| 梅文内,张冬冬.多中心型卡斯尔曼病与 IgG4相关性疾病的 1例罕见交汇:诊断挑战与个体化治疗探索[J].安徽医药,2026,30(9):1770-1773. |
| 多中心型卡斯尔曼病与 IgG4相关性疾病的 1例罕见交汇:诊断挑战与个体化治疗探索 |
| A rare intersection of multicentric Castleman disease and IgG4-related disease: diagnostic challenges and exploration of individualized treatment |
| |
| DOI：10.3969/j.issn.1009-6469.2026.09.013 |
| 中文关键词: 卡斯尔曼病 多中心型卡斯尔曼病 IgG4相关性疾病 司妥昔单抗 白细胞介素 -6 |
| 英文关键词: Castleman disease Multicentric Castleman disease IgG4-related disease Siltuximab Interleukin-6 |
| 基金项目:国家自然科学基金资助项目( 82200214) |
|
| 摘要点击次数: 327 |
| 全文下载次数: 42 |
| 中文摘要: |
| 目的探讨多中心型卡斯尔曼病(MCD)与免疫球蛋白 G(IgG)4相关性疾病( IgG4-RD)并存的诊断和治疗挑战以及可能的治疗策略。方法总结 2024年 4月襄阳市第一人民医院收治的 1例罕见的 MCD与 IgG4-RD并存病人的诊断与治疗过程。结果男， 38岁，因反复咳嗽 10余天，症状加重 4d就诊。表现为全身多发性淋巴结肿大、贫血、低蛋白血症及显著炎症反应。通过病理检查明确诊断为浆细胞型 MCD合并 IgG4-RD。初始治疗采用 CVP(环磷酰胺、长春新碱和强的松)方案联合硼替佐米，在疗效不佳的情况下，进一步加入抗白细胞介素 -6(IL-6)(司妥昔单抗)治疗及局部放疗。病人症状显著改善，生化指标恢复正常，达到部分缓解。结论该病例凸显了在 MCD与 IgG4-RD重叠病例中， IL-6抑制剂的潜在疗效和局部放疗的有效性。同时，为罕见病的诊疗提供了新思路，并强调了进一步研究其重叠机制及优化治疗策略的必要性。 |
| 英文摘要: |
| Objective To explore the diagnostic and therapeutic challenges as well as potential treatment strategies for coexistingmulticentric Castleman disease (MCD) and IgG4.related disease (IgG4.RD).Methods The diagnostic and therapeutic course of a rarecase of concurrent MCD and IgG4.RD admitted to Xiangyang No. 1 People's Hospital in April 2024 was summarized. Results A 38.year.old male presented with a recurrent cough for over 10 days that had worsened over the preceding 4 days. Clinical manifesta.tions included generalized lymphadenopathy, anemia, hypoalbuminemia, and marked inflammatory response. Pathological examinationestablished the diagnosis of plasma.cell.variant MCD complicated by IgG4.RD. Initial treatment consisted of the CVP (cyclophospha.mide, vincristine, prednisone) regimen combined with bortezomib. Due to an inadequate response, the anti-interleukin-6 (IL-6) agentsiltuximab and local radiotherapy were further administered. The patient achieved significant symptomatic improvement, normalizationof biochemical markers, and a partial response.Conclusions This case underscores the potential efficacy of IL.6 inhibition and localradiotherapy in complex overlapping conditions. It offers new perspectives for the diagnosis and management of rare diseases. Furtherinvestigations are needed to clarify the mechanisms underlying MCD.IgG4.RD overlap and optimize therapeutic strategies. |
|
查看全文
查看/发表评论 下载PDF阅读器 |
| 关闭 |
