Analysis Of Clinical Characteristics And Prognostic Factors Of Extranodal NK/T Cell Lymphoma

Comparison of Nasal with Extranasal Patients with Extranodal Natural Killer/T-cell LymphomaPurpose: Extranodal natural killer(NK)/T-cell lymphoma,nasal type(ENKTL)is a clinically heterogeneous disease with different primary tumor sites.Compared with nasal type,patients with extranasal disease often present with rapid disease progression,adverse prognosis,and shorter survival.In order to better understand the differences between nasal and extranasal types,we investigated the clinical features of the patients with ENKTL at our institution in the southeast area of China.Patients and Methods: We analyzed 98 patients with untreated ENKTL,including80(81.6%)patients with primary upper aerodigestive tract diseases designated as nasal type,while 18(18.4%)patients with primary tumor sites presented in skin(n=7,38.8%),gastrointestinal tract(n=4,22.2%),lung(n=3,16.7%),lymph node(n=3,16.7%)and penis(n=1,5.6%),were designated as extranasal type.Results: Patients had different patterns of clinical features and survival outcomes.Of the 80 nasal cases who were assessed for treatment response,half patients achieved complete reponse(CR)or unconfirmed complete response(CRu)and 25(31.3%)achieved partial response(PR),while only two patients achieved CR/CRu in extranasal cohort(P=0.003).Extranasal patients had poor response to pegaspargase-based chemotherapy.With the median follow-up time of 17 months,33(33.7%)patients died.In nasal cohort,the 1-,3-,and 5-year overall survival(OS)rates were 86.3%,77.0%,and 67.0%,respectively.Whereas extranasal cases had worse survival,with the 1-year OS rate of only 37%.The primary extranasal tumor presentation,Ann Arbor stage ?/?,poor performance status,B symptoms,regional lymphadenopathy,lower albumin,elevated C-reactive protein(CRP),lymphocytopenia,monocytopenia,anemia,decreasing absolute counts of T cell subsets,inverse ratio of CD4/CD8,high and intermediate-high risk international prognostic index(IPI)and Korean prognostic index(KPI)were related with worse OS(P<0.05).In multivariate analysis,the results showed that the independent prognostic markers for worse OS were primary extranasal tumor presentation,anemia and elevated CRP.Conclusion: ENKTL patients with primary extranasal tumor infiltration showed more adverse clinical outcomes and aggressive biologic behavior.Even though pegaspargase-based treatments were given,the extranasal patients were still associated with inferior survival outcomes compared with those in nasal cases.The strategy should be optimized for extranasal cases.Circulating Low Absolute CD4~+ T Cell Counts May Predict Poor Prognosis in Extranodal NK/T-Cell Lymphoma Patients Treating with Pegaspargase-Based ChemotherapyPurpose: Extranodal natural killer/T cell lymphoma,nasal type(ENKTL)is a rare subtype of non-Hodgkin lymphoma,and asparaginase-based regimens are the best first-line treatments.Data on the role of specific circulating lymphocyte subsets in the progression of ENKTL are limited.The aim of this study was to investigate the clinical correlation and distribution of circulating absolute CD4~+ T-cell counts(ACD4C)in ENKTL.Patients and Methods: We retrospectively searched medical records for 70 newly diagnosed ENKTL patients treated with pegaspargase-based regimens.Comparison of ACD4C as a continuous parameter in different groups was calculated.Univariate and multivariate analyses were used to assess prognostic factors for overall survival(OS)and progression-free survival(PFS).Results: Ann Arbor stage ?/?,B symptoms,elevated lactate dehydrogenase,monocytopenia,high-intermediate and high risk International Prognostic Index(IPI)and Korean Prognostic Index(KPI),high risk Prognostic Index of Natural Killer Lymphoma(PINK),and lower lymphocytes were significantly associated with low ACD4C at diagnosis.With a median follow-up time of 32 months,patients who had an ACD4C <0.30×10~9/L had a worse OS.Median OS was 11 months and median PFS was 5 months in the low ACD4C cohort.There were significant differences in both OS and PFS between the two cohorts.Moreover,multivariate Cox analysis identified ACD4C as an independent predictor for OS and PFS.Conclusion: Low ACD4C was associated with poorer survival and could act as a negative predictor for ENKTL patients treated with asparaginase-based regimens.