| Objective: To investigate the clinical features,treatment strategies and prognostic factors of extranodal NK/T cell lymphoma,nasal type-associated hemophagocytic syndrome(NK/T-LAHS).Methods: The clinical data of 16 NK/T-LAHS admitted to the First Affiliated Hospital of Guangxi Medical University from June 2012 to June 2019 were collected.Retrospective analysis of its clinical characteristics,therapeutic efficacy and prognosis.Results:1.16 cases of NK/T-LAHS with a male to female ratio of 3:1.The age range is19-62,with a median age of 30.The primary lesion was in the upper respiratory tract in 13 patients.87.5% of patients with Ann Arbor stage III/IV,B Group.Most of patients were in the high/medium-risk group(81.2%)with IPI/aa IPI scores.16 patients had positive EBER expression in tumor tissues.93.7% of patients showed increased LDH levels and the invasion area outside the tumor was ≥2.2.The most common clinical manifestations were fever(100%)and splenomegaly(68.75%),and the most common laboratory abnormalities were elevated serum ferritin(100%),elevated transaminase(93.75%),pancytopenia(75%),hyponatremia(68.75%),hemophagocytosis(57.1%),and decreased NK cell activity(53.3%),followed by hypofibrinogenemia(50%),elevated s CD25(50%),elevated bilirubin(50%),prolonged APTT(43.75%),hypertriglyceridemia(26.7%)and prolonged PT(18.75%).Patients with HLH onset at ENKTL diagnosis were often older(43.30 vs 27.83,P = 0.011)and had a higher IPI / aa IPI score(100% vs 50%,P = 0.036)than that at ENKTL progress/relapse.The incidence of hypoproteinemia in patients with HLH at ENKTL progress/relapse was significantly higher than that onset at ENKTL diagnosis(100% vs 20%,P = 0.007).3.Among the 16 NK/T-LAHS patients,10(62.5%)had combined HLH at the initial diagnosis,and 6(37.5%)were at ENKTL progress/relapse.The median interval between ENKTL diagnosis and combined HLH in 16 patients in this study was 13.5 days,with an average interval of 5 months.The median interval between ENKTL diagnosis and HLH combination was 9.3 months in 6 patients with HLH during ENKTL progression/recurrence.4.Among the 16 patients with NK/T-LAHS,2 patients abandoned treatment,2patients were treated with hormone therapy alone,2 patients were treated with HLH-94 regimen,and the remaining 10 patients were treated mainly for lymphoma;4 patients were treated with SMILE regimen;5 patients were treated with P-GEMOX regimen;1 patient underwent ASCT after CR was achieved with SMILE chemotherapy.The maximum follow-up time of the 16 patients was 85.6 months.4 of the patients were lost to follow-up,and only 2 of the 12 patients who could be followed survived.The median OS of ENKTL was 11.2months,the 1-year survival rate was 48.2%,and the 2-year survival rate was28.9%.The median OS after NK/ T-LAHS diagnosis was 2.8 months,the 1-year survival rate was 34.8%,and the 2-year survival rate was 23.2%.The time of occurrence of HLH in ENKTL patients had no effect on the overall prognosis(P= 0.565).Conclusion:1.NK/T-LAHS mostly occurred in middle-aged men,mostly in advanced stage,group B,high/medium risk group of IPI when diagnosed.2.The clinical manifestations of NK/T-LAHS showed no specificity,and half of the patients developed HLH within 2 weeks after the initial diagnosis of ENKTL.Patients in the elderly and high/medium risk groups were more likely to develop HLH at the time of initial diagnosis of ENKTL.3.NK/T-LAHS progresses rapidly,patients respond poorly to treatment and have poor prognosis. |
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