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Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease
Author(s) -
Nishimura Yoshito,
Fajgenbaum David C.,
Pierson Sheila K.,
Iwaki Noriko,
Nishikori Asami,
Kawano Mitsuhiro,
Nakamura Naoya,
Izutsu Koji,
Takeuchi Kengo,
Nishimura Midori Filiz,
Maeda Yoshinobu,
Otsuka Fumio,
Yoshizaki Kazuyuki,
Oksenhendler Eric,
Rhee Frits,
Sato Yasuharu
Publication year - 2021
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.26292
Subject(s) - anasarca , organomegaly , medicine , castleman disease , lymph node , pathology , nephrogenic systemic fibrosis , kidney disease , disease
Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly (TAFRO) syndrome is a heterogeneous entity manifesting with a constellation of symptoms described above that can occur in the context of idiopathic multicentric Castleman disease (iMCD) as well as infectious diseases, malignancies, and rheumatologic disorders. So, iMCD‐TAFRO is an aggressive subtype of iMCD with TAFRO syndrome and often hyper‐vascularized lymph nodes. Since we proposed diagnostic criteria of iMCD‐TAFRO in 2016, we have accumulated new insights on the disorder and additional cases have been reported worldwide. In this systematic review and cohort analysis, we established and validated a definition for iMCD‐TAFRO. First, we searched PubMed and Japan Medical Abstracts Society databases using the keyword “TAFRO” to extract cases. Patients with possible systemic autoimmune diseases and hematologic malignancies were excluded. Our search identified 54 cases from 50 articles. We classified cases into three categories: (1) iMCD‐TAFRO (TAFRO syndrome with lymph node histopathology consistent with iMCD), (2) possible iMCD‐TAFRO (TAFRO syndrome with no lymph node biopsy performed and no other co‐morbidities), and (3) TAFRO without iMCD or other co‐morbidities (TAFRO syndrome with lymph node histopathology not consistent with iMCD or other comorbidities). Based on the findings, we propose an international definition requiring four clinical criteria (thrombocytopenia, anasarca, fever/hyperinflammatory status, organomegaly), renal dysfunction or characteristic bone marrow findings, and lymph node features consistent with iMCD. The definition was validated with an external cohort (the ACCELERATE Natural History Registry). The present international definition will facilitate a more precise and comprehensive approach to the diagnosis of iMCD‐TAFRO.