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Medical Science Monitor Basic Research


An Autopsy Case of TAFRO Syndrome with Type II Respiratory Failure

Rare disease

Mikio Wada, Akihiro Nagata, Atsushi Kawashima, Keizo Kagawa

Japan Fukuchiyama City Hospital Ooe-branch, Fukuchiyama, Kyoto, Japan

Am J Case Rep 2020; 21:e926721

DOI: 10.12659/AJCR.926721

Available online: 2020-09-04

Published: 2020-10-13


BACKGROUND: TAFRO syndrome (thrombocytopenia, anasarca, fever, myelofibrosis, renal dysfunction, and organomegaly) is a systemic inflammatory disorder. The histological features of TAFRO syndrome are not fully understood and few autopsy cases have been reported.
CASE REPORT: A 66-year-old man with type II respiratory failure was diagnosed with TAFRO syndrome. He was initially treated with tocilizumab. Although some improvements were observed, his condition worsened, and the medication was switched to rituximab. His condition remained steady for 1 year with intermittent artificial ventilation. However, he died due to exacerbation of respiratory failure about 20 months after diagnosis. An autopsy revealed mucous fluid retention in the spaces between the axis cylinder and the myelin sheath of peripheral nerves and among the peripheral nerves, suggesting that this retention contributed to neurodegeneration with demyelination. Skeletal muscles, including respiratory muscles, were highly atrophic, which could have led to type II respiratory failure.
CONCLUSIONS: Fluid accumulation other than pleural effusion and ascites could occur in intra-organs at a cellular level.

Keywords: Edema, Giant Lymph Node Hyperplasia, Nerve Degeneration, Respiratory Insufficiency