Clinical features and prognosis of Castleman disease in 29 children
10.3760/cma.j.cn141217-20250429-00136
- VernacularTitle:儿童Castleman病29例临床特征及预后分析
- Author:
Xiaofei ZHU
1
;
Zhaoling WANG
;
Lifeng ZHANG
;
Xiaojun XU
;
Meiping LU
Author Information
1. 浙江大学医学院附属儿童医院风湿免疫过敏科，杭州 310052
- Publication Type:Journal Article
- Keywords:
Child;
Castleman disease;
Therapy;
Prognosis;
Paraneoplastic pemphigus
- From:
Chinese Journal of Rheumatology
2025;29(7):583-589
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features, treatment strategies, and prognosis of pediatric Castleman disease (CD), with the aim of enhancing disease recognition and improving diagnostic and therapeutic approaches.Methods:A retrospective analysis was conducted on the clinical data of 29 children diagnosed with CD at the Children′s Hospital, Zhejiang University School of Medicine, between January 2016 and January 2024. Age, sex, initial presentations, routine blood test, inflammatory indicators, coagulation function test, immunoglobulin level, histopathological examination, imaging examination, treatment and follow-ups were recorded. Patients were classified into two groups based on the presence or absence of symptoms: the symptomatic Castleman disease group (SC) and the asymptomatic Castleman disease group (AC). All patients were followed for a minimum of six months. Clinical characteristics, therapeutic regimens, and outcomes were compared between the two groups.For the measurement information, the independent samples t test was used for comparison between groups of data that conformed to normal distribution; the Mann-Whitney U test was used for comparison between groups of data that did not conform to normal distribution. The Chi-square test or Fisher′s exact probability method was used for comparison between groups of count data. Results:A total of 29 pediatric CD cases were included, comprising 15 males and 14 females. The number of patients diagnosed as unicentric Castleman disease (UCD) was 21, with the rest 8 as multicentric Castleman disease (MCD). There were 11 patients (37.9%) in the SC group and 18 patients (62.1%) in the AC group. The median age at onset was 10.0 years (interquartile range: 6.5-12.2 years). The most common initial presentation was mass ( n=20), followed by fever ( n=2), amaemia ( n=2), fever with fatigue ( n=2), rash ( n=1), abdominal pain ( n=1), and cough ( n=1). The frequently involved sites were neck ( n=8), abdominal cavity ( n=5), mediastinum ( n=3), retroperitoneum ( n=2), and one case each in the back, upper arm, and pelvis. The maximum lesion volume reached 1 040 cm 3. Compared to the AC group, the SC group had significantly lower hemoglobin levels [108.0(92.0, 123.0)g/L vs. 127.0(117.5, 139.0)g/L, Z=-2.35, P=0.019] and significantly higher levels of C-reactive protein (CRP) [38.0(3.0, 87.0)mg/L vs. 0.6(0.5, 3.8)mg/L, Z=-2.19, P=0.029], prothrombin time[12.6(11.4, 13.3)s vs. 11.3(10.5, 11.7)s, Z=-2.64, P=0.008], and fibrinogen [4.5(3.5, 5.4)g/L vs. 2.1(1.9, 2.6)g/L, Z=-3.04, P=0.002]. All patients underwent MRI/CT and ultrasonography; however, only 2 cases were diagnosed as CD by ultrasound. All patients underwent surgical excision of the mass, and diagnoses were confirmed by histopathological examination. Among the SC group, 4 patients (4/11, 36.4%) required postoperative treatment with glucocorticoids alone or in combination with immunosuppressants and biologics. The overall prognosis was favorable, with follow-up ranging from 6 months to 7 years and no reported mortality. One case, initially misdiagnosed as systemic lupus erythematosus, was later confirmed to be CD complicated with paraneoplastic pemphigus (PNP); this patient experienced recurrent fever, rash, and elevated CRP, and continued to require low-dose glucocorticoids and tacrolimus during the 4-year follow-up. Conclusion:In chileren, CD is most common in the neck and surgical resection yields favorable outcomes. Approximately 37.9% of patients present with systemic symptoms, of which about 36.4% require postoperative systemic therapy such as glucocorticosteroids. CD complicated with PNP may be misdiagnosed. Symptomatic cases are often associated with anemia, elevated CRP levels, and coagulation abnormalities. UCD pediatric patients without complications have a good prognosis and there′s no need for long-term medications after surgery. About 37.5% of MCD pediatric patients require glucocorticoids, immunosuppressants, biotherapy or chemotherapy to control symptoms in the postoperative period. Imaging modalities have limited diagnostic value, and histopathological examination remains the gold standard for diagnosis.
