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A clinical study of 21 patients with hemophagocytic syndrome in 295 cases diagnosed with nasal type, extranodal nature killer/T cell lymphoma.
- Source :
-
Cancer biology & therapy [Cancer Biol Ther] 2017 Apr 03; Vol. 18 (4), pp. 252-256. Date of Electronic Publication: 2017 Feb 25. - Publication Year :
- 2017
-
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).
- Subjects :
- Adolescent
Adult
Aged
Blood Cell Count
China epidemiology
Female
Follow-Up Studies
Humans
Incidence
Kaplan-Meier Estimate
L-Lactate Dehydrogenase blood
Lymphohistiocytosis, Hemophagocytic blood
Lymphohistiocytosis, Hemophagocytic drug therapy
Lymphoma, Extranodal NK-T-Cell blood
Lymphoma, Extranodal NK-T-Cell drug therapy
Male
Middle Aged
Prognosis
Rare Diseases blood
Rare Diseases epidemiology
Rare Diseases etiology
Retrospective Studies
Risk Factors
Young Adult
Antineoplastic Combined Chemotherapy Protocols therapeutic use
Lymphohistiocytosis, Hemophagocytic epidemiology
Lymphohistiocytosis, Hemophagocytic etiology
Lymphoma, Extranodal NK-T-Cell complications
Lymphoma, Extranodal NK-T-Cell epidemiology
Subjects
Details
- Language :
- English
- ISSN :
- 1555-8576
- Volume :
- 18
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Cancer biology & therapy
- Publication Type :
- Academic Journal
- Accession number :
- 28278074
- Full Text :
- https://doi.org/10.1080/15384047.2017.1295176