Successful treatment by tocilizumab without steroid in a very severe case of TAFRO syndrome

Abstract

A 59-year-old Japanese man was admitted to our hospital with leg edema, fatigue and fever. He had a past medical history of duodenal ulcer, fatty liver, hyperuricemia and positive rheumatoid factor, but was not receiving any medication. His son had died 2 years prior to this admission after following a clinical course that suggested TAFRO syndrome.The patient showed mild fever (about 37-38 °C), polypnea and severe leg edema on admission. Chest examination revealed decreased breath sounds in the right lung. No superficial lymph nodes were palpable. Laboratory tests on admission revealed mild thrombocytopenia, hypoproteinemia, elevation of C-reactive protein (CRP), alkaline phosphatase (ALP), creatinine (Cr), and urine N-acetyl-β-d-glucosaminidase (NAG) ( Table 1). Urine testing showed granular casts without proteinuria or hematuria. Concentrations of aspartate aminotransferase (AST) and alanine transaminase (ALT) were elevated, but unchanged compared with the results of a medical check 6 months before admission, and so were attributed to fatty liver. Chest X-ray and computed tomography (CT) showed mild lymphadenopathy in the mediastinum, bilateral pleural effusion and ascites (Fig. 1).Blood, sputum and urine culture tests were all negative and no other infectious diseases were present. Negative results were obtained for all autoantibodies. Among the tumor markers, only soluble interleukin-2 receptor (sIL-2R) was elevated. Bone marrow biopsy was performed to rule out malignant lymphoma, revealing normocellular Abstract Successful use of tocilizumab (TCZ) to treat TAFRO syndrome has recently been reported. In those cases, TCZ was used with steroid. We present herein the case of a 59-year-old man with very severe TAFRO syndrome who was successfully treated using TCZ without steroid. He showed rapidly progressive anasarca, acute renal failure and very severe thrombocytopenia. We initially used steroid, but its efficacy was limited. Moreover, steroid use had to be stopped as soon as possible, because hemorrhagic shock developed due to severe duodenal ulcer. After overcoming infections (about 40 days after stopping steroid), administration of TCZ was started and the patient was discharged in clinical remission.

Plasma proteomics identifies a ‘chemokine storm’ in idiopathic multicentric Castleman disease

“…Both iMCD‐TAFRO patients were anti‐IL‐6 nonresponders and all three iMCD‐NOS patients treated with anti‐IL‐6‐therapy responded. Importantly, there have been reports of iMCD‐TAFRO cases that have responded to anti‐IL‐6‐therapy and others that have not . The proteomic differences between the two groups may represent differences between clinical subtypes or be a signature of anti‐IL‐6 response.…”

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.…”

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

“…Tocilizumab (TCZ) could be used safely in patients with renal impairment, and it might be a reasonable choice considering that excessive IL-6 secretion is one of the main pathophysiological events in TAFRO syndrome. Fujiki et al reported a case of TAFRO syndrome complicated with severe infection during corticosteroid therapy, which was successfully treated with TCZ alone [17]. However, cases of TCZ-resistant TAFRO syndrome have been reported [15,18].…”

“…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).…”