A National, Multicenter, Retrospective Study of Castleman Disease Implementing Cdcn Criteria: 'severe Imcd' Is Indeed Severe

Zhang, Lu; Dong, Yujun; Peng, Hongling; Li, Hao; Zhang, Mingzhi; Wang, Hui-Han; Liu, Qinhua; Su, Liping; Zhong, Liye; Wu, Wenjun; Huang, Liang; Yan, Xiaojing; Fan, Lei; Tang, Wenjiao; Li, Zhenling; Bi, Lintao; Li, Yan; Gao, Guangxun; Gao, Li; Liu, Tingbo; Wei, Yongqiang; Liu, Yao; Yu, Li; Hui, Zhou; Sun, Chunyan; Qian, Wenbin; Zou, Dehui; Zhang, Huilai; Ding, Kaiyang; Wang, Xiaobo; Bai, Ou; Huang, Wenrong; Chen, Bing; Yang, Lin; Song, Jia; Gao, Da; Chen, Tong; Luo, Jun; Wang, Shuye; Ma, Liangming; Li, Jian

doi:10.1182/blood-2022-158675

Cited by 3 publications

(6 citation statements)

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“…6 The inert course of iMCD-IPL was further confirmed in a 20-year data review of 40 centres by Zhang et al. 5 Our study showed significantly better OS in iMCD-IPL compared with iMCD-NOS w/o IPL. Although Kojima et al reported no difference in the 5-year OS rate between the IPL and non-IPL subtypes, we hypothesise that this inconsistency may be mainly due to small sample sizes.…”

Section: Discussionsupporting

confidence: 72%

“…Three sets of diagnostic criteria (Mori et al in 1980, 6 S1) are currently used to diagnose iMCD-IPL, with slight differences. In this study, iMCD-IPL was defined as described by Zhang et al 5 : (1) eligibility for the diagnostic criteria of iMCD-NOS 2 ; (2) elevated serum immunoglobulin G (IgG) level (>17.4 g/L);…”

Section: Patientsmentioning

confidence: 99%

“…4 Recently, some patients with iMCD-NOS have been noted to present with distinct clinical manifestations and thus may be further 'specified'. 5 These patients present with polyclonal hypergammaglobulinaemia, plasmacytic or mixed-type lymph node histopathology and thrombocytosis, which were previously described as iMCD-idiopathic plasmocytic lymphadenopathy (IPL). 6 IPL was initially proposed in 1980 by Mori et al and is characterised by systemic nodal plasmacytosis associated with severe polyclonal hyperimmunoglobulinaemia.…”

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confidence: 99%

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Idiopathic multicentric Castleman disease (iMCD)‐idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD‐not otherwise specified with different clinical features and better survival

Gao,

Liu,

Zhang

et al. 2024

Br J Haematol

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SummaryIdiopathic multicentric Castleman disease (iMCD) is subclassified into iMCD‐thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly (TAFRO) and iMCD‐not otherwise specified (NOS) according to the Castleman Disease Collaborative Network (CDCN) consensus criteria. With a deeper understanding of iMCD, a group of patients with iMCD‐NOS characterised by polyclonal hypergammaglobulinaemia, plasmacytic/mixed‐type lymph node histopathology and thrombocytosis has attracted attention. This group of patients has been previously described as having idiopathic plasmacytic lymphadenopathy (IPL). Whether these patients should be excluded from the current classification system lacks sufficient evidence. This retrospective analysis of 228 patients with iMCD‐NOS identified 103 (45.2%) patients with iMCD‐IPL. The clinical features and outcomes of patients with iMCD‐IPL and iMCD‐NOS without IPL were compared. Patients with iMCD‐IPL showed a significantly higher inflammatory state but longer overall survival. No significant difference in overall survival was observed between severe and non‐severe patients in the iMCD‐IPL group according to the CDCN severity classification. Compared with lymphoma‐like treatments, multiple myeloma‐like and IL‐6‐blocking treatment approaches in the iMCD‐IPL group resulted in significantly higher response rates and longer time to the next treatment. These findings highlight the particularities of iMCD‐IPL and suggest that it should be considered a new subtype of iMCD‐NOS.

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Section: Discussionsupporting

confidence: 72%

Section: Patientsmentioning

confidence: 99%

mentioning

confidence: 99%

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Idiopathic multicentric Castleman disease (iMCD)‐idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD‐not otherwise specified with different clinical features and better survival

Gao,

Liu,

Zhang

et al. 2024

Br J Haematol

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“…A recent study from China characterizing 418 iMCD-NOS patients applied the same definition of severity and found that only 87 (21%) patients met criteria for severe disease at presentation. 13 They also noted a lower 3-year overall survival (OS) among NOS patients with severe (76%) compared to mild/moderate (94%) disease and found that severity at diagnosis was associated with death. Notably, our study showed a high proportion of patients in both younger (<30) and advanced (>60) age groups who presented with severe disease.…”

Section: Discussionmentioning

confidence: 96%

“…11,12 Threeyear survival estimates based on a recent large cohort of patients in China were 65.7% for TAFRO patients, 87.2% for NOS patients without IPL characteristics, and 98.5% for NOS patients with IPL characteristics. 13 In the U.S. and Europe, siltuximab, a monoclonal antibody directed against IL6, is the only approved treatment and firstline recommended therapy. 14,15 Tocilizumab, a monoclonal antibody directed against the IL6 receptor, is recommended when siltuximab is not available and is approved for use in iMCD in Japan.…”

Section: Introductionmentioning

confidence: 99%

Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease

Bustamante,

Pierson,

Ren

et al. 2024

haematol

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Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. iMCD patients present with vastly different clinical subtypes, with some patients demonstrating thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly (TAFRO) and others demonstrating more mild/moderate symptoms with potential for severe disease (not otherwise specified, NOS). Due to its rarity and heterogeneity, the natural history and long-term burden of iMCD are poorly understood. We investigated real-world medical data from ACCELERATE, a large natural history registry of Castleman disease patients, to better characterize the long-term disease burden experienced by these patients. We found that iMCD-TAFRO patients face significant hospitalization burden, requiring more time in the hospital than iMCD-NOS patients during the year surrounding diagnosis (median [IQR] 36 [18, 61] days vs. 0 [0, 4] days; p

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Continuous therapy in HHV-8 negative Multicentric Castleman Disease and prolonged progression-free survival

Liu,

Yin,

Ding

et al. 2024

J Hematol Oncol

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The optimal treatment endpoints and duration of continuous therapy for multicentric Castleman disease (MCD) remain controversial. We retrospectively analyzed data from 123 patients with Human Herpesvirus (HHV)-8 negative MCD. We demonstrated that continuous therapy significantly enhanced progression-free survival (PFS) in patients who achieved an optimal response after initial treatment. These findings underscore the critical role of continuous therapy in HHV-8 negative MCD. Further studies with larger cohorts are required to validate these findings. Supplementary Information The online version contains supplementary material available at 10.1186/s13045-024-01588-9.

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A National, Multicenter, Retrospective Study of Castleman Disease Implementing Cdcn Criteria: 'severe Imcd' Is Indeed Severe

Cited by 3 publications

References 0 publications

Idiopathic multicentric Castleman disease (iMCD)‐idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD‐not otherwise specified with different clinical features and better survival

Idiopathic multicentric Castleman disease (iMCD)‐idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD‐not otherwise specified with different clinical features and better survival

Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease

Continuous therapy in HHV-8 negative Multicentric Castleman Disease and prolonged progression-free survival

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