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Retrospective cohort evaluation of non-HIV Castleman disease from a single academic center in Beijing, China

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Abstract

The purpose is to ascertain the clinical impact of Castleman disease (CD) by reassessment of the real-world data from Peking University First Hospital (PKUFH). The results will contribute to the standardization of diagnosis and treatment on CDs. Based on the last 15-year retrospective real-world data from Peking University First Hospital (PKUFH), we reclassified and re-evaluated the clinical and pathological information of patients with pathologically suspected diagnosis of CD. A total of 203 patients were included in our study, in which the diagnosis of CD was confirmed in 189 cases, including 118 patients with unicentric CD (UCD, n = 118, 62.4%) and 71 patients with multicentric CD (MCD, n = 71, 37.6%). A total of 44.1% (n = 52) of UCDs in our cohort were complicated with paraneoplastic pemphigus (PNP). The treatment of UCD is primarily surgical, with a 5-year overall survival (OS) of 88.1%. Patients with PNP had a poorer prognosis than those without PNP (82.9% (95% CI 123–178) vs 92.8% (95% CI 168–196), log-rank p = 0.041). The rate of concurrent systemic symptoms was 74.6% (n = 53), and renal involvement occurred in 49.3% (n = 35) MCD patients. The MCD treatments were mainly chemotherapy regimens, with a 5-year OS of 77.6% (95% CI, 143–213). Patients with UCD demonstrate a better overall prognosis than patients with MCD. But the prognosis of those complicated with PNP was poor. The differential diagnosis of MCD is extensive. MCD treatment in China is heterogeneous. The inaccessibility of anti-IL-6–targeted drugs in China may contribute to the poor prognosis for patients with MCD.

A preprint has previously been published (Guo et al. 34).

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The data underlying this article will be shared on reasonable request to the corresponding authors.

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Acknowledgements

We thank all the authors and participants for their contributions to the trial. We thank Dr. Nong L and her team for reviewing the pathological slides.

Funding

This study was supported by Beijing Natural Science Foundation No. 7232175.

Author information

Author notes

  1. Meiyu Guo and Lin Nong equally contributed to this paper.

Authors and Affiliations

  1. Department of Hematology, Peking University First Hospital, No. 7 Xishiku St. Xicheng District, Beijing, 100034, China

    Meiyu Guo, Yang Zhang, Lihong Wang, Yuhua Sun, Qingyun Wang, Huihui Liu, Jinping Ou, Xinan Cen, Hanyun Ren & Yujun Dong

  2. Department of Pathology, Peking University First Hospital, Beijing, China

    Lin Nong

  3. Department of Dermatology, Peking University First Hospital, Beijing, China

    Mingyue Wang

Authors
  1. Meiyu Guo
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Contributions

GMY collected, analyzed, and interpreted data and co-wrote the manuscript. NL and her team performed the pathological analysis and co-wrote the manuscript. WMY, ZY, WLH, SYH, WQY, LHH, OJP, CXN, and RHY participated in the collection and interpretation of data. DYJ designed and co-wrote the manuscript and coordinated and supervised the project. All authors approved the final version of the manuscript.

Corresponding author

Correspondence to Yujun Dong.

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The authors declare no competing interests.

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Key points

• A large cohort of CD from a single center were reclassified and re-evaluated according to the 2017 CDCN diagnostic criteria.

• In the real-world setting, UCD patients complicated with PNP and MCD have poor prognosis even after intensive therapeutic treatments.

Supplementary information

ESM 1

Supplement Tab.1 Treatment and deaths of UCD patients, Supplement Tab.2 Treatment and deaths of MCD patients, Supplement Tab.3 Different chemotherapy regimens and cycles of MCD patients, Supplement Fig.1 Proportion of regions affected by UCD, Supplement Fig.2 Representative pathological images of different subtype of UCD and MCD. (DOCX 358 kb)

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Guo, M., Nong, L., Wang, M. et al. Retrospective cohort evaluation of non-HIV Castleman disease from a single academic center in Beijing, China. Ann Hematol 103, 153–162 (2024). https://doi.org/10.1007/s00277-023-05472-3

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  • DOI: https://doi.org/10.1007/s00277-023-05472-3

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