Clinical, Pathological, and Prognostic Characteristics Of Mature Nodal Or Extranodal T-Cell and NK-Cell Non-Hodgkin´s Lymphoma (NHL) In a Single Mexican Institution

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1763-1763
Author(s):  
Gladys P Agreda-Vásquez ◽  
Erick Crespo-Solis ◽  
Gustavo J Ramos-Blas ◽  
Cesar Lara-Torres ◽  
Carmen Lome-Maldonado ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
T Cell ◽  
High Risk ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Univariate Analysis ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Number Of Patients ◽  
Who 2008

Abstract Background Mature nodal or extranodal T-cell and NK-cell NHL are a rare and heterogeneous group of NHL with aggressive behavior and poor clinical outcome. Their incidence varies according to geographical region and racial characteristics. Mexico is included in those countries known to have a high incidence of extranodal T/NK-cell lymphoma, type nasal (NKTCL). Objective To evaluate the outcome and prognosis of patients with mature nodal or extranodal T-cell or NK-cell NHL in a single institution in Mexico City. Methods Clinicopathological characteristic, treatment, outcome, and prognosis of patients admitted to our institution between August, 1991 and December 2009 were analyzed. Prognostic Index T-cell (PIT) was used in all subtypes of lymphomas except in NKTCL subtype. All tissue biopsies and immunophenotypic markers were reviewed by an expert hematopathologist and reclassified according to the WHO 2008 classification. Univariate analysis using log-rank test was used to determine the correlation between clinical features and overall survival (OS). Multivariate analysis using Cox proportional hazard models were performed. A p value < 0.05 was considered significant. Results A total of 67 patients were analyzed. Median age was 37 years. B symptoms were presented in 83.6%, 74.6% had at least one site with extranodal disease, 73.1% advanced clinic stage, 32.8% high risk by International Prognostic Index (IPI) and 47.5% high risk by PIT. According to WHO 2008 classification the most common subtype was peripheral T-cell lymphoma not otherwise (PTCL NOS) specified in 38 patients (56.7 %), angioimmunoblastic T-cell lymphoma (AITL) and NKTCL were the second most common subtypes with 8 cases in each group (11.9 %), anaplastic large cell lymphoma (ALCL) kinase-positive (ALK-positive) was identified in 3 patients (4.5 %), ALCL ALK-negative in 2 cases (3.0 %), lymphoblastic lymphoma and subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with 3 patients in each group (4.5 %), hepatosplenic T-cell lymphoma (HSTL) and aggressive NK-cell leukemia with one case in each group (1.5 %). CHOP-like therapy was used in 71.6 % of patients. Nine percent of patients did not receive treatment. The response was evaluated in 53 patients in whom overall response was 71.7 % with 44.8 % achieving complete remission (CR). Median OS was 2760 days (CI 95 % 1153.145-4366.855). Histopathology subtype did not predict OS. Both prognostic scores, IPI and PIT, were able to identify 4 groups of patients with different outcomes. The analysis failed to demonstrate any advantage of adding etoposide to the chemotherapy schedule. Multivariate analysis showed that, IPI, PIT, and CR were predictive for OS (Table 1). Conclusion Previous publications in Mexican population, with larger number of patients included, were particularly focus on clinical characteristics and prognosis of NKTCL. Our series provides data of mature nodal or extranodal T-cell and NK-cell NHL in Mexico. The current study confirms the poor prognosis of aggressive forms of mature nodal or extranodal T-cell and NK-cell NHL regardless of the chemotherapy schedule employed. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Baseline and interim functional imaging with PET effectively risk stratifies patients with peripheral T-cell lymphoma

2019 ◽  
Vol 3 (2) ◽  
pp. 187-197 ◽  
Author(s):  
Neha Mehta-Shah ◽  
Kimiteru Ito ◽  
Kurt Bantilan ◽  
Alison J. Moskowitz ◽  
Craig Sauter ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
T Cell ◽  
Confidence Interval ◽  
Cell Lymphoma ◽  
Univariate Analysis ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Standardized Uptake Value ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma

