[Expert consensus on diagnosis and treatment of lymphoma-associated hemophagocytic lymphohistiocytosis (2025 edition)].

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by the undue activation of cytotoxic T lymphocytes, natural killer cells due to various triggers, and the stimulation of mononuclear phagocyte system, resulting in excessive secretion of inflammatory cytokines. Lymphoma is a major trigger for secondary HLH. In order to improve the understanding of lymphoma-associated HLH (LA-HLH) among clinicians, based on the "Chinese Expert Consensus on Diagnosis and Treatment of Lymphoma-associated Hemophagocytic Lymphohistiocytosis (2022 edition)" and current published evidence, the China Anti-Cancer Association Lymphoma Committee, the China Anti-Cancer Association Hematology Oncology Committee and the Chinese Expert Alliance for Hemophagocytic Lymphohistiocytosis organized 57 experts in related fields to revise the diagnosis and treatment of LA-HLH, reaching this expert consensus. A total of 14 recommendations were put forward from 8 aspects, including clinical classification, early diagnosis, stratified treatment, and efficacy evaluation. On this basis, the diagnosis and treatment procedures of LA-HLH in China were updated, aiming to improve the diagnosis and treatment level of LA-HLH and guide standardized treatment in China.