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Castleman Disease, TAFRO Syndrome and Related Disorders

Yasufumi Masaki, Yusuke Ueda, Hiroto Yanagisawa, Tomoyuki Sakai, Kazuyuki Yamada

Castleman B described patients with mediastinal tumors mimicking thymomas with characteristic histopathology, and this condition was named Castleman disease (CD). CD has been classified clinically as Unicentric CD (UCD) and multicentric CD (MCD). Histopathologically, CD has been classified as hyaline vascular type, plasma cell type, mixed type, hyper-vascular type, and plasmablastic type. MCD has been etiologically classified as human herpesvirus- 8-related (HHV-8-related); HHV-8-unrelated, aka idiopathic MCD (iMCD); polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome-related, and others. Furthermore, iMCD has also been classified as iMCD with thrombocytopenia, anasarca, fever, reticulin myelofibrosis or renal insufficiency and organomegaly (TAFRO syndrome), and iMCD not otherwise specified. Thus far, we have discussed diseases that have been named after the renowned pathologist Castleman. However, the use of eponyms and vague disease names has contributed to confusion in understanding disorders. Recently, some eponyms and indistinct disease names have been replaced by disease-specific nomenclature. As such, the question arises as to what nomenclature should be adopted for diseases such as HHV8+MCD, iMCD-NOS, POEMS and TAFRO. For instance, iMCD could be referred to as idiopathic hyper-IL-6 syndrome with polyclonal hypergammaglobulinemia and lymphadenopathy. We have proposed more specific nomenclature for CD related-disorders, and believe that the selection of the optimal therapeutic strategy for each condition depends on accurate diagnosis.