Abstract The prognosis of peripheral T-cell lymphoma (PTCL) is heterogenous. Baseline or interim imaging characteristics may inform risk-adapted treatment paradigms. We identified 112 patients with PTCL who were consecutively treated with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)/CHOP-like regimens with the intent to consolidate with an autologous transplant. Baseline (n = 93) and interim (after 4 cycles, n = 99) positron emission tomography (PET) images were reevaluated, and we calculated baseline total metabolic tumor volume (TMTV). Interim PET (iPET) responses were graded visually by 5-point score (i5PS) and by percentage change of standardized uptake value. By univariate analysis, predictors of event-free survival (EFS) included Prognostic Index for Peripheral TCL (PIT) higher than 1 (hazard ratio [HR], 1.83; P = .021), International Prognostic Index (IPI) higher than 3 (HR, 2.01; P = .021), high TMTV (&gt;125 cm3; HR, 3.92; P = .003), and positive iPET (HR, 3.57; P &lt; .001). By multivariate analysis, high baseline TMTV predicted worse overall survival (OS; HR, 6.025; P = .022) and EFS (HR, 3.861; P = .005). Patients with i5PS of 1 to 3 had a longer median OS and EFS (104 months, 64 months) than those with i5PS of 4 to 5 (19 months, 11 months; P &lt; .001). Four-year OS and EFS for patients with i5PS of 1 to 3 and PIT of 1 or less were 85% and 62%, respectively. However, 4-year OS and EFS for those with i5PS of 4 to 5 and PIT higher than 1 were both 0% (P &lt; .001). In multivariate analysis, after controlling for IPI and PIT, i5PS was independently prognostic for EFS (HR, 3.400 95% confidence interval, 1.750-6.750; P &lt; .001) and OS (HR, 10.243; 95% confidence interval, 4.052-25.891; P &lt; .001). In conjunction with clinical parameters, iPET helps risk stratify patients with PTCL and could inform risk-adapted treatment strategies. Prospective studies are needed to confirm these findings.

scholarly journals Prognostic Value of NCCN-IPI and Interim PET/CT Based on Visual Assessment in the Treatment of Peripheral T Cell Lymphomas

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 1822-1822
Author(s):  
Seo-Yeon Ahn ◽  
Ho-Young Yhim ◽  
Young Rok Do ◽  
Sung-Hoon Jung ◽  
Jae-Sook Ahn ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
High Risk ◽  
Cell Lymphoma ◽  
Prognostic Index ◽  
Visual Assessment ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Interim Pet ◽  
Pet Ct

Abstract Background It has been well known that peripheral T cell lymphoma (PTCL) has undergone poor prognosis compared with other non-Hodgkin lymphomas (NHL). Although the National Comprehensive Cancer Network-International Prognostic Index (NCCN-IPI) has been proposed to determining prognosis for patients with diffuse large B-cell lymphoma (DLBCL) at 2014, there is no study examines whether NCCN-IPI could apply to the T-cell NHLs. In addition, a few studies suggest prognostic utility of interim PET/CT in PTCL, but the role of interim PET/CT is not clear. Purpose We evaluate the predictive efficacy of the NCCN-IPI and interim PET/CT based on visual assessment in patients with newly diagnosed PTCLs. Methods This study included 153 patients with de novo peripheral PTCLs, diagnosed from January 2010 to August 2015. The NCCN-IPI was calculated as following the original references. Survival outcomes were compared with a matched result of IPI and/or Prognostic Index for peripheral T cell lymphoma, unspecified (PIT). Visual assessment of interim PET/CT based on Deauville five point scales was performed at the time of diagnosis, mid-treatment and completion of CHOP/CHOP-like or other non-anthracycline chemotherapy. Results The subtypes of PTCLs included PTCL, not otherwise specified (PTCL-NOS) (26%), angioimmunoblastic T cell lymphoma (20%), anaplastic large cell lymphoma (13%), extranodal NK/T cell lymphoma, nasal type (35%), and the others (6%). The NCCN-IPI showed better risk-based prognostic discrimination than IPI and PIT, especially between high-intermediate and high risk subgroups (3-year overall survival 40% vs. 27% vs. 26% among the high-intermediate risk group, respectively; 3-year overall survival 15% vs. 33% vs. 32% among the high risk group, respectively) with a median follow-up of 25.1 months (Figure 1). The absolute difference of survival rates between the low and high risk groups was 75% based on the NCCN-IPI stratification compared with 45% on the IPI stratification or 54% on the PIT stratification, respectively. When divided into two histologic subgroups (nodal vs. extra-nodal type), the NCCN-IPI showed considerable discriminatory capacity in both histologic groups. However, the IPI or PIT classification could not have discrimination in extra-nodal PTCLs. The interim PET-CT was significantly predicting for progression free survival in all PTCL patients, however, it also showed no predictive value in the patients with extranodal PTCLs, especially NK/T cell lymphoma. Conclusions The NCCN-IPI is a powerful prognostic model in PTCLs predicting overall survival among high-intermediate and high risk patients. Also, interim PET/CT response based on visual assessment could be a valuable prediction tool in nodal PTCLs, however, it should be carefully interpreted in the treatment of extranodal subtypes. Disclosures No relevant conflicts of interest to declare.

