Guangqiang Meng,1 Manzhi Wang,1 Congcong Sun,1 Gege Feng,2 Jing Ren,1 Saran Feng,1 Yan Wang1 1Department of Hematology, The First Affiliated Hospital of Shandong First Medical University 2Department of Hematology, Qilu Hospital of Shandong University, Jinan, Shandong, Peopleâs Republic of ChinaCorrespondence: Guangqiang Meng, Email menggq2001@163.comAbstract: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome characterised by the reactive activation of cytotoxic T-lymphocytes and macrophages along with a substantial amount of cytokine secretion caused by various inductions. Natural killer/T-cell lymphoma (NKTL)-associated HLH (NK/T-LAHLH) is rare in clinical practice with an incidence rate of 7.1â 11.9% in NKTL patients. Currently, there is no standard first-line treatment with good efficacy for NK/T-LAHLH. The treatment of NK/T-LAHLH is still mainly based on chemotherapy regimens containing etoposide and dexamethasone. Recently, many new therapeutic drugs and schemes have been trialled for the treatment of NK/T-LAHLH, such as ruxolitinib, immune checkpoint inhibitors, pegaspargase, and the DEP regimen. However, NK/T-LAHLH is associated with overall poor prognosis. Improving overall understanding of NK/T-LAHLH is of great significance to ameliorating patient prognosis. This review systematically discussed the epidemiology, pathogenesis, clinical features, current treatment regimens, and prognosis of NK/T-LAHLH to comprehensively elucidate this disease.Keywords: NK/T-cell lymphoma, hemophagocytic lymphohistiocytosis, pathogenesis, treatment regimens, prognosis
Meng et al. (Tue,) studied this question.