Clinical characteristics and therapeutic effect of 38 cases of unicentric Castleman disease
10.3760/cma.j.cn115356-20250317-00042
- VernacularTitle:单中心型Castleman病38例临床特征与疗效
- Author:
Fujin SUN
1
;
Yu PAN
;
Qian WU
;
Tong WANG
;
Shuhua YI
Author Information
1. 山东省立医院菏泽医院 菏泽市立医院血液内科,菏泽 274000
- Keywords:
Unicentric Castleman disease;
Pathological conditions, signs and symptoms;
Treatment outcome
- From:
Journal of Leukemia & Lymphoma
2025;34(8):472-476
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To explore the clinical characteristics and influencing factors of therapeutic effect of unicentric Castleman disease (UCD).Methods:A retrospective case series study was conducted. The clinical data of 38 patients with UCD who were admitted to Heze Municipal Hospital, Tianjin First Central Hospital or Hematology Hospital of Chinese Academy of Medical Sciences from January 2015 to October 2024 were collected, and their general data, clinical manifestations, laboratory tests, pathological types, treatment methods and therapeutic effect were analyzed. Multivariate logistic regression model was used to analyze the independent influencing factors of incomplete remission of UCD after treatment.Results:Among the 38 patients, 20 (52.6%) were male and 18 (47.4%) were female, with the age of (36±12) years; there were 11 cases (28.9%) of fever, 10 cases (26.3%) of fatigue and 9 cases (23.7%) of night sweats. Cervical lymphadenopathy was most commonly found (17 cases, 44.7%); there were 10 cases (26.3%) of anemia, 5 cases (13.2%) of leukocytosis, 8 cases (21.1%) of thrombocytosis, 18 cases (47.4%) of elevated C-reactive protein (CRP), 18 cases (47.4%) of elevated erythrocyte sedimentation rate (ESR), 11 cases (28.9%) of elevated IgG, 4 cases (10.5%) of elevated IgA, and 7 cases (18.4%) of elevated IgM; the main pathological type was hyaline vascular type (25 cases, 65.8%), 7 cases (18.4%) were plasma cell type, and 6 cases (15.8%) were mixed type. Twenty-seven cases (71.1%) underwent surgery at the beginning (20 cases received complete resection), the other 11 cases (28.9%) were treated by drugs to get tumor shrinkage, and among them, 8 cases were re-operated (5 cases received complete resection, 3 cases received partial resection). There were complete remission in 24 cases (63.2%), partial remission in 7 cases (18.4%), disease stability in 5 cases (13.2%) and disease progression in 2 cases (5.3%). In the incomplete remission group, the proportion of patients with hyaline vascular type was lower than that in the complete remission group [35.7% (5/14) vs. 83.3% (20/24)], elevated ESR [78.6% (11/14) vs. 29.2% (7/24)] and elevated CRP [71.4% (10/14) vs. 33.3% (8/24)] were higher than those in the complete remission group, and the differences were statistically significant (all P < 0.05); there were no statistically significant differences in the proportions of patients with different gender, age > 36 years, fatigue, night sweats, fever, elevated white blood cells, elevated platelets, anemia, lymphadenopathy, elevated IgG, elevated IgA, and elevated IgM between the two groups (all P > 0.05). Multivariate logistic regression analysis showed that the plasma cell or mixed pathological type (compared with hyaline vascular type, OR = 4.32, 95% CI: 1.47-12.67, P = 0.008) and elevated ESR (compared with non-elevated ESR, OR = 3.56, 95% CI: 1.25-10.13, P = 0.016) were independent risk factors for incomplete remission of UCD patients. Conclusions:The clinical manifestations of UCD patients are heterogeneous; ESR and pathological type may be the influencing factors of therapeutic effect, and they are the potential indicators for evaluating the therapeutic effect of such patients.
