1. Asymptomatic multicentric Castleman disease: a potential early stage of idiopathic MCD.
- Author
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Zhang L, Liu QH, Zhou H, Zhang HL, Dong YJ, Wang XB, Wang LQ, Su LP, Yan XJ, Li Y, Zhang MZ, Ding KY, Wang HH, Peng HL, Zhong LY, Yang L, Bi LT, Gao D, Gao GX, Huang L, Sun CY, Song J, Qian WB, Huang WR, Li ZL, Liu Y, and Li J
- Subjects
- Humans, Female, Male, Middle Aged, Adult, Retrospective Studies, Adolescent, Young Adult, Aged, Disease Progression, Herpesvirus 8, Human isolation & purification, Asymptomatic Diseases, Prognosis, Castleman Disease diagnosis, Castleman Disease mortality, Castleman Disease pathology
- Abstract
Abstract: According to the diagnostic criteria for human herpesvirus 8 (HHV-8)-negative/idiopathic multicentric Castleman disease (iMCD) proposed by Castleman Disease Collaborative Network in 2017, there is a group of HHV-8-negative patients with multicentric Castleman disease (MCD) who do not have symptoms and hyperinflammatory state and do not meet the iMCD criteria. This retrospective study enrolled 114 such patients, described as asymptomatic MCD (aMCD), from 26 Chinese centers from 2000 to 2021. With a median follow-up time of 46.5 months (range, 4-279 months), 6 patients (5.3%) transformed to iMCD. The median time between a diagnosis of aMCD and iMCD in these 6 patients was 28.5 months (range, 3-60). During follow-up, 7 patients died; 3 of them died from progression of MCD. Despite that, 37.7% of patients received systemic treatment targeting MCD; this strategy was neither associated with a lower probability of iMCD transformation nor a lower death rate. The 5-year estimated survival of all patients with aMCD was 94.1% (95% confidence interval, 88.8-99.6). Transformation to iMCD was an important predictor of death (log-rank P = .01; 5-year estimated survival, 83.3%). This study suggests that patients with aMCD may represent a potential early stage of iMCD, who may not require immediate treatment but should be closely monitored., (© 2024 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)
- Published
- 2024
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