20 June 2025
: Case report
[In Press] Successful Management of Severe Refractory ARDS in Hemophagocytic Lymphohistiocytosis with VV-ECMO: A Case Report and Analysis
Challenging differential diagnosis, Management of emergency care, Rare disease
Mengqi Guan12BCDE, Yan Qian3C, Zihua Tang12F, Yingya Cao124C, Xiaogan Jiang12ADG, Weihua Lu12ACG, Qiancheng Xu124ADOI: 10.12659/AJCR.949154
Am J Case Rep In Press; DOI: 10.12659/AJCR.949154
Available online: 2025-06-20, In Press, Corrected Proof
Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule
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.
Keywords: Atherosclerosis; Case Reports; Glucose Intolerance; Immunotherapy; Pulmonary Edema
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