| 韩洪军,封其华,闵月,等.儿童卡斯尔曼病 20例临床诊疗分析[J].安徽医药,2024,28(12):2508-2512. |
| 儿童卡斯尔曼病 20例临床诊疗分析 |
| Clinical analysis of 20 cases of Castleman disease in children |
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| DOI：10.3969/j.issn.1009-6469.2024.12.035 |
| 中文关键词: 卡斯尔曼病 单中心型 多中心型 透明血管型 浆细胞型 儿童 |
| 英文关键词: Castleman disease Monocentric Polycentric Hyaline vascular type Plasma cell type Children |
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| 中文摘要: |
| 目的增加对儿童卡斯尔曼病( CD)的临床表现特点、病理特征及预后的了解，并探讨目前儿童 CD的治疗方案。方法对 2007年 7月至 2022年 12月苏州大学附属儿童医院经过病理诊断的 20例 CD病人的临床表现、病理特征、治疗方案、后等进行回顾性分析。结果 20例病人中男性为 8例，女性为 12例，年龄 7.0~15.6岁。临床分类:单中心型 CD(UCD)14例预(70%)多中心型 CD(MCD)6例(30%)。病理分类:透明血管型( HV)14例(70%)浆细胞型( PC)5例( 25%)混合型 1例( 5%)。手术完全，切除 10例( 50%)均为 UCD，化疗后再手术切除 4例( 20%)完全化疗 6例(，30%)，方案采用 CHOP(环，磷酰胺、阿霉素、长春新碱、泼尼松)、 TCP(沙利度胺、环磷酰胺、泼尼松)、 R-CVP(利妥昔，单抗、环磷酰胺、长春新碱)、托珠单抗联合激素及利妥昔单抗联合大剂量激素治疗。随访至 2023年 4月， 19例均生存， 1例因肺部间质病变及噬血细胞综合征死亡。结论儿童 CD是一种临床少见疾病，以淋巴结肿大为主要表现， UCD较 MCD常见。病理为诊断金标准，病理类型中 HV型较 PC型多见。 |
| 英文摘要: |
| Objective To increase the understanding of the clinical features, pathological features and prognosis of Castleman dis-ease (CD) in children, and to explore the current treatment of CD in children.Methods The clinical manifestations, pathological fea-tures, treatment plan and prognosis of 20 patients with CD diagnosed pathologically in Children's Hospital of Soochow University from July 2007 to December 2022 were retrospectively analyzed.Results Among the 20 patients, 8 were males and 12 were females, aged 7.0~15.6 years. Clinical classification: unicentric type (UCD) 14 cases (70%), multicentric type (MCD) 6 cases (30%). Pathological classification: 14 cases (70%) of clear vascular type (HV), 5 cases (25%) of plasma cell type (PC), 1 case (5%) of mixed type (mixed).UCD was found in 10 patients (50%) who underwent complete surgical resection, and in 4 patients (20%) who underwent re-surgical re-section after chemotherapy, and in 6 patients (30%) who underwent complete chemotherapy. CHOP (cyclophosphamide, doxorubicin,vincristine, prednisone), TCP (thalidomide, cyclophosphamide, prednisone), R-CVP(Rituximab, cyclophosphamide, vincristine) and tozil were adopted Mab combined with hormone and rituximab combined with high-dose hormone therapy. Follow-up to April 2023, 19 cases survived, 1 case died due to pulmonary interstitial disease and hemophagocytic syndrome.Conclusions CD in children is a rare clinical disease, the main manifestation of which is lymph node enlargement, UCD is more common than MCD. Pathology is the gold standard for diagnosis, and HV is more common than PC. The remission rate of UCD after surgical treatment is high, while MCD is mostly treated with drugs. With the improvement of drug treatment program, the remission rate of disease is constantly increasing. |
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