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

Abstract

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.

Keywords

• idiopathic multicentric castleman disease
• tafro syndrome
• tocilizumab