初级节性淋巴细胞主导霍奇金淋巴瘤 (NLPHL):在幼儿中首次报告的病例
Josselyn Sofia Vergara1, Lianna Jean Marks2, Oscar Silva1
1Department of Pathology, Stanford University School of Medicine, Stanford, CA, USA.
概括
在儿童中,结节性淋巴细胞主导的霍奇金淋巴瘤 (NLPHL) 很少主要出现在脏中. 这份病例报告详细介绍了一名患有初级脏NLPHL的儿童,在脏切除术后3年,他没有接受进一步治疗.
科学领域:
- 血液学
- 儿童瘤学
- 病理学
背景情况:
- 结节性淋巴细胞占主导地位的霍奇金淋巴瘤 (NLPHL) 是一种罕见的B细胞淋巴瘤,通常不发作,影响淋巴结.
- 在NLPHL中脏的参与往往表明疾病已晚期,预后较差.
- 主要的脏NLPHL,特别是在没有节点参与的儿科病例中,异常罕见.
研究的目的:
- 在儿童中报告第一个已知的初级脏结节性淋巴细胞主导的霍奇金淋巴瘤.
- 突出了脊髓切除术后小儿原发性脊髓NLPHL的潜在好处.
主要方法:
- 病例报告详细介绍了一名被诊断患有原发性脊髓NLPHL的儿童患者.
- 对临床表现,诊断结果,治疗 (脊髓切除术) 和随访情况的审查.
- 脊髓组织中NLPH的病理确认.
主要成果:
- 患者出现了孤立的脏干扰,缺乏明显的节点疾病.
- 在脊髓切除术后,儿童在3年的随访中保持无疾病状态,不需要额外的治疗.
- 这一结果与NLPHL中脏参与相关的普遍较差的预后形成鲜明对比.
结论:
- 虽然很少见,但儿童的原发性脏NLPHL的预后可能很好.
- 单独的脊髓切除术可能是儿童初级脊髓NLPH的足够治疗方法.
- 这一案例扩大了对儿科患者的NLPHL表现和治疗的理解.
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