VV-ECMO成功治疗吞噬淋巴组织细胞增多症重症难治性ARDS 1例报告与分析。

IF 0.7 Q3 MEDICINE, GENERAL & INTERNAL
Mengqi Guan, Yan Qian, Zihua Tang, Yingya Cao, Xiaogan Jiang, Weihua Lu, Qiancheng Xu
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引用次数: 0

摘要

噬血细胞性淋巴组织细胞增多症(HLH)是一种罕见的、危及生命的全身性炎症性疾病,以细胞因子风暴、凝血异常和全血细胞减少为特征,可迅速发展为多器官功能衰竭。虽然急性呼吸窘迫综合征(ARDS)是HLH不常见但严重的并发症,但静脉-静脉体外膜氧合(VV-ECMO)可以作为对标准治疗无反应的病例的救命干预措施。新出现的病例报告表明,在适当的情况下,VV-ECMO可以提供实质性的临床益处。病例报告一名45岁女性,表现为1周高热、疲劳、厌食和进行性呼吸困难。初步检查显示血小板减少(血小板30×10 /L), c反应蛋白升高,胸部计算机断层扫描显示双侧磨玻璃影。尽管有肺保护设置,她的PaO₂/FiO₂比率仍低于80 mmHg。在重症监护室的第2天开始VV-ECMO,迅速恢复了SpO₂并减少了血管加压药的需求。进一步评估符合HLH-2004标准:高铁蛋白血症、高可溶性CD25、脾肿大、骨髓噬血细胞增多和EBV DNA升高。在ECMO支持下,患者在第18天接受大剂量甲基强的松龙(1 g/day×5天)、强的松逐渐减少和依托泊苷治疗。患者于第8天脱离ECMO,第20天拔管,第45天出院,实验室指标正常。在4年的随访中,她的病情完全缓解。结论:通过提供靶向免疫抑制和化疗的窗口,早期VV-ECMO可以挽救成年hlh相关ARDS患者的生命。快速识别HLH、多学科管理和及时启动ECMO至关重要。进一步的研究应该完善患者的选择、时机和综合治疗方案。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Successful Management of Severe Refractory ARDS in Hemophagocytic Lymphohistiocytosis with VV-ECMO: A Case Report and Analysis.

BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening systemic inflammatory disorder characterized by cytokine storm, coagulation abnormalities, and pancytopenia, which can rapidly progress to multi-organ failure. Although acute respiratory distress syndrome (ARDS) is a less common but severe complication of HLH, veno-venous extracorporeal membrane oxygenation (VV-ECMO) can serve as a lifesaving intervention in cases unresponsive to standard treatments. Emerging case reports indicate that, when appropriately indicated, VV-ECMO can offer substantial clinical benefits. CASE REPORT A 45-year-old woman presented with 1 week of high-grade fever, fatigue, anorexia, and progressive dyspnea. Initial workup showed thrombocytopenia (platelets 30×10⁹/L), elevated C-reactive protein, and bilateral ground-glass opacities on chest computed tomography. Despite lung-protective settings, her PaO₂/FiO₂ ratio stayed below 80 mmHg. VV-ECMO was started on day 2 in the Intensive Care Unit, promptly restoring SpO₂ and reducing vasopressor needs. Further evaluation met HLH-2004 criteria: hyperferritinemia, high soluble CD25, splenomegaly, bone marrow hemophagocytosis, and elevated EBV DNA. Under ECMO support, she received high-dose methylprednisolone (1 g/day×5 days), a prednisone taper, and etoposide on day 18. She was weaned from ECMO on day 8, extubated on day 20, and discharged on day 45, with normalized laboratory values. At the 4-year follow-up, she remained in complete remission. CONCLUSIONS Early VV-ECMO can be life-saving in adult patients with HLH-associated ARDS by providing a window for targeted immunosuppression and chemotherapy. Rapid HLH recognition, multidisciplinary management, and timely ECMO initiation are essential. Further studies should refine patient selection, timing, and integrated treatment protocols.

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来源期刊
American Journal of Case Reports
American Journal of Case Reports Medicine-Medicine (all)
CiteScore
1.80
自引率
0.00%
发文量
599
期刊介绍: American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.
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