scholarly journals Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project

Blood ◽  
2011 ◽  
Vol 118 (1) ◽  
pp. 148-155 ◽  
Author(s):  
Jan Delabie ◽  
Harald Holte ◽  
Julie M. Vose ◽  
Fred Ullrich ◽  
Elaine S. Jaffe ◽  
...  
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
World Health ◽  
Peripheral T Cell Lymphoma

Abstract Few large, international series of enteropathy-associated T-cell lymphoma (EATL) have been reported. We studied a cohort of 62 patients with EATL among 1153 patients with peripheral T-cell or natural killer (NK)–cell lymphoma from 22 centers worldwide. The diagnosis was made by a consensus panel of 4 expert hematopathologists using World Health Organization (WHO) criteria. Clinical correlations and survival analyses were performed. EATL comprised 5.4% of all lymphomas in the study and was most common in Europe (9.1%), followed by North America (5.8%) and Asia (1.9%). EATL type 1 was more common (66%) than type 2 (34%), and was especially frequent in Europe (79%). A clinical diagnosis of celiac sprue was made in 32.2% of the patients and was associated with both EATL type 1 and type 2. The median overall survival was only 10 months, and the median failure-free survival was only 6 months. The International Prognostic Index (IPI) was not as good a predictor of survival as the Prognostic Index for Peripheral T-Cell Lymphoma (PIT). Clinical sprue predicted for adverse survival independently of the PIT. Neither EATL subtype nor other biologic parameters accurately predicted survival. Our study confirms the poor prognosis of patients with EATL and the need for improved treatment options.

International Prognostic Index, Serum IgA Level, and Monocytes Count Are Independently Associated with Overall Survival in Patients with HTLV-I-Negative Nodal Peripheral T-Cell Lymphoma

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1577-1577
Author(s):  
Aiko Kato ◽  
Yukihiro Imai ◽  
June Takeda ◽  
Nobuhiko Yamauchi ◽  
Yuki Funayama ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Prognostic Score ◽  
Univariate Analysis ◽  
Bone Marrow Involvement ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Poor Survival ◽  
Risk Of Death ◽  
Serum Iga

