Successful Treatment of TAFRO Syndrome with Tocilizumab, Prednisone, and Cyclophosphamide

Abstract

TAFRO syndrome is a rare systemic inflammatory disease characterized by thrombocytopenia, pleural effusion, fever, renal dysfunction, reticulin fibrosis of the bone marrow, and organomegaly. The clinical course varies significantly among patients. However, the prognosis is usually dismal in patients with severe TAFRO syndrome, and no optimal treatment has yet been established. We herein describe the first case of TAFRO syndrome, which was successfully treated with combination therapy consisting of tocilizumab, prednisone, and cyclophosphamide.

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

“…However, cases of TCZ-resistant TAFRO syndrome have been reported [15,18]. Clinical experiences of using rituximab, cyclophosphamide, and thalidomide have been reported from Japan [19][20][21] In the present case, as combination treatment with PSL and CsA appeared ineffective even after 5 weeks, and TCZ was considered an alternative immunosuppressant. However, the patient's condition became unstable before TCZ administration was initiated.…”

“…Although anti-SS-A antibody was positive and IgG4 was elevated in the present case, the patient did not meet the criteria for either SjS or IgG4-related disease. We found cases of a 72-year-old Japanese man with positive anti-SS-A antibody and a 50-year-old Japanese woman with positive anti-SS-A and anti-SS-B antibodies without indicative symptoms of SjS [20,33], a case of a 25-year-old Japanese woman with possible TAFRO syndrome having primary SjS [34], and a case of a 46-year-old Japanese woman who was diagnosed with SjS during hospitalization [35]. A previous report of a case of an 81-year-old French man with Fig.…”

“…Some severe cases might require temporary hemodialysis (HD). We reviewed previously reported cases of TAFRO syndrome requiring HD during the clinical course in the English literature and identified 19 cases (16 Japanese cases [17,19,20,28,33,[43][44][45][46][47][48][49][50][51]] and 3 non-Japanese cases [42,52,53]). Japanese patients were older than non-Japanese patients (mean age, 57.6 vs. 35.7 years).…”

TAFRO Syndrome with an Anterior Mediastinal Mass and Lethal Autoantibody-Mediated Thrombocytopenia: An Autopsy Case Report

“…These antibodies have been reported to cause immune-mediated platelet transfusion-refractoriness [19]. In fact, several cases of TAFRO syndrome have been reported to present with refractoriness to platelet transfusions [5, 15, 16, 20, 21], suggesting that thrombocytopenia might occur as a result of antibody-mediated platelet destruction. A detailed analysis of the mechanism of antibody production against platelets could provide a key to understanding the pathogenesis of TAFRO syndrome.…”

TAFRO Syndrome Presenting with Retroperitoneal Panniculitis-like Computed Tomography Findings at Disease Onset

“…There is no standard therapy for TAFRO syndrome, but in Japan, glucocorticoid therapy is usually administered (4). In cases refractory to glucocorticoid therapy, other immunosuppressants, such as cyclosporin, rituximab, cyclophosphamide (CPA), and tocilizumab, have been prescribed (4)(5)(6)(7).…”