关键词: Castleman病, 透明血管型, 浆细胞型, 巨淋巴结增生症

Abstract:

Objective To describe the clinical features of the hyaline-vascular and plasma-cell variants of Castleman disease (CD) and to raise clinical awareness of their presentation and management. Methods We retrospectively analyzed two cases of different CD subtypes and reviewed the relevant literature to summarize key diagnostic and therapeutic points. Results Case 1 presented with bilateral painless inguinal lymphadenopathy and achieved clinical stabilization with systemic glucocorticoids combined with tocilizumab. Case 2 had generalized lymphadenopathy, low-grade fever, and marked elevation of serum IgE; inflammatory activity was effectively controlled with siltuximab plus immunomodulatory therapy. Both patients experienced diagnostic delay. Definitive diagnosis required histopathological examination and ancillary testing, including interleukin-6 (IL-6) measurement and assessment of immunoglobulin G4 (IgG4)-related markers, to differentiate CD from lymphoma and IgG4-related disease. Conclusion Unicentric CD can often be cured by surgical excision or managed with therapies targeting the IL-6 pathway, while multicentric CD typically requires prolonged multidisciplinary care with emphasis on infection control and immune modulation. Precise subtyping based on pathology and molecular markers is essential to guide individualized treatment plans and to monitor for recurrence and comorbidities.

Key words: Castleman disease, Hyaline-vascular variant, Plasma-cell variant, Giant lymph node hyperplasia