Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?
Journal
American journal of hematology
ISSN: 1096-8652
Titre abrégé: Am J Hematol
Pays: United States
ID NLM: 7610369
Informations de publication
Date de publication:
09 2019
09 2019
Historique:
received:
07
02
2019
revised:
05
06
2019
accepted:
06
06
2019
pubmed:
22
6
2019
medline:
11
3
2020
entrez:
22
6
2019
Statut:
ppublish
Résumé
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.
Types de publication
Clinical Trial
Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
975-983Subventions
Organisme : Kanazawa Medical University
ID : S2004-16
Pays : International
Organisme : Kanazawa Medical University
ID : S2007-5
Pays : International
Organisme : Kanazawa Medical University
ID : K2011-7
Pays : International
Organisme : Kanazawa Medical University
ID : H2011-11
Pays : International
Organisme : Kanazawa Medical University
ID : AR2012-06
Pays : International
Organisme : Ministry of Education, Culture, Sports, Science and Technology
ID : 17591060
Pays : International
Organisme : Ministry of Education, Culture, Sports, Science and Technology
ID : 15K09510
Pays : International
Organisme : Ministry of Health, Labour and Welfare
ID : H27-28 Nanchi, etc. (Nan)- General-002; H27-Nanchi
Pays : International
Informations de copyright
© 2019 Wiley Periodicals, Inc.
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