Annals of Hematology

, Volume 97, Issue 9, pp 1641–1647 | Cite as

Effectiveness of rituximab-containing treatment regimens in idiopathic multicentric Castleman disease

  • Yujun Dong
  • Lu Zhang
  • Lin Nong
  • Lihong Wang
  • Zeyin Liang
  • Daobin Zhou
  • David C. Fajgenbaum
  • Hanyun Ren
  • Jian Li
Original Article


Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. To evaluate the efficacy of rituximab in this rare and heterogeneous disease, clinical and pathological data from 27 cases of iMCD were retrospectively analyzed from two large medical centers in China. The novel diagnostic criteria for iMCD were applied, and POEMS syndrome, IgG4-related diseases, and follicular dendritic cell sarcomas cases were excluded from analyses. Total response rate of rituximab- and cyclophosphamide-containing regimens was 55.5%, with 33.3% (9/27) of the cases reaching CR and 22.2% (6/27) PR. In the 14 cases of R-R iMCD, total response rate was only 42.9% (CR 14.3% [2/14], PR 28.6% [4/14]). The 5-year OS of these 27 iMCD cases was 81% (95% CI 64–98; 27 total patients, 4 events, 23 censored) after receiving these regimens, but the 5-year PFS was 43% (95% CI 19–66; 25 total patients, 11 events, 14 censored). Thus, rituximab-based regimens should be considered for the treatment of iMCD patients when siltuximab is not available and potentially in siltuximab-refractory cases.


Idiopathic multicentric Castleman disease Rituximab Lymphoproliferative disease 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Supplementary material

277_2018_3347_MOESM1_ESM.docx (96 kb)
Supplement Table 3 (DOCX 95 kb)
277_2018_3347_MOESM2_ESM.jpg (214 kb)
Supplement Fig. 3 The flowchart of procedure of patients screening. POEMS: POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin pigmentation); SLE: systemic lupus erythematosus; IgG4RD: IgG4-related diseases; FDCS: follicular dendritic cell sarcomas; HHV8: human herpes virus type 8; CN: Chemotherapy naïve iMCD; R-R: Refractory-relapsed iMCD (JPG 214 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of HematologyPeking University First HospitalBeijingChina
  2. 2.Department of Hematology, Peking Union Medical College HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina
  3. 3.Department of PathologyPeking University First HospitalBeijingChina
  4. 4.Division of Translational Medicine and Human Genetics, Perelman School of MedicineUniversity of PennsylvaniaPhiladelphiaUSA

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