单次 CD19 CAR-T 细胞输注后难治性特发性多中心 Castleman 病获得持续完全缓解：附机制研究的病例报告

Idiopathic multicentric Castleman disease (iMCD) is rare and life-threatening; although siltuximab is standard first-line therapy, a substantial proportion of patients are refractory or experience relapse.

We report a 31-year-old woman with HHV-8-negative iMCD with idiopathic plasmacytic lymphadenopathy (IPL; iMCD-IPL), intractable to five prior lines of therapy, who declined siltuximab and enrolled in ChiCTR1900025419. After fludarabine/cyclophosphamide lymphodepletion, she received 1 10 6 CAR + T cells/kg of autologous CD19 CAR T cells on day 0. Grade 1 cytokine release syndrome (T max 38. 2 C) resolved with supportive care; no ICANS occurred. CAR transgene levels expanded (peaking on day +11) and remained detectable through day +58, and flow cytometry showed CD19 + B-cell depletion from day +7 to day +198.

By day +198, she met CDCN criteria for complete biochemical and clinical response with normalization of inflammatory markers and hematologic recovery. Panhypogammaglobulinemia was managed with intravenous immunoglobulin replacement. At > 12 months post-infusion, she remains in complete, treatment-free remission. This observation supports a B-cell-centric model for refractory iMCD-IPL and motivates prospective evaluation of CD19 CAR T-cell therapy.