儿科原发性持续性淋巴细胞B细胞淋巴瘤呈现为血瘤在前端对面区域:一个病例报告
Ayşe Ceyda Ören1, Esra Pekpak Şahinoğlu1, Sibel Cangi2
1Department of Pediatrics, Division of Pediatric Hematology-Oncology.
Journal of pediatric hematology/oncology
|September 12, 2024
概括
本报告详细介绍了第一例小儿原发性淋巴细胞B细胞淋巴瘤的病例,这是一种罕见的中枢神经系统淋巴瘤. 这名3岁的患者成功完成了治疗,并且仍然没有疾病,突出显示了一种新的治疗方法.
科学领域:
- 儿科瘤学 儿科瘤学
- 神经病理学神经病理学
- 血液学 血液学 血液学
背景情况:
- 主要的中枢神经系统淋巴瘤 (PCNSL) 很少见,其脑膜起源和没有系统性参与是非常罕见的.
- 儿科持续性淋巴瘤几乎没有报告,这给诊断和治疗带来了挑战.
研究的目的:
- 报告了首例儿科原发性硬性淋巴细胞B细胞淋巴瘤的确诊病例.
- 描述这种罕见疾病的临床表现,诊断工作和成功管理.
主要方法:
- 3岁男孩头痛,恶心和吐的临床表现.
- 诊断成像包括大脑CT和FDG-PET/CT.
- 持续质量的手术切除,随后进行体质病理学检查和免疫组织化学.
- 骨髓检查以排除系统性参与.
- 根据NHL BFM 2012淋巴细胞淋巴瘤协议进行治疗.
主要成果:
- 组织病理学证实了淋巴细胞性B细胞淋巴瘤,其Ki-67扩散指数高 (80%).
- 影像成像和骨髓活检排除了全身性疾病,证实了初级体内源.
- 该患者完成了NHL BFM 2012协议,并在约2年的随访期间保持无疾病.
结论:
- 这一病例代表了首次报告的儿科原发性硬性淋巴细胞B细胞淋巴瘤.
- 使用标准的淋巴细胞淋巴瘤治疗方案的成功治疗表明它在这种罕见的实体中具有潜在的有效性.
- 早期诊断和及时治疗对于儿科PCNSL的良好结果至关重要.
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