外节自然杀手/与外围T细胞淋巴瘤共存的T细胞淋巴瘤,未另有说明
Kenta Hayashino1,2, Chikamasa Yoshida1, Yoshiyuki Ayata3
1Department of Hematology, Okayama City Hospital, Okayama-shi, Okayama, Japan.
Journal of clinical and experimental hematopathology : JCEH
|March 27, 2024
概括
一位最初被诊断为患有外围T细胞淋巴瘤 (PTCL-NOS) 的患者后来患上了外节自然杀手/T细胞淋巴瘤 (ENKTL). 这一案例凸显了爱斯坦-巴尔病毒 (EBV) 活性化如何复杂化T细胞淋巴瘤,这表明EBV-DNA监测至关重要.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 传染性疾病 传染性疾病
背景情况:
- 周围T细胞淋巴瘤,未另有说明 (PTCL-NOS) 是一个异质的群体的侵略性非霍奇金淋巴瘤.
- 外节自然杀手/T细胞淋巴瘤 (ENKTL) 是一种与爱斯坦-巴尔病毒 (EBV) 相关的独特亚型.
- 不同淋巴瘤亚型的同时发生或连续发展可能会发生,特别是在免疫受损状态下.
研究的目的:
- 报告一个罕见的PTCL-NOS和EBV相关ENKTL在单个患者的连续发展的罕见病例.
- 调查EBV再激活在PTCL-NOS之后的ENKTL病变发生中的潜在作用.
- 强调EBV-DNA监测在处理如此复杂的病例中的诊断效用.
主要方法:
- 基于淋巴结活检和免疫类型 (CD3+,CD5+,CD56-,EBER-) 的PTCL-NOS的初始诊断.
- 患者接受了化疗,并发生了败血症,并接受了抗微生物药物和普得尼松治疗.
- 随后的ENKTL诊断通过检测CD3 +,CD56 +,EBER +异型淋巴细胞在炎和骨髓中得到证实,其EBV-DNA水平升高.
主要成果:
- 这位患者最初被诊断为PTCL-NOS,随后患上骨髓参与的ENKTL.
- 第二次诊断中的非典型淋巴细胞对CD3,CD56和爱斯坦-巴尔病毒 (EBV) 编码的小RNA (EBER) 呈阳性.
- 在全血和单克隆EBV模式中观察到EBV-DNA水平升高,这表明EBV重新激活.
结论:
- 化疗,败血症和普德尼松治疗PTCL-NOS可能会诱导免疫缺陷,促进EBV的重新激活和ENKTL的发展.
- 这一案例强调了在免疫受损患者中考虑EBV相关的淋巴增殖性疾病的重要性.
- 监测血液中的EBV-DNA是早期检测EBV相关淋巴瘤并发症的宝贵工具.
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