CT findings in 11 patients with TAFRO syndrome: a variant of multicentric Castleman's disease

Aim To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease. Materials and meth...

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Bibliographic Details
Published in:Clinical radiology 2017-10, Vol.72 (10), p.905.e1-905.e5
Main Authors: Kiguchi, T, Sato, C, Takai, K, Nakai, Y, Kaneko, Y, Matsuki, M
Format: Article
Language:English
Subjects:
Adult
Aged
Castleman Disease - diagnostic imaging
Castleman Disease - pathology
Edema - complications
Edema - diagnostic imaging
Edema - pathology
Female
Fever - complications
Fever - pathology
Fibrosis - complications
Fibrosis - diagnostic imaging
Fibrosis - pathology
Humans
Male
Middle Aged
Radiology
Reproducibility of Results
Reticulin
Retrospective Studies
Syndrome
Thrombocytopenia - complications
Thrombocytopenia - diagnostic imaging
Thrombocytopenia - pathology
Tomography, X-Ray Computed
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Summary:Aim To assess detailed computed tomography (CT) findings in patients with the recently described thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome, in order to contribute to imaging interpretation in the challenging diagnosis of this disease. Materials and methods The institutional review board approved this retrospective study and waived the need for informed consent. Eleven patients (six men, five women; mean age, 52.5 years) with confirmed TAFRO syndrome were included in this study. Chest-to-pelvis CT images were analysed for the presence of anasarca, organomegaly, bone lesions, and lung lesions. Results Anasarca was present in all patients and involved multiple cavities and tissues; pleural effusion and ascites were found in 100% of patients; pericardial effusion in 64%; periportal collar in 91%; gallbladder wall oedema in 78%; subcutaneous oedema in 91%; retroperitoneal oedema in 100%; and mesenteric oedema in 100%. Organomegaly involved multiple organs: hepatomegaly in 73%, splenomegaly in 82%, lymphadenopathy in 100%, and enlarged anterior mediastinum in 64% (solitary, well-circumscribed mass, 0%; infiltrative mass, 0%; non-mass-forming infiltrative lesion, 64%). Bone lesions were present in 91% patients and all bone lesions had ground-glass density with diffuse distribution. None of the patients had any lesions in their lungs. Conclusion The present study revealed that the findings of anasarca, organomegaly, and diffuse bony ground-glass appearance were observed in detail on CT in patients with TAFRO syndrome. A “matted” appearance of the enlarged anterior mediastinum is the characteristic CT finding of TAFRO syndrome, and it is possible to diagnose TAFRO syndrome from the combination of several CT findings.
ISSN:0009-9260
1365-229X
DOI:10.1016/j.crad.2017.05.007