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Ann Hematol. 2018 Sep;97(9):1641-1647. doi: 10.1007/s00277-018-3347-0. Epub 2018 May 7.

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

Author information

1
Department of Hematology, Peking University First Hospital, No. 8 Xishiku St., Xicheng District, Beijing, 100034, China.
2
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 1# Shuai Fu Yuan, Dongcheng District, Beijing, 100730, China.
3
Department of Pathology, Peking University First Hospital, Beijing, China.
4
Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
5
Department of Hematology, Peking University First Hospital, No. 8 Xishiku St., Xicheng District, Beijing, 100034, China. renhy0813@163.com.
6
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 1# Shuai Fu Yuan, Dongcheng District, Beijing, 100730, China. lijian@pumch.cn.

Abstract

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.

KEYWORDS:

Idiopathic multicentric Castleman disease; Lymphoproliferative disease; Rituximab

PMID:
29732477
DOI:
10.1007/s00277-018-3347-0
[Indexed for MEDLINE]

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