Abstract Abstract 1577 Introduction: Except for minor subtypes of disease with prolonged clinical courses, peripheral T cell lymphoma (PTCL) is clinically aggressive and associated with poor survival. Although the International Prognostic Index (IPI) and the PTCL prognostic index (PIT) are used for prognostic stratification, their predictive utility is in need of improvement. PTCL can be subdivided into two types, nodal and extra-nodal, the latter of which is composed of diseases with characteristic clinical presentations. The nodal group includes PTCL not otherwise specified (NOS), angioimmunoblastic T cell lymphoma (AITL), anaplastic large cell lymphoma (ALCL), and adult T cell leukemia (ATL). While ATL is a distinct disease caused by HTLV-I, the remaining three diseases have several characteristics in common, and their differential diagnosis is sometimes difficult. Patients and Methods: Patients who were newly diagnosed with biopsy-proven, HTLV-I-negative nodal PTCL and referred to our institution between May 1994 and February 2012 were retrospectively analyzed. Patients treated with regimens not intended to induce remission, as well as those with insufficient clinical data, were excluded. This study was Institutional Review Board-approved and complied with the Declaration of Helsinki. The diagnoses were primarily based on histopathology, and in select instances, molecular and immunological analysis was used to support the diagnosis. The overall survival rate (OS) and progression-free survival rate (PFS) were calculated using the Kaplan-Meier method and significance was determined by log-rank test. Univariate and multivariate analyses were performed with the Cox proportional hazards regression model. Results: A total of 77 patients, including 50 PTCL-NOS, 17 AITL, and 10 ALCL patients, 5 of whom were ALK-positive, were identified. The median follow-up time for survivors was 49 months. The median age was 65 years (range, 23–83), and there was a male predominance (male/female ratio: 1.95). 61 had advanced stage (stage III/IV), 20 had more than 1 site of extranodal involvement, and 20 had documented bone marrow involvement. 35 had ECOG performance status of greater than 1. Laboratory data showed elevated serum LDH level in 55. The IPI score was greater than 2 in 47, and the PIT score was greater than 1 in 50. All but one were treated with anthracycline-containing combination chemotherapies. In addition, 16 received high-dose chemotherapy with autologous stem cell rescue. The 5 year OS for the entire population was 42%, and histological diagnosis did not significantly affect OS (the OS for PTCL-NOS, ALCL, and AITL was 35%, 67%, and 47%, respectively). To explore prognostic factors further, univariate analysis was performed using various pretreatment characteristics. Variables significantly associated with poor survival were advanced stage, extranodal involvement > 1 site, bone marrow involvement, anemia, monocyte ≥800 /μL, soluble interleukin-2 receptor > 3,000 U/mL, serum IgG ≥1700 mg/dL, and serum IgA ≥410 mg/dL. The IPI classification was highly correlated with prognosis in this cohort: the relative risk of death was 2.78, 3.99, and 5.61 times higher for pts with IPI of LI, HI, and H, respectively, when compared with IPI L pts (log-rank test; P = 0.0213). By contrast, the PIT classification did not have prognostic value. Then, by using variables associated with poor survival by univariate analysis and not included into IPI, prognostic variables independent of IPI were identified on multivariate analysis. Monocytosis and elevated serum IgA levels were significantly associated with poor survival independently of IPI. The dichotomized monocyte and IgA were combined to generate an IgA/monocytosis prognostic score and pts were stratified into three risk groups: low (IgA < 410 mg/dL and monocyte < 800 /μL), int. (IgA ≥410 or monocyte ≥800), and high-risk (IgA ≥410 and monocyte ≥800) populations. The relative risk of death was 8.56 and 2.83 times higher for pts in the high and int. risk groups, respectively, when compared to the low-risk population (P < 0.0001). Clearly, the new prognostic score was able to stratify pts by risk in a manner comparable to the IPI. Conclusions: Monocytosis and high IgA levels were a novel prognostic factor independent of IPI in the limited number of HTLV-I-negative nodal PTCL patients included in this retrospective study. Further analysis is warranted in a greater number of patients. Disclosures: No relevant conflicts of interest to declare.

Is triple-positive serology for Epstein-Barr virus (VCA-IgG, VCA-IgM, EBNA-IgG) a specific feature of angioimmunoblastic T-cell lymphoma?

