The CDCN is dedicated to providing high-impact and updated information about Castleman disease to all of our members. Below is a list of recent and significant Castleman disease publications with corresponding full-text versions. 


Treatment Guidelines: van Rhee F, et al. “International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease.” Blood. 2018. (full-text). **CDCN led**

Diagnostic Criteria: Fajgenbaum D, et al. “International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease.” Blood. 2017. (full-text). **CDCN led**

Translational Research Findings:

Arenas D et al. “Increased mTOR activation in idiopathic multicentric Castleman disease.” Blood. 2020. (full-text). **CDCN led**

Pai RA et al. “Type I IFN response associated with mTOR activation in the TAFRO subtype of idiopathic multicentric Castleman disease. JCI Insight. 2020. (full-text). **CDCN led**

Fajgenbaum, D. “Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease.” Blood. 2018. (full-text). **CDCN led**

Morra D, et al. “Predictors of response to anti-IL6 monoclonal antibody therapy (siltuximab) in idiopathic multicentric Castleman disease: secondary analyses of phase II clinical trial data.” British Journal of Haematology. 2018. (full-text). **CDCN led**

Pierson S, et al. “Plasma proteomics identifies a ‘chemokine storm’ in idiopathic multicentric Castleman disease.” American Journal of Hematology. 2018. (full-text). **CDCN led (SPEED I study; 1 minute video here)**

Baker T, et al “A novel FAS mutation with variable expressivity in a family with unicentric and idiopathic multicentric Castleman disease” Blood Advances, 2018.

Nagy A, et al. “Next-generation sequencing of idiopathic multicentric and unicentric Castleman disease and follicular dendritic cell sarcoma.” Blood Advances. 2018. (full-text)

Fajgenbaum D, Shilling D. “Castleman Disease Pathogenesis.” Hematology/Oncology Clinics of North America. 2017. (full-text). **CDCN led**

To access the full textbook, email

Fajgenbaum D, van Rhee F, Nabel C. “HHV-8-negative or idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and treatment.” Blood. 2014; 123(19): 2924-2933. (full-text) **CDCN led**

Kunch-Choa Chang et al. “Monoclonality and cytogenetic abnormalities in hyaline vascular Castleman disease.” Modern Pathology. 2014; 27(6):823-831.

Stone K et al. “Interleukin-6 Receptor Polymorphism Is Prevalent in HIV- negative Castleman Disease and Is Associated with Increased Soluble Interleukin-6 Receptor Levels.” PLOS one. 2013; 8(1):e54610. (full-text) **CDCN led**

Clinico-Pathological Characterizations of iMCD and TAFRO Syndrome Subtype of iMCD:

Oksenhendler E, et al. “The full spectrum of Castleman disease: 273 patients studied over 20 years.” British Journal of Haematology. 2018. (full-text). **CDCN co-led**

Li Y, et al. “Clinical and pathological charactersistics of HIV- and HHV8- negative Castleman disease.” Blood. 2017. (full-text). **CDCN co-led**

Noriko Iwaki, David C. Fajgenbaum, Christopher S. Nabel, et al. “Clinicopathologic analysis of TAFRO syndrome demonstrates a distinct subtype of HHV-8-negative multicentric Castleman disease.” American Journal of Hematology 2016. 2016; 91:220–226. (full-text) **CDCN co-led**

To access the above article translated into Portuguese, please click here

Louis C, et al. “TAFRO Syndrome in Caucasians: A Case Report and Review of the Literature.” Frontiers in Medicine. 2017. (full-text) **CDCN co-led**

Srkalovic G, et al. “TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease.” Bosn J Basic Med Sci. 2017. (full-text) **CDCN led**

Clinical Case Series of Treatment Data and Reviews of Treatments:

Dong Y, et al. “Effectiveness of rituximab-containing treatment regimens in idiopathic multicentric Castleman disease.” Annals of Hematology. 2018. (full-text). **CDCN co-led**

Fajgenbaum D, Kurzrock R. “Siltuximab: a targeted therapy for idiopathic multicentric Castleman disease.” Immunotherapy. 2016; 8(1), 17–26. **CDCN led**

Liu A et al “Idiopathic multicentric Castleman’s disease: a systematic literature review.” Lancet Haematology. 2016. (full-text) **CDCN led**

van Rhee F, Fajgenbaum D. “Ask the Hematologist: Treatment of Castleman disease.” The Hematologist. 2015; 2(12):4-5. **CDCN led**

van Rhee F, et a. “Siltuximab for multicentric Castleman’s disease: a randomised, double-blind, placebo-controlled trial.” The Lancet Oncology. 2014; 15(9): 966-974. **CDCN led**

Markham A, Patel T. “Siltuximab: first global approval.” Drugs. 2014; 74(10): 1147-1152.

Turcotte LM et al. “Sustained remission of severe Multicentric Castleman disease following multiagent chemotherapy and tocilizumab maintenance.” Pediatric Blood Cancer. 2014; 61(4): 737-739.

