Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?

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Abstract
Castleman disease (CD) is a rare lymphoproliferative disorder that can be unicentric or multicentric. Multicentric CD (MCD) is further subdivided into human herpesvirus type‐8‐associated, POEMS syndrome‐associated, and idiopathic (iMCD). TAFRO syndrome is a newly identified disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction, and organomegaly. The TAFRO syndrome is sometimes regarded as a subtype of iMCD (TAFRO‐iMCD), whereas iMCD without TAFRO syndrome is considered “not otherwise specified” (iMCD‐NOS). However, a proportion of patients with TAFRO syndrome have been diagnosed without lymph node biopsies (TAFRO syndrome without proven iMCD; TAFRO‐w/op‐iMCD). To clarify the clinical features of iMCD‐NOS, TAFRO‐iMCD, and TAFRO‐w/op‐iMCD, we retrospectively analyzed 220 patients extracted from the database of the Multicenter Collaborative Retrospective Study for Establishing the Concept of TAFRO Syndrome. The patients included 87 with iMCD‐NOS, 63 with TAFRO‐iMCD, and 19 with TAFRO‐w/op‐iMCD. Patients in all three groups exhibited anemia, hypoalbuminemia, and elevated serum C‐reactive protein and interleukin‐6 levels. No significant differences in clinical, laboratory, and prognostic features were noted between the TAFRO‐iMCD, and TAFRO‐w/op‐iMCD groups. However, the iMCD‐NOS group exhibited polyclonal hyper‐γ‐globulinemia. The five‐year survival rates of patients in the iMCD‐NOS and TAFRO‐involved groups were 100% and 66.5%, respectively (dropping markedly during the first few months in the latter). The iMCD‐NOS and the TAFRO‐iMCD samples typically showed plasma cell and mixed‐type histologies, respectively. Thus, iMCD can be classified into two distinct subtypes, iMCD‐NOS and TAFRO‐iMCD. As such, TAFRO‐iMCD and TAFRO‐w/op‐iMCD may be considered the same entity, requiring prompt diagnosis and intensive care.
Funding Information
  • Kanazawa Medical University (S2004‐16, S2007‐5, K2011‐7, H2011‐11, AR2012‐06)
  • Ministry of Education, Culture, Sports, Science and Technology (17591060, 15K09510)
  • Ministry of Health, Labour and Welfare (H27‐28 Nanchi, etc. (Nan)‐ General‐002; H27‐Nanchi)

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