A phase 2 prospective study of bortezomib, cyclophosphamide, and dexamethasone in patients with newly diagnosed iMCD

Letter to Blood| May 25, 2023 A phase 2 prospective study of bortezomib, cyclophosphamide, and dexamethasone in patients with newly diagnosed iMCD Clinical Trials & Observations Hao Zhao, Hao Zhao 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0003-0736-9700 Search for other works by this author on: This Site PubMed Google Scholar Miao-yan Zhang, Miao-yan Zhang 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0002-7776-3642 Search for other works by this author on: This Site PubMed Google Scholar Kai-ni Shen, Kai-ni Shen 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China Search for other works by this author on: This Site PubMed Google Scholar Jun Feng, Jun Feng 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0001-6631-6129 Search for other works by this author on: This Site PubMed Google Scholar Xin-xin Cao, Xin-xin Cao 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0001-7884-3073 Search for other works by this author on: This Site PubMed Google Scholar Ming-hui Duan, Ming-hui Duan 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0003-2130-6300 Search for other works by this author on: This Site PubMed Google Scholar Dao-bin Zhou, Dao-bin Zhou 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0002-1592-1932 Search for other works by this author on: This Site PubMed Google Scholar Lu Zhang, Lu Zhang 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0002-0860-9625 Search for other works by this author on: This Site PubMed Google Scholar Jian Li Jian Li 1Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China https://orcid.org/0000-0002-4549-0694 Search for other works by this author on: This Site PubMed Google Scholar Blood (2023) 141 (21): 2654–2657. https://doi.org/10.1182/blood.2023020009 Article history Submitted: February 6, 2023 Accepted: March 11, 2023 First Edition: March 16, 2023 Share Icon Share Facebook Twitter LinkedIn Email Tools Icon Tools Request Permissions Cite Icon Cite Search Site Citation Hao Zhao, Miao-yan Zhang, Kai-ni Shen, Jun Feng, Xin-xin Cao, Ming-hui Duan, Dao-bin Zhou, Lu Zhang, Jian Li; A phase 2 prospective study of bortezomib, cyclophosphamide, and dexamethasone in patients with newly diagnosed iMCD. Blood 2023; 141 (21): 2654–2657. doi: https://doi.org/10.1182/blood.2023020009 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search Search Dropdown Menu toolbar search search input Search input auto suggest filter your search All ContentAll JournalsBlood Search Subjects: Clinical Trials and Observations, Lymphoid Neoplasia TO THE EDITOR: Human herpesvirus 8 (HHV-8)–negative idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder.1 Patients with iMCD experience a wide spectrum of life-threatening cytokine storm–related symptoms including fatigue, fever, anorexia, weight loss, and ascites.2 A subset of patients with iMCD have shared manifestations, including thrombocytopenia, anasarca/ascites, reticulin fibrosis in bone marrow, renal dysfunction, and organomegaly (TAFRO) and are identified as having the iMCD-TAFRO clinical subtype.3 Patients with iMCD not meeting the criteria for iMCD-TAFRO are considered to have iMCD-not otherwise specified (iMCD-NOS). Interleukin-6 (IL-6) is known to play a key role in a portion of patients. Siltuximab, as an IL-6–directed monoclonal antibody, induced a complete or partial response rate of 34% in an international randomized controlled trial and showed a significantly improved progression-free survival (median, 14.5 months) compared with the placebo group.4,5... References 1.Fajgenbaum DC, Uldrick TS, Bagg A, et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-1657.Google ScholarCrossrefSearch ADS PubMed 2.Dispenzieri A, Armitage JO, Loe MJ, et al. The clinical spectrum of Castleman's disease. Am J Hematol. 2012;87(11):997-1002.Google ScholarCrossrefSearch ADS PubMed 3.Kawabata H, Takai K, Kojima M, et al. Castleman-Kojima disease (TAFRO syndrome): a novel systemic inflammatory disease characterized by a constellation of symptoms, namely, thrombocytopenia, ascites (anasarca), microcytic anemia, myelofibrosis, renal dysfunction, and organomegaly: a status report and summary of Fukushima (6 June, 2012) and Nagoya meetings (22 September, 2012). J Clin Exp Hematop. 2013;53(1):57-61.Google ScholarCrossrefSearch ADS 4.van Rhee F, Wong RS, Munshi N, et al. Siltuximab for multicentric Castleman's disease: a randomised, double-blind, placebo-controlled trial. Lancet Oncol. 2014;15(9):966-974.Google ScholarCrossrefSearch ADS PubMed 5.van Rhee F, Rosenthal A, Kanhai K, et al. Siltuximab is associated with improved progression-free survival in idiopathic multicentric Castleman disease. Blood Adv. 2022;6(16):4773-4781.Google ScholarCrossrefSearch ADS PubMed 6.Mukherjee S, Martin R, Sande B, Paige JS, Fajgenbaum DC. Epidemiology and treatment patterns of idiopathic multicentric Castleman disease in the era of IL-6-directed therapy. Blood Adv. 2022;6(2):359-367.Google ScholarCrossrefSearch ADS PubMed 7.Zhang L, Zhao AL, Duan MH, et al. Phase 2 study using oral thalidomide-cyclophosphamide-prednisone for idiopathic multicentric Castleman disease. Blood. 2019;133(16):1720-1728.Google ScholarCrossrefSearch ADS PubMed 8.Zhang L, Zhang MY, Cao XX, et al. A prospective, multicenter study of bortezomib, cyclophosphamide, and dexamethasone in relapsed/refractory iMCD. Leuk Lymphoma. 2022;63(3):618-626.Google ScholarCrossrefSearch ADS PubMed 9.Kumar S, Flinn I, Richardson PG, et al. Randomized, multicenter, phase 2 study (EVOLUTION) of combinations of bortezomib, dexamethasone, cyclophosphamide, and lenalidomide in previously untreated multiple myeloma. Blood. 2012;119(19):4375-4382.Google ScholarCrossrefSearch ADS PubMed 10.Fajgenbaum DC, June CH. Cytokine storm. N Engl J Med. 2020;383(23):2255-2273.Google ScholarCrossrefSearch ADS 11.Zhang S, Kulkarni AA, Xu B, et al. Bortezomib-based consolidation or maintenance therapy for multiple myeloma: a meta-analysis. Blood Cancer J. 2020;10(3):33.Google ScholarCrossrefSearch ADS PubMed © 2023 by The American Society of Hematology2023 © 2023 by The American Society of Hematology2023 You do not currently have access to this content. Sign in via your Institution