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A national, multicenter, retrospective study of Castleman disease in China implementing CDCN criteria
Lu Zhang1,2, Yu-Jun Dong3, Hong-Ling Peng4
1Department of Haematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Insights
Castleman disease (CD) subtypes were characterized in China using the CDCN criteria. Severe idiopathic multicentric CD (iMCD) was associated with worse outcomes, necessitating more intensive treatment strategies.
Area of Science:
- Hematology
- Oncology
- Rare Diseases
Background:
- Castleman disease (CD) is a rare lymphoproliferative disorder with diverse subtypes, including unicentric CD (UCD), human herpesvirus-8-associated multicentric CD (HHV8-MCD), and HHV-8-negative/idiopathic multicentric CD (iMCD).
- Previous studies on CD have limitations due to varying inclusion criteria and the recent availability of diagnostic criteria for iMCD and UCD from the Castleman Disease Collaborative Network (CDCN).
Purpose of the Study:
- To characterize the clinical features, treatment modalities, and prognostic factors of Castleman disease in China.
- To evaluate the utility of CDCN diagnostic criteria in a large cohort and assess outcomes, particularly for severe idiopathic multicentric CD (iMCD).
Main Methods:
- A national, multicenter, retrospective study was conducted involving 1634 CD patients diagnosed between 2000 and 2021 across 40 Chinese institutions.
- Patients were classified according to CDCN criteria, including UCD, HHV8-MCD, and HHV-8-negative MCD (further divided into asymptomatic MCD and iMCD).
- Clinical data, treatment approaches, and survival outcomes were analyzed to identify prognostic factors and validate diagnostic criteria.
Main Results:
- The study identified 903 UCD and 731 MCD patients. Among UCD, 17.9% exhibited an MCD-like inflammatory state.
- In the MCD cohort, 12 were HHV8-MCD and 719 were HHV-8-negative MCD, including 139 asymptomatic MCD and 580 iMCD.
- Severe iMCD, as defined by CDCN criteria, was significantly associated with worse outcomes (HR=3.747, p<0.001), indicating a need for more intensive treatment.
Conclusions:
- This study provides a comprehensive overview of CD in China, detailing patient characteristics, treatment trends, and survival data.
- The findings validate the CDCN's definition of severe iMCD and its association with poorer prognosis, highlighting the importance of timely and aggressive management.
- A shift towards continuous treatment over pulse combination chemotherapy was observed for iMCD patients receiving first-line therapy.
Background:
Castleman disease (CD) is a group of rare and heterogenous lymphoproliferative disorders including unicentric CD (UCD), human herpesvirus-8(HHV-8)-associated multicentric CD (HHV8-MCD), and HHV-8-negative/idiopathic multicentric CD (iMCD). Knowledge of CD mainly comes from case series or retrospective studies, but the inclusion criteria of these studies vary because the Castleman Disease Collaborative Network (CDCN) diagnostic criteria for iMCD and UCD were not available until 2017 and 2020, respectively. Further, these criteria and guidelines have not been systematically evaluated.
Methods:
In this national, multicenter, retrospective study implementing CDCN criteria, we enrolled 1634 CD patients (UCD, n = 903; MCD, n = 731) from 2000 to 2021 at 40 Chinese institutions to depict clinical features, treatment options, and prognostic factors of CD.
Findings:
Among UCD, there were 162 (17.9%) patients with an MCD-like inflammatory state. Among MCD, there were 12 HHV8-MCD patients and 719 HHV-8-negative MCD patients, which included 139 asymptomatic MCD (aMCD) and 580 iMCD meeting clinical criteria. Of 580 iMCD patients, 41 (7.1%) met iMCD-TAFRO criteria, the others were iMCD-NOS. iMCD-NOS were further divided into iMCD-IPL (n = 97) and iMCD-NOS without IPL (n = 442). Among iMCD patients with first-line treatment data, a trend from pulse combination chemotherapy toward continuous treatment was observed. Survival analysis revealed significant differences between subtypes and severe iMCD (HR = 3.747; 95% CI: 2.112-6.649, p < 0.001) had worse outcome.
Interpretation:
This study depicts a broad picture of CD, treatment options and survival information in China and validates the association between the CDCN's definition of severe iMCD and worse outcomes, requiring more intensive treatment.
Fundings:
Beijing Municipal Commission of Science and Technology, CAMS Innovation Fund and National High Level Hospital Clinical Research Funding.
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