Successful Management of Severe Refractory ARDS in Hemophagocytic Lymphohistiocytosis with VV-ECMO: A Case Report and Analysis

静脉-静脉体外膜肺氧合(VV-ECMO)成功治疗噬血细胞性淋巴组织细胞增生症合并重度难治性急性呼吸窘迫综合征:病例报告及分析

阅读:3

Abstract

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.

特别声明

1、本页面内容包含部分的内容是基于公开信息的合理引用;引用内容仅为补充信息,不代表本站立场。

2、若认为本页面引用内容涉及侵权,请及时与本站联系,我们将第一时间处理。

3、其他媒体/个人如需使用本页面原创内容,需注明“来源:[生知库]”并获得授权;使用引用内容的,需自行联系原作者获得许可。

4、投稿及合作请联系:info@biocloudy.com。