Hemophagocytic Lymphohistiocytosis: an updated overview and management approach in the critical care setting

Abstract

Hemophagocytic lymphohistiocytosis (HLH) constitutes a rare and potentially life-threatening immunological syndrome. It manifests in both primary (genetic) and secondary (acquired) forms, exhibiting a diverse range of clinical and laboratory features, thereby posing challenges to accurate diagnosis, particularly within critical care settings. Early identification and intervention are of vital importance for improving patient survival. This article comprehensively explores the existing literature, encompassing the classification, pathophysiology, and clinical presentation of HLH. Special emphasis is placed on identifying prognostic factors, organ failure, and associated complications. Moreover, a detailed narrative of proposed management strategies, including immuno- and myelosuppressive options, is presented. Further research directions are suggested to enhance the understanding and treatment of this complex disorder.