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已发表论文
自然杀伤/T细胞淋巴瘤相关噬血细胞性淋巴组织细胞增多症：一种罕见且危险的疾病
Authors Meng G , Wang M , Sun C, Feng G, Ren J, Feng S, Wang Y
Received 15 October 2025
Accepted for publication 19 January 2026
Published 27 January 2026 Volume 2026:16 574445
DOI https://doi.org/10.2147/BLCTT.S574445
Checked for plagiarism Yes
Review by Single anonymous peer review
Peer reviewer comments 2
Editor who approved publication: Dr Wilson Gonsalves
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 & Shandong Provincial Qianfoshan Hospital, Jinan, Shandong, People’s Republic of China; 2Department of Hematology, Qilu Hospital of Shandong University, Jinan, Shandong, People’s Republic of China
Correspondence: Guangqiang Meng, Email menggq2001@163.com
Abstract: 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 7.1– 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
1Department of Hematology, The First Affiliated Hospital of Shandong First Medical University & Shandong Provincial Qianfoshan Hospital, Jinan, Shandong, People’s Republic of China; 2Department of Hematology, Qilu Hospital of Shandong University, Jinan, Shandong, People’s Republic of China
Correspondence: Guangqiang Meng, Email menggq2001@163.com
Abstract: 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 7.1– 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