2019 ◽  
Vol 106 (5) ◽  
pp. 424-426
Author(s):  
Davide Facchinelli ◽  
Alice Parisi ◽  
Mauro Krampera ◽  
Dino Veneri
Keyword(s):  
T Cell ◽  
Epstein Barr Virus ◽  
Cell Lymphoma ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Positive Serology ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
Barr Virus ◽  
Number Of Patients ◽  
Epstein Barr

Purpose: We assessed the frequency of triple-positive serology (viral capsid antigen [VCA]–immunoglobulin G [IgG], VCA–immunoglobulin M, Epstein-Barr nuclear antigen–IgG) for Epstein-Barr virus (EBV) in a small number of patients with angioimmunoblastic T-cell lymphoma (AITL) at disease onset. Methods: Nine patients with newly diagnosed AITL were retrospectively enrolled in the present study. For all of them, EBV serology data were available. Results: Of 9 patients, 7 (77.7%) had a triple-positive serology (VCA-IgG, VCA-IgM, EBNA-IgG ) for EBV. These patients were characterized by bone marrow involvement, high incidence of thrombocytopenia, and poor prognosis according to Revised International Prognostic Index and Prognostic Index for Angioimmunoblastic T-cell Lymphoma scores. Conclusion: Assessment of both viremia and serology for EBV could be useful in patients with clinical and laboratory data suggesting lymphoma diagnosis; furthermore, although our data need to be validated in a larger cohort of patients, triple positivity for EBV serology might help to direct the diagnosis toward AITL.

scholarly journals Nutrition-Incorporated Pink-E Predicts Survival of Extranodal NK/T-Cell Lymphoma

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2476-2476
Author(s):  
Tiange Lu ◽  
Xiangxiang Zhou ◽  
Yiqing Cai ◽  
Shunfeng Hu ◽  
Yujie Jiang ◽  
...  
Keyword(s):  
T Cell ◽  
High Risk ◽  
Nutritional Status ◽  
Natural Killer ◽  
Validation Cohort ◽  
Cell Lymphoma ◽  
Prognostic Significance ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Conut Score

