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

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

doi:10.2169/internalmedicine.56.7991

CASE REPORTS

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

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

Author information

Mizuho Nara
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Atsushi Komatsuda
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Fumiko Itoh
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Hajime Kaga
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Masaya Saitoh
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Masaru Togashi
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Yoshihiro Kameoka
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Hideki Wakui
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
Department of Life Science, Akita University Graduate School of Engineering Science, Japan
Naoto Takahashi
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan

Keywords: adrenal hemorrhage, Castleman disease, procalcitonin, rituximab, TAFRO syndrome

JOURNAL OPEN ACCESS

2017 Volume 56 Issue 10 Pages 1247-1252

DOI https://doi.org/10.2169/internalmedicine.56.7991

Details

Abstract

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.

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