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Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging

2017; The Japanese Society of Internal Medicine; Volume: 56; Issue: 10 Linguagem: Inglês

10.2169/internalmedicine.56.7991

1349-7235

Mizuho Nara, Atsushi Komatsuda, Fumiko Itoh, Hajime Kaga, Masaya Saitoh, Masaru Togashi, Yoshihiro Kameoka, Hideki Wakui, Naoto Takahashi,

Kawasaki Disease and Coronary Complications

Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.