[Guidelines for the diagnosis and treatment of hemophagocytic lymphohistiocytosis (2026 edition)].

Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory response syndrome caused by inherited or acquired dysregulation of immune function. In recent years, with the continuous advancement of basic and clinical research on HLH, novel diagnostic and therapeutic concepts as well as new therapeutic agents have emerged, and international clinical practice guidelines have been updated successively. To promote standardized management of the disease, academic organizations including the Lymphocyte Group of Hematology Branch of the Chinese Medical Association, Professional Committee of Oncological Hematology of the China Anti-Cancer Association, Hematology Group of the Pediatrics Branch of the Chinese Medical Association, Infectious Diseases Branch of the Chinese Medical Association, and National Clinical Research Center for Rheumatic and Autoimmune Diseases jointly initiated the guideline revision. A panel of 80 domestic experts from hematology, pediatrics, infectious diseases, and rheumatology and immunology disciplines was assembled to revise the 2022 edition of Chinese Guidelines for the Diagnosis and Treatment of HLH, on the basis of the latest domestic and international research advances and current evidence-based medical data. The guidelines provides 16 recommendations covering 11 aspects including HLH classification, diagnostic criteria, laboratory testing, therapeutic strategies and efficacy evaluation, aiming to improve the diagnosis and treatment of HLH in China and optimize patients' clinical outcomes.