title: A clinical study of 21 patients with hemophagocytic syndrome in 295 cases diagnosed with nasal type, extranodal nature killer/T cell lymphoma.
abstract: Nasal type, extranodal nature killer (NK)/T cell lymphoma-associated hemophagocytic syndrome (NK/T-LAHS) is a rare and fatal disorder with extremely poor prognosis. To investigate its clinical characteristics and risk factors, we retrospectively analyzed 295 patients with nasal type, extranodal nature killer/T cell lymphoma, of which 21 were diagnosed with hemophagocytic syndrome, with a cumulative incidence of 7.1%. The most frequently clinical characteristics were fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, hyperferritinemia, liver dysfunction, hypertriglyceridemia, hypofibrinogenemia and evaluated lactate dehydrogenase (LDH) level. After a median follow-up of 27 months, the 2-year survival for the 295 patients was 74.6%. Significant difference for 2-year survival was found between patients with and without hemophagocytic syndrome (4.8% vs. 80.0%, P<0.001). After developing hemophagocytic syndrome, all patients survived no more than 3 months, with a median survival of 35 days. Risk factors for NK/T-LAHS were bone marrow (BM) involvement (P = 0.019; relative risk, 13.649; 95% confidence interval (CI): 1.538-121.103), hepatosplenomegaly (P = 0.003; relative risk, 9.616; 95%CI: 2.154-42.918), and elevated LDH level (>314U/L) (P = 0.038; relative risk, 6.293; 95%CI: 1.108-35.735). We conducted a risk model for all 295 patients based on the 3 adverse factors as follows: low risk (233 cases, 79.0%), no factor; intermediate risk (43 cases, 14.6%), one factor; high risk (19 cases, 6.4%), 2 or 3 factors. The probabilities for developing LAHS were 0.9% for low-, 14.0% for intermediate-, and 68.4% for high-risk group. Significant differences in the 3 risk groups were observed (P<0.001).
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Na
authors.initials: N
authors.lastname: Li
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Li
authors.initials: L
authors.lastname: Zhang
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Jie
authors.initials: J
authors.lastname: Liu
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Jing
authors.initials: J
authors.lastname: Zhang
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Hua-Wei
authors.initials: HW
authors.lastname: Weng
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Hong-Yu
authors.initials: HY
authors.lastname: Zhuo
authors.affiliation: a Department of Medical Oncology of Cancer Center, State Key Laboratory of Biotherapy , West China Hospital, Sichuan University , Chengdu , China.
authors.forename: Li-Qun
authors.initials: LQ
authors.lastname: Zou
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001772:Blood Cell Count; D002681:China / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D053208:Kaplan-Meier Estimate; D007770:L-Lactate Dehydrogenase / Q000097:blood; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology*; D054391:Lymphoma, Extranodal NK-T-Cell / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D035583:Rare Diseases / Q000097:blood / Q000453:epidemiology / Q000209:etiology; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
chemical_list: D007770:L-Lactate Dehydrogenase
keywords: Clinical characteristics; lymphoma-associated hemophagocytic syndrome; nature killer/T cell; prognosis; risk factor; survival; treatment