department. RESULTS: We found that the hospitalizations were significantly longer among patients with TAFRO syndrome compared to those with idiopathic Castleman disease (median: 87 days; range: 34-236 days vs. median: 30 days; range: 13-59 days; p < 0.01). Patients with TAFRO syndrome were more likely to present with fever, abdominal pain, and elevated inflammatory markers and be misdiagnosed with an infectious disease during the first hospital visit. Approximately 40% of patients with TAFRO syndrome had no radiographically enlarged lymph nodes. CONCLUSIONS: TAFRO syndrome may present like an infectious disease with an aggressive clinical course. Our study highlights the importance of placing significance on chief complaints and laboratory data. Physicians need to recognize the clinical and laboratory features of this disease to avoid missing this potentially fatal disorder. This article is protected by copyright. All rights reserved.
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