Tocilizumab for the treatment of TAFRO syndrome: a systematic literature review
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REVIEW ARTICLE
Tocilizumab for the treatment of TAFRO syndrome: a systematic literature review Mitsuhiro Akiyama 1
&
Yuko Kaneko 1 & Tsutomu Takeuchi 1
Received: 17 August 2020 / Accepted: 17 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract TAFRO syndrome is a newly recognized disease entity characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. The objective of this study was to investigate the effectiveness of tocilizumab, an anti-interleukin-6 receptor antibody, in patients with TAFRO syndrome. We performed a systematic literature review from inception to July 5, 2020, for articles reporting tocilizumab administration for the treatment of TAFRO syndrome. We identified 31 patients with TAFRO syndrome treated with tocilizumab. The mean age was 49.8 years, and 61.3% of the patients were male. The mean observation period was 12.6 months. Tocilizumab was used at the standard intravenous dose (8 mg/kg) weekly or every 2 weeks in combination with other immunosuppressive drugs, such as glucocorticoids, rituximab, cyclosporine, or cyclophosphamide, in most of the patients. Eighteen patients (58.1%) received tocilizumab as a first-line treatment, while it was a second-line or a third-line treatment for 13 patients with insufficient responses to the prior treatments. Sixteen patients (51.6%) obtained complete response to tocilizumab treatment, whereas 15 patients showed only partial or no response. Detailed factors of ineffectiveness included persistent thrombocytopenia (n = 7), persistent anasarca (n = 5), persistent renal dysfunction (n = 2), and persistent fever (n = 2). A total of four patients (12.9%) succumbed to the disease, while the remaining twenty-seven patients survived. Two patients achieved drug-free remission at last visit, and disease remission was maintained with tocilizumab monotherapy in five patients. No new safety signal was reported. Tocilizumab was effective in ~ 50% of the patients, suggesting it could serve as a treatment choice for TAFRO syndrome. Poor clinical response to tocilizumab observed in other patients highlights the need for the additional therapeutic treatment options. Keywords TAFRO syndrome . Castleman disease . Immune thrombocytopenia . Tocilizumab . Anti-interleukin-6 receptor . Interleukin-6
Introduction Castleman disease is a rare lymphoproliferative disorder, historically classified either as a unicentric or a multicentric type. Multicentric Castleman disease (MCD) is further divided into human herpesvirus 8–positive MCD or human herpesvirus 8– negative idiopathic MCD (iMCD) [1]. iMCD has three distinct subtypes: POEMS (polyneuropathy, organomegaly,
* Yuko Kaneko [email protected] 1
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan
endocrinopathy, monoclonal plasma cell proliferation, and skin change) syndrome, TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunc
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