Kurzock R et al. “A phase I, open-label study of siltuximab, an anti-IL-6 monoclonal antibody, in patients with B-cell non-Hodgkin lymphoma, multiple myeloma, or Castleman disease.” Clin. Cancer Res. 2013; 19(13): 3659-3670. **CDCN led**

El-Osta H, Janku F, Kurzrock R. “Successful treatment of Castleman’s disease with interleukin-1 receptor antagonist (Anakinra).” Mol. Cancer Ther. 2010; 9(6):1485-1488.

Matsuyama M et al. “Anti-interleukin-6 receptor antibody (tocilizumab) treatment of multricentric Castleman’s disease.” Intern Med. 2007; 46(11):771-774.

Nishimoto N. “Humanized anti–interleukin-6 receptor antibody treatment of multicentric Castleman disease.” Blood. 2005. 106(8):2627-2632.


Dispenzieri A, Fajgenbaum DC. “Overview of Castleman disease.” Blood. 2020;135(16):1353‐1364. doi:10.1182/blood.2019000931 (full-text

Fajgenbaum D. “Leveraging information and collaboration to cure disease.” Information Services & Use. 37 (2017) 307-311.

Fajgenbaum D, Ruth J, Kelleher D, Rubenstein A. “The collaborative network approach: a new framework for accelerating Castleman’s disease and other rare disease research.” Lancet Haematology. 2016. (full-text) **CDCN led**

To access the above article translated into Portuguese, please click here.  

Williamson H, et al. “Rare disease research requires (and benefits from) global collaboration: three examples from the Castleman Disease Collaborative Network.” Rev Med (Sao Paulo) 2016. (full-text) **CDCN led**

Newman S at al. “Taking Control of Castleman disease: Leveraging Precision Medicine Technologies to Accelerate Rare Disease Research.” Yale Journal of Biology & Medicine. 2015. (full-text) **CDCN led** 


Thomas S Uldrick, Mark N Polizzotto, Robert Yarchoan. “Recent advances in Kaposi sarcoma herpesvirus-associated multicentric Castleman disease.” Current Opinion in Oncology. 2012; 24(5):495-505


Bowne W, et al. “The Management of Unicentric and Multicentric Castleman’s Disease: A Report of 16 Cases and a Review of the Literature.” Cancer. 1999; 85(3): 706-717.

Talat N, Belgaumkar AP, Schulte KM. “Surgery in Castleman’s disease: a systematic review of 404 published cases.” Ann Surg. 2012; 255:677.


van Rhee F, et al. “Castleman disease in the 21st century: an update on diagnosis, assessment, and therapy.” Clin Adv Hematol Oncol. 2010; 8(7):486-98.


Jun Z, et al. “Clinical and Experimental Study of Castleman Disease in Children.” Pediatric Blood & Cancer. 2014. DOI 10.1002/pbc

Burlakov V, Kozlova A, Shcherbina A, et al. “Clinical Characterization of Castleman’s Disease in A Group of Pediatric Patients.” Annals of the Rheumatic Diseases. 2016;75:404-405.

Benmiloud S, Chaouki S, Atmani S, Hida M. “Multicentric Castleman’s Disease in a Child Revealed by Chronic Diarrhea.” Case Reports in Pediatrics, vol. 2015, Article ID 689206, 4 pages, 2015.


Soudet S, et al. “Schnitzler syndrome co-occurring with idiopathic multicentric Castleman disease that responds to anti-IL-1 therapy: A case report and clue to pathophysiology.” Current Research Translational Medicine. 2018. (full-text). **CDCN co-led**

Behnia F, Elojeimy S, Matesan M, Fajgenbaum D. “Potential value of FDG PET-CT in diagnosis and follow-up of TAFRO syndrome.” Ann Hematol. 2016. (full-text) **CDCN co-led**

Fajgenbaum D, et al. “Eruptive cherry hemangiomatosis associated with multicentric Castleman disease.” JAMA Dermatology. 2013. (full-text) **CDCN led**

Dong Y, et al. ” Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor.” British Journal of Haematology. 2015. (full-text


Pierson S, et al. “Quantification of Plasma Proteins from Idiopathic Multicentric Castleman Disease Flares and Remissions Reveals ‘Chemokine Storm’ and Separates Clinical Subtypes.” Blood 2017. 130:3592. **CDCN led**

Fajgenbaum D, et al. “Prolonged Remission Achieved in a Relapsing Idiopathic Multicentric Castleman Disease Patient with a Novel, Targeted Treatment Approach.” Blood 2017. 130:3593. **CDCN led**

Nabel S, et al. “Virome Capture Sequencing in Castleman Disease Identifies Associations with Herpesviridae Family Members but No Novel Viruses.” Blood 2017. 130:3589. **CDCN led**

Fajgenbaum D, et al. “Preliminary Results from Accelerate, an International, Web-Based, Natural History Registry of Castleman Disease.” Blood 2017. 130:4647. **CDCN led**

Fajgenbaum D, et al. “HHV-8-Negative, Idiopathic Multicentric Castleman Disease (iMCD): A Description of Clinical Features and Therapeutic Options through a Systematic Literature Review.” Blood 2014. 124:4861. **CDCN led**

**Abbreviations: UCD = unicentric Castleman disease, MCD = multicentric Castleman disease, CD = Castleman disease, HHV-8 = Human Herpes Virus-8**

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