Tocilizumab-resistant TAFRO Syndrome Complicated by Type II Respiratory Failure

Abstract

A 66-year-old man with a several year history of thrombocytopenia, pleural effusion and ascites, anasarca, and organomegaly presented with general fatigue, appetite loss, dyspnea with type II respiratory failure. The precise history of the patient and the re-evaluation of lymph node and bone marrow biopsies conducted by the previous physician indicated TAFRO syndrome. The patient's laboratory data improved for a year with tocilizumab, but then worsened to the point that the patient required artificial ventilation due to the deterioration of type II respiratory failure. The replacement of tocilizumab with rituximab yielded a steady improvement, but it was necessary to address the patient's persistent respiratory failure. Peripheral nerve disorder might have been involved with the patient's respiratory failure.

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

“…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]. 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.…”

“…Abbreviations ALP: Alkali phosphatase; CAG: Coronary angiography; CD: Castleman disease; CMV: Cytomegalovirus; CRP: C-reactive protein; CRRT: Continuous renal replacement therapy; CsA: Cyclosporine A; CT: Computed tomography; FDG-PET: 18…”

“…Echocardiography performed on day 2 revealed normal wall motions without any sign of valvular disease, but a collapsed inferior vena cava was observed (maximum diameter < 5 mm). 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET) revealed no apparent FDG uptake, except for slight uptake in the para-aortic lymph nodes ( Fig. 1d).…”

TAFRO syndrome with skin manifestations treated with bortezomib and tocilizumab; a case report

“…There are other cases demonstrating this overlap. Presence of neuropathy is also seen in TAFRO patients while this presentation is mostly seen with POEMS (5). Regardless of different and overlapping presentations, managing TAFRO is another challenge.…”

“…Addition of cyclophosphamide may reduce the therapeutic dose of steroids and reduce the adverse effects of steroid (6). Aoki et al reported a case of TAFRO syndrome which did not respond to tocilizumab (5). They suggested that rituximab is a good choice in patients who do not respond to tocilizumab therapy (5).…”