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

• Published in: Internal Medicine Vol. 56; no. 10; pp. 1247 - 1252
• Main Authors: Nara, Mizuho, Komatsuda, Atsushi, Itoh, Fumiko, Kaga, Hajime, Saitoh, Masaya, Togashi, Masaru, Kameoka, Yoshihiro, Wakui, Hideki, Takahashi, Naoto
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2017; Japan Science and Technology Agency
• Subjects: Adrenal Gland Diseases - complications; adrenal hemorrhage; Adult; Antibodies, Monoclonal, Humanized - therapeutic use; Asian Continental Ancestry Group; Calcitonin - blood; Case Report; Castleman disease; Castleman's disease; Edema - diagnosis; Edema - drug therapy; Etiology; Female; Fever; Fever - diagnosis; Fever - drug therapy; Fibrosis; Fibrosis - diagnosis; Fibrosis - drug therapy; Hemorrhage - drug therapy; Hemorrhage - etiology; Humans; Inflammatory diseases; Internal medicine; Kidneys; Male; Middle Aged; Monoclonal antibodies; POEMS Syndrome - diagnosis; POEMS Syndrome - drug therapy; Procalcitonin; Renal failure; Renal Insufficiency - diagnosis; Renal Insufficiency - drug therapy; Reticulin; Rituximab; TAFRO syndrome; Thrombocytopenia; Thrombocytopenia - diagnosis; Thrombocytopenia - drug therapy; Thrombocytopenia - therapy; Treatment Outcome; TAFRO syndrome; adrenal hemorrhage; procalcitonin; Castleman disease; rituximab
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.56.7991

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.