| Summary: | Yi-Zhen Liu,1,2,* Lan-Qing Bi,3,* Gui-Lin Chang,4 Ye Guo,5 Si Sun1,2 1Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China; 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China; 3Department of Medical Oncology, Suzhou Municipal Hospital, The Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215000, China; 4Department of Geratology, Fudan University Shanghai Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai 200032, China; 5Department of Medical Oncology, Shanghai East Hospital, Tongji University School of Medicine, Shanghai 200120, China *These authors contributed equally to this work Background: Extranodal natural killer (NK) / T cell lymphoma is a subtype of non-Hodgkin’s lymphoma (NHL) that usually has an aggressive clinical course. It is the predominant trigger of lymphoma-associated hemophagocytic syndrome (LAHS), which is highly lethal and with extremely poor prognosis. This study is aiming to characterize the associated clinical features and prognostic factors of the disease. Patients and methods: Twenty-eight patients with extranodal NK/T cell lymphoma associated hemophagocytic lymphohistiocytosis (HLH) were retrospectively analyzed. The clinical records were collected, and the associations between clinical or laboratory parameters and overall survival (OS) were assessed. Results: The most frequently clinical characteristics were fever (96.4%), and splenomegaly (81.5%). Concerning the laboratory findings, the most common features were hyperferritinemia (91.7%), grade III/IV thrombocytopenia (64.3%), hypertriglyceridemia (48%), severe anemia (46.4%), hypofibrinogenemia (45%), and grade III/IV neutropenia (32.1%). The interval between the diagnosis of NK/T LAHS and death / last contact was between 4 to 701 days with the median interval of 15 days. We found that higher serum lactic dehydrogenase (LDH) at HLH, hypofibrinogenemia, and splenomegaly were significantly associated with worse survival (P=0.002, 0.003, 0.003). Furthermore, Eastern Cooperative Oncology Group (ECOG) score, extra-upper aerodigestive tract NK/T cell lymphoma (EUNKTL) and cutaneous involvement were risk factors of HLH. Conclusion: Our data indicated that levels of LDH, fibrinogen, and presence of splenomegaly were prognostic factors of the disease. Higher ECOG scores, EUNKTL and cutaneous involvement were risk factors of NK/T LAHS. Additional independent, prospective clinical trials will be needed to explore optimal treatment. Keywords: NK/T-cell lymphoma, hemophagocytic lymphohistiocytosis, risk factor, prognosis
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