Characteristics of thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly syndrome: a retrospective study from a large Western cohort.
Adult
Biopsy
Castleman Disease
/ diagnosis
Clinical Decision-Making
Combined Modality Therapy
Diagnosis, Differential
Disease Management
Disease Susceptibility
Female
Humans
Immunohistochemistry
Male
Middle Aged
Phenotype
Positron Emission Tomography Computed Tomography
Prognosis
Retrospective Studies
Syndrome
Treatment Outcome
Young Adult
Western thrombocytopenia
anasarca
fever
idiopathic multicentric Castleman disease
reticulin fibrosis and organomegaly syndrome
rituximab
tocilizumab
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
02 2022
02 2022
Historique:
revised:
14
09
2021
received:
16
07
2021
accepted:
17
09
2021
pubmed:
30
9
2021
medline:
15
2
2022
entrez:
29
9
2021
Statut:
ppublish
Résumé
Idiopathic multicentric Castleman disease (iMCD) is a non-clonal inflammatory lymphoproliferative disorder of unknown origin. Recently, TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) emerged as a singular variant of iMCD in Asia and was associated with a severe course and a poor outcome. The present study describes the first large Western cohort of TAFRO syndrome patients (n = 25) meeting the All Japan TAFRO Syndrome Research Group diagnostic criteria. Characteristics of TAFRO patients were compared to iMCD-not otherwise specified (iMCD-NOS) patients used as a control group (n = 43). Our results show that despite baseline characteristics in accordance with previously reported series, Western TAFRO syndrome patients do not appear to present with a worse outcome than iMCD-NOS patients. There were no significant differences between the two groups regarding treatment choice, response to rituximab (71% vs. 67%) or tocilizumab (69% vs. 91%) in TAFRO and iMCD-NOS, respectively. The two-year overall survival was above 95% in both groups. Limits of inclusion and exclusion criteria for TAFRO definition are also discussed. Our findings raise the question of the singularity of the TAFRO entity in Western countries. The data should promote further research using unsupervised models to identify markers of disease severity in Western cohorts of iMCD patients.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
599-605Informations de copyright
© 2021 British Society for Haematology and John Wiley & Sons Ltd.
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