累及儿童多部位的浆细胞型Castleman病1例并文献复习

Multicentric plasma cell type Castleman disease involving multiple sites in children:a case report and literature review

目的探讨多中心型Castleman病的临床表现、诊断依据、鉴别诊断和治疗方法。方法回顾性分析1例病理类型为浆细胞型的多中心型Castleman病患儿的临床资料,并对相关文献进行总结。结果多中心型Castleman病的发病机制尚不明确,通常无典型临床表现及病理特征,诊断极为困难。患儿治疗采用利妥昔单抗静滴,期间辅以地塞米松、抗生素和丙种球蛋白控制鼻部感染和渗出。治疗28 d后全身病损明显消退,血生化指标逐渐恢复,但嗜酸性粒细胞及炎症因子仍维持较高水平。随访13个月,患儿恢复良好,无不良反应及后遗症发生。结论多中心型Castleman病是一种罕见的淋巴增生性疾病,发生于鼻部的极为少见,目前的治疗方案主要针对于成人,儿童方面鲜有报道,仍需个性化制定治疗措施。对于难以手术切除和治疗效果不佳的患儿,利妥昔单抗是安全有效的治疗药物。

Objective To explore the pathogenesis,clinical presentation,diagnostic basis,differential diagnosis,and treatment options for multicentric Castleman disease.Methods Clinical data of a child diagnosed with multicentric Castleman disease of the plasma cell type were retrospectively analyzed,and the relevant literature was summarized.Results Multicentric Castleman disease is characterized by unclear pathogenesis and often lacks typical clinical manifestations and pathological features,making its diagnosis extremely challenging.In this case,the child received treatment with rituximab static drops,supplemented with dexamethasone,antibiotics,and gamma globulin to control the nasal infection and exudation.Following 28 days of treatment,the systemic disease subsided significantly and the blood biochemical index gradually recovered.After 13 months,the child had recovered well with no adverse effects or sequelae.Conclusion Multicentric Castleman disease is a rare lymphoproliferative disease,with rare occurrence in the nasal region.The current treatment plans mainly focus on adults,with limited reporting on children.Personalized treatment measures still need to be developed for children.Rituximab is a safe and effective treatment option for children with challenging surgical resection and unfavorable treatment outcomes.