Clinical Characteristics, Pathological Insights, and Treatment Results of Castleman Disease
Overview
This study presents a retrospective analysis of 12 patients with Castleman disease (CD), highlighting clinical manifestations, treatment outcomes, and the effectiveness of various therapeutic approaches.
Background
Castleman disease is a rare lymphoproliferative disorder with significant clinical heterogeneity and a low incidence rate. Accurate diagnosis and effective treatment are critical, especially given the challenges in distinguishing CD from other conditions in clinical practice. This study aims to provide real-world evidence from a Chinese cohort.
Data Highlights
Characteristic
Details
Number of Patients
12
Gender
10 males, 2 females
Age Range
20–67 years (median 45 years)
Diagnosis
7 unicentric CD, 5 multicentric CD
Follow-up Outcomes
All patients alive, no recurrence in UCD cases
Key Findings
7 patients diagnosed with unicentric CD (5 hyaline vascular type, 2 mixed type).
5 patients diagnosed with multicentric CD (4 plasma cell type, 1 mixed type).
All patients tested negative for HHV-8 and HIV.
Complete surgical resection in UCD cases resulted in no recurrence.
MCD patients treated with R-CHOP achieved favorable outcomes with no recurrence during follow-up.
Oral TCD regimen was effective for a refractory MCD case.
Clinical Implications
Multicentric CD typically necessitates systemic therapy. In settings lacking access to anti-IL-6 therapies, R-CHOP may serve as a treatment option for idiopathic multicentric Castleman disease.
Conclusion
This analysis provides insights into the clinical management of Castleman disease.