[Clinicopathologic features and treatment of 13 cases of Castleman's disease]

Objective: To analyze and summarize the clinical and pathological characteristics, therapeutic strategies, and prognostic outcomes of adult patients with Castleman disease. Methods: In this single-center case series, clinical data from 13 adult patients treated for confirmed Castleman disease at Zhongda Hospital Affiliated with Southeast University between April 2020 and December 2024 were retrospectively analyzed. Primary observation indicators included demographic characteristics, clinical manifestations, histological classification, and findings from laboratory and imaging examinations. Treatment modalities and prognostic outcomes were also assessed. Fisher's exact test was used for group comparisons. Results: Among the 13 patients, 7 were male and 6 were female, with a median age at onset of 49 years (range, 22-71 years). Three patients were diagnosed with unicentric Castleman disease (UCD), whereas 10 presented with multicentric Castleman disease (MCD). Most UCD patients were asymptomatic systemically (2/3), while one patient presented with paraneoplastic pemphigoid (PNP). All MCD patients exhibited generalized lymphadenopathy at multiple sites; seven had serous cavity effusions, and three demonstrated renal involvement. Diagnosis was confirmed via lymph node or mass biopsy, with pathological classification revealing seven cases of hyaline vascular type, five cases of plasma cell type, and one case of mixed type. No statistically significant differences were observed in pathological subtypes or immunohistochemical staining results between MCD and UCD patients (all P>0.05). Laboratory findings showed that only the median levels of C-reactive protein and the median percentage of reticulocytes in MCD patients were above the normal reference range, while the median or mean levels of all other detectable indicators were within normal limits in both MCD and UCD patients. Treatment was tailored to clinical subtype, surgical feasibility, and disease characteristics. Five patients with localized disease and no systemic symptoms (2 UCD, 3 MCD) underwent surgery alone; two with localized disease and comorbidities (1 UCD with PNP, 1 human herpesvirus 8-positive MCD) received surgery plus pharmacotherapy; four MCD patients with multicentric lymphadenopathy and multiorgan involvement received pharmacotherapy alone; and two MCD patients with superficial lesions and no systemic symptoms were managed by observation alone. All patients improved and were discharged. At a median follow-up of 12 months (range, 6-17 months), all remained alive with no recurrence. Conclusions: Adult UCD was predominantly asymptomatic systemically or localized, whereas MCD was associated with generalized lymphadenopathy and multisystem involvement. Treatment for UCD primarily involved surgical resection, while MCD required comprehensive pharmacotherapy. Prognosis was favorable for UCD, and MCD patients also achieved remission following treatment; however, long-term follow-up remains essential.