TAFRO Syndrome Mimicking Solid Cancer and Successfully Treated by Tocilizumab: A Case and Literature Review

TAFRO syndrome, or now formally termed idiopathic multicentric Castleman disease (iMCD) with thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly (iMCD-TAFRO), describes a unique subtype of iMCD. Hypersecretion of pro-inflammatory cytokines, such as interleu...

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Bibliographic Details
Main Authors: Pei-An Fu, Chia-Chi Wu, Ya-Ting Hsu
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2023-10-01
Series:Journal of Cancer Research and Practice
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Online Access:https://journals.lww.com/10.4103/ejcrp.eJCRP-D-23-00021
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Summary:TAFRO syndrome, or now formally termed idiopathic multicentric Castleman disease (iMCD) with thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly (iMCD-TAFRO), describes a unique subtype of iMCD. Hypersecretion of pro-inflammatory cytokines, such as interleukin-6, plays a critical role in this disease. Several anti-inflammatory medications are used for treatment, such as tocilizumab, siltuximab, and rituximab, and they have demonstrated efficacy in some cases; however, the disease remains incurable. Here, we report a 56-year-old woman who presented with ileus and progressive ascites formation. She received several operations for suspected secondary peritonitis, but thrombocytopenia, lymphadenopathy, and anasarca progressed. It took 1 year to reach the diagnosis of iMCD-TAFRO, and tocilizumab was given soon after the diagnosis. After tocilizumab treatment, her symptoms improved dramatically. Due to the heterogeneous clinical manifestations of iMCD-TAFRO, awareness of iMCD-TAFRO and a multidisciplinary team approach are required for a timely and accurate diagnosis of iMCD-TAFRO.
ISSN:2311-3006