BACKGROUND: Although thrombocytopenia, anasarca, fever, reticulin fibrosis and 
organomegaly (TAFRO) syndrome was first described as a variant of idiopathic 
multicentric Castleman disease (CD), patients with TAFRO syndrome demonstrate 
more aggressive clinical features. Because these patients may present with fever 
of unknown origin, general physicians need to recognise its characteristic 
laboratory data and clinical features during hospitalisation.
AIMS: to describe the features, symptoms and characteristics of TAFRO syndrome 
and to compare them to those of idiopathic CD.
METHODS: This was a retrospective study of patients with histopathologically 
confirmed TAFRO syndrome and idiopathic multicentric CD who were diagnosed and 
managed between April 2012 and June 2018 in a Japanese university hospital's 
General Medicine Department.
RESULTS: We found that the hospitalisations were significantly longer among 
patients with TAFRO syndrome compared to those with idiopathic CD (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 as an infectious disease with an 
aggressive clinical course. Our study highlights the importance of giving 
significance to chief complaints and laboratory data. Physicians need to 
recognise the clinical and laboratory features of this disease to avoid missing 
this potentially fatal disorder.