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Research Article: Patients with Hemophagocytic Lymphohistiocytosis Who Need Intensive Care Can Be Successfully Rescued by Timely Using Etoposide-Based HLH Regimens

Date Published: 2024-02-03

Abstract:
As a cytokine release syndrome caused by uncontrolled immune activation, hemophagocytic lymphohistiocytosis (HLH) can quickly lead to organ infiltration, systemic disease, and poor prognosis. Genetic defects in primary HLH affect cytotoxic activity, which has been widely studied in pediatric patients. Secondary HLH is often caused by infection, malignancy, and autoimmune diseases and can be seen in patients of all age groups. Adult HLH patients lack comprehensive data, and treatment measures depend on expert opinions and the HLH-/ protocol verified by pediatric patients., Based on the rapid and dangerous disease process of HLH, some patients have multiple organ involvement at diagnosis, including respiratory failure, coma, shock, acute liver and kidney failure, and so on. Mortality in intensive care units (ICU) is –%., Therefore, in addition to standard organ support, thorough etiological screening and emergency treatment of HLH triggers are necessary for HLH treatment. “Specific” HLH therapies (corticosteroids, multivalent immunoglobulins, etoposide, anti-cytokine therapy, and so on) are usually used for the most severe cases of HLH in the ICU.,, The death-related risk factors are poorly understood among critically ill HLH patients who need intensive care. The efficacy of the etoposide-based regimens has also not been intensively identified and reported.

Introduction:
As a cytokine release syndrome caused by uncontrolled immune activation, hemophagocytic lymphohistiocytosis (HLH) can quickly lead to organ infiltration, systemic disease, and poor prognosis. Genetic defects in primary HLH affect cytotoxic activity, which has been widely studied in pediatric patients. Secondary HLH is often caused by infection, malignancy, and autoimmune diseases and can be seen in patients of all age groups. Adult HLH patients lack comprehensive data, and treatment measures depend on expert…

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