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表现为乳糜胸和谵妄的成熟 NK 细胞淋巴瘤:一例罕见急症病例

英文原题:Mature NK-cell lymphoma presenting with chylothorax and delirium: a rare emergency case.

查看英文原题

Mature NK-cell lymphoma presenting with chylothorax and delirium: a rare emergency case.

PubMed 2025/12/29(内容时间) Int J Emerg Med Q2 · IF 1.9(JCR 2025)

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研究概要

该病例表明,成熟 NK 细胞淋巴瘤在急诊情况下可能表现为此前未报道的乳糜胸合并急性谵妄。

中文摘要

结外NK/T细胞淋巴瘤占非霍奇金淋巴瘤不足1%,中枢神经系统受累和乳糜胸均属极罕见表现;这种表现组合此前未见急诊医学文献报道。病例:一名既往健康的51岁中国男性因进行性呼吸困难、高热和双侧胸腔积液就诊,症状持续8天。体格检查发现广泛瘀斑、双肺实变和脾肿大。实验室检查显示全血细胞减少(白细胞3.26×10⁹/L、血小板58×10⁹/L)、严重凝血障碍及乳酸脱氢酶升高(773 U/L)。胸腔穿刺证实乳糜胸,甘油三酯为1.46 mmol/L,苏丹III染色阳性。住院第10天,患者出现急性躁动型谵妄,伴视觉幻觉和偏执行为。流式细胞术显示外周血(68.09%)、骨髓(13.75%)及胸腔积液(87.34%)中均有异常NK细胞扩增,确诊为多部位受累的成熟NK细胞淋巴瘤。PET-CT显示脾受累(SUVmax 9.0)及左枕叶低密度病灶。尽管给予氧疗、经验性抗生素和镇静等支持治疗,患者病情仍迅速进展为噬血细胞性淋巴组织细胞增多症和多器官功能衰竭,发病后4周内死亡。

该病例显示,成熟NK细胞淋巴瘤在急诊中可表现为此前未报道的乳糜胸与急性谵妄组合。遇到原因不明的胸腔积液、神经精神症状和血细胞减少时,急诊医生应警惕血液系统恶性肿瘤;尽管此类疾病通常进展迅猛,早期识别并及时请血液科会诊仍可能影响临床结局。

展开英文摘要原文

Extranodal natural killer (NK)/T-cell lymphomas constitute less than 1% of non-Hodgkin lymphomas, with central nervous system involvement and chylothorax representing exceptionally rare presentations. This unique combination has not been previously reported in emergency medicine literature. CASE PRESENTATION: A previously healthy 51-year-old Chinese male presented with progressive dyspnea, high fever, and bilateral pleural effusion over eight days. Physical examination revealed extensive ecchymoses, bilateral lung consolidation, and splenomegaly. Laboratory studies demonstrated pancytopenia (WBC 3.26 10 /L, platelets 58 10 /L), severe coagulopathy, and elevated lactate dehydrogenase (773 U/L). Thoracentesis confirmed chylothorax with triglyceride levels of 1.46 mmol/L and positive Sudan III staining. On hospital day 10, the patient developed acute hyperactive delirium with visual hallucinations and paranoid behavior. Flow cytometry revealed abnormal NK-cell expansion across peripheral blood (68.09%), bone marrow (13.75%), and pleural fluid (87.34%), establishing the diagnosis of mature NK-cell lymphoma with multi-site involvement. PET-CT imaging demonstrated splenic involvement (SUVmax 9.0) and a hypodense lesion in the left occipital lobe. Despite supportive care including oxygen therapy, empirical antibiotics, and sedation, the patient rapidly progressed to hemophagocytic lymphohistiocytosis and multi-organ failure, resulting in death within four weeks of presentation.

This case demonstrates that mature NK-cell lymphoma may present with the previously unreported combination of chylothorax and acute delirium in emergency settings. Emergency physicians should maintain heightened awareness of hematologic malignancies when encountering patients with unexplained pleural effusion, neuropsychiatric symptoms, and cytopenias, as early recognition and prompt hematology consultation may influence clinical outcomes despite the typically aggressive disease course.

论文信息

作者
Shang W、Guo Z、Ma Q、Wang J、Xu Y
第一作者单位
Department of Emergency, Peking University Third Hospital, 49 North Garden Rd.,Haidian District, Beijing, 100191, P. R. China.China
通讯作者单位
Department of Hematology, Peking University Third Hospital, 49 North Garden Rd.,Haidian District, Beijing, 100191, P. R. China. wangjj368@163.com.China
期刊
International journal of emergency medicine2025 Dec 29
原文标识
PubMed 41462436 · DOI 10.1186/s12245-025-01076-y