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Organ dysfunction, thrombotic events and malignancies in patients with idiopathic multicentric castleman disease: a population-level US health claims analysis

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Fig. 1: Development of morbidities over time in iMCD patients before and after diagnosis relative to non-disease controls.

Data availability

The datasets analyzed during the current study were made available to the authors through licensing from the data provider. The data cannot be made publicly available due to the data use agreement.


  1. Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020;135:1353–64.

    Article  PubMed  Google Scholar 

  2. Yu L, Tu M, Cortes J, Xu-Monette Z, Miranda R, Zhang J, et al. Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease. 2021. (accessed December 9, 2021).

  3. Pierson SK, Stonestrom AJ, Shilling D, Ruth J, Nabel CS, Singh A, et al. Plasma proteomics identifies a “chemokine storm” in idiopathic multicentric Castleman disease. Am J Hematol. 2018;93:902–12.

    Article  CAS  PubMed  Google Scholar 

  4. Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Haematol. 2016;3:e163–175.

    Article  PubMed  Google Scholar 

  5. Dispenzieri A, Armitage JO, Loe MJ, Geyer SM, Allred J, Camoriano JK, et al. The clinical spectrum of Castleman’s disease. Am J Hematol. 2012;87:997–1002.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Kulaylat AS, Schaefer EW, Messaris E, Hollenbeak CS. Truven Health Analytics MarketScan Databases for Clinical Research in Colon and Rectal Surgery. Clin Colon Rectal Surg. 2019;32:54–60.

    Article  PubMed  PubMed Central  Google Scholar 

  7. Mukherjee S, Martin R, Sande B, Paige J, Fajgenbaum DC. Epidemiology and treatment patterns of idiopathic multicentric Castleman disease in the era of IL-6 directed therapy. Blood Adv. 2021:bloodadvances.2021004441.

  8. Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129:1646–57.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Simpson D. Epidemiology of Castleman disease. Hematol Clin. 2018;32:1–10.

    Google Scholar 

  10. Pierson SK, Khor JS, Ziglar J, Liu A, Floess K, NaPier E, et al. ACCELERATE: a Patient-Powered Natural History Study Design Enabling Clinical and Therapeutic Discoveries in a Rare Disorder. Cell Rep Med. 2020;1:100158.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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The authors would like to thank Bob Lou and Ryan Rumantir, employees of EVERSANA, for their help in collecting and analyzing the data presented in this paper. DF receives funding support from the National Institutes of Health, National Heart, Lung, and Blood Institute (R01HL141408). SM received funding support from Velosano 2020 Impact Award for Rare Diseases (Cleveland Clinic Taussig Cancer Institute).

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Authors and Affiliations



DK, AG, and HK were responsible for the data extraction and performed the data analysis. SM, DCF, KK, RM and FS jointly conceived, analyzed and interpreted the data, critically reviewed and edited the paper; DK and JSP collected and assembled the data, analyzed and interpreted the data and drafted the paper.

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Correspondence to Sudipto Mukherjee.

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Competing interests

The author(s) received no financial support for the research, authorship, and/or publication of this manuscript. DCF has received research funding from EUSA Pharma, received consulting fees from EUSA Pharma, and has a provisional patent pending related to JAK1/2 inhibition in iMCD and predictive biomarkers in iMCD. JSP is a paid consultant for Eversana, Inc. DK, AG, and HK are employees of Eversana, Inc. KK, RM, and FS are employees of EUSA Pharma. SM has received research funding from BMS, Novartis and Jazz Pharmaceuticals; honorarium from EUSA Pharma, Novartis, Celgene/Acceleron, BMS, Blueprint Medicines, Genentech and AbbVie, Aplastic Anemia and MDS International Foundation, EUSA, and Partnership for Health Analytic Research, LLC (PHAR, LLC).

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Mukherjee, S., Kanhai, K., Kauffman, D. et al. Organ dysfunction, thrombotic events and malignancies in patients with idiopathic multicentric castleman disease: a population-level US health claims analysis. Leukemia 36, 2539–2543 (2022).

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