Abstract Introduction: Patients with aggressive lymphomas are at high risk of losing body resources, resulting in malnutrition, immunodeficiency, impaired life quality and inferior outcomes. Nutritional status is closely associated with the development and treatment arrangements of malignancies, but often neglected in the prognostic assessment. Thus, this study aimed to explore the prognostic significance of nutrition-related parameters in extranodal NK/T-cell lymphoma (ENKTL), an aggressive lymphoma with dismal outcomes. Methods: We retrospectively analyzed patients diagnosed with ENKTL between 2011 and 2020 from Shandong Provincial Hospital and Affiliated Hospital of Qingdao University. The observation endpoints were overall survival (OS) and progression-free survival (PFS). Univariate (UVA) and multivariate (MVA) Cox regression analyses were conducted to examine the significance of variables at diagnosis on OS and PFS. A reformative nutrition assessment-incorporated score system was constructed based on the MVA results. Its performance was tested in the training and validation cohort from multiple aspects, including calibration, discrimination and clinical utility. Results: A total of 160 patients with a median age of 50 (39, 58.75) years and male predominance (67.5%) were included. The prognostic significance of two emerging nutritional indexes, prognostic nutritional index (PNI) and controlling nutritional status (CONUT) score, was assessed. UVA analysis showed PNI was a meaningful marker to OS (HR 2.275, P=0.014) but not to PFS (HR 1.653, P=0.089) while CONUT score was a significant predictor not only to OS (HR 29.385, P＜0.001) but to PFS (HR 12.516, P＜0.001). MVA analysis further verified that CONUT score could independently predict OS (HR 10.247, P=0.001) and PFS (HR 5.587, P=0.001) in addition to prognostic index of natural killer lymphoma plus EBV (PINK-E), another independent marker (PINK-E=2, HR 3.842, P=0.034, PINK-E=3/4/5, HR 9.185, P=0.006 for OS; PINK-E=2, HR 2.308, P=0.06, PINK-E=3/4/5, HR 4.535, P=0.004 for PFS) (Figure 1). Based on this finding, a modified prognosis scoring tool, CONUT-PINK-E, was developed from the training cohort (TC) and tested in the independent external validation cohort (VC). The novel model contains six factors, age≥60 years old, Ann Arbor III/IV stage, distant lymph node involvement, non-nasal type, detectable EBV-DNA in blood and CONUT score≥5 (moderate and severe malnutrition). CONUT-PINK-E differentiated patients into low-, intermediate- and high-risk grades according to the imparities of survival probability revealed by Kaplan-Meier curves. Significant survival differences existed among three risk grades that the median OS was 74, 36, 5 months and median PFS was 31, 14, 4 months, respectively, for the low-, intermediate-, high-risk group (P＜0.001) in TC and the corresponding data were 72, 26, 7 months and 35, 14, 4 months in VC (P＜0.001, Figure 2). Subsequently, the superiority of CONUT-PINK-E was examined by comparison with international prognostic index (IPI), Korean Prognostic Index (KPI), Prognostic index of natural killer lymphoma (PINK) and PINK-E. The 1- to 5-year time-dependent areas under receiver operating characteristic curve (AUCs) hinted that CONUT-PINK-E (TC: 0.832-0.961, VC: 0.773-0.937) possessed higher discrimination than IPI (TC: 0.710-0.785, P&lt;0.001; VC: 0.646-0.840, P&lt;0.001), KPI (TC: 0.659-0.826, P&lt;0.001; VC: 0.623-0.691, P&lt;0.001), PINK (TC: 0.802-0.865, P&lt;0.001; VC: 0.663-0.842, P&lt;0.001) and PINK-E(TC: 0.815-0.902, P&lt;0.001; VC: 0.741-0.894, P&lt;0.001) (Figure 3A, 3B). Decision curve analysis plainly showed CONUT-PINK-E brought higher clinical net benefits than IPI, KPI, PINK and PINK-E in forecasting OS and PFS of ENKTL (Figure 3C, 3D). In addition, net reclassification index (NRI) and integrated discrimination improvement (IDI) conformably demonstrated that CONUT-PINK-E achieved significant improvements than the prior stratification tools. Conclusion: This is the first time that CONUT score was recognized capable of acting as an independent prognostic marker in newly-diagnosed ENKTL patients. More importantly, the first risk score covering nutritional status assessment for ENKTL, CONUT-PINK-E, was proposed and presented striking performance. The newly-built model could deliver vital reference to the risk stratification and comprehensive managements of ENKTL patients. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Pretreatment Whole Blood Epstein-Barr Viremia Is a Prognostic Factor in Peripheral T-Cell Lymphoma

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4143-4143
Author(s):  
Yu Ri Kim ◽  
Soo-Jeong Kim ◽  
June-Won Cheong ◽  
Hyewon Lee ◽  
Haerim Chung ◽  
...  
Keyword(s):  
Overall Survival ◽  
Multivariate Analysis ◽  
T Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Free Survival

Abstract Abstract Peripheral T-cell lymphoma (PTCL) is the highly aggressive lymphoid malignancies, treatment outcome is very poor. There are increasing evidence about the role of Epstein-Barr virus (EBV) in PTCL. Because of its rarity, there was few studies about the prognostic factors incorporating EBV in PTCL. The aim of this study was to evaluate the role of EBV as prognostic factors in PTCL. We retrospectively reviewed the 174 PTCL patients (peripheral T-cell lymphoma, not otherwise specified; n=123, anaplastic large cell lymphoma; n=19, angioimmunoblastic T-cell lymphoma; n=26, enteropathy related T-cell lymphoma, n=5, hepatosplenic gammadelta T-cell lymphoma; n=1). Median age of the patients was 63 (20~94) years with 107 (61.5%) male patients. One-year OS and PFS was 55.5%, 37.5%, respectively. Stage 3 or 4 patients were 150 (86.2%). Bone marrow involvement were detected 73 (42.0%) patients among 163 available patients. For IPI scores, 29 (16.7%) patients were classified as low risk, 42 (24.1%) as low-intermediate risk, 57 (32.8%) as high-intermediate risk, and 46 (26.4%) as high risk. For PIT scores, 18 (11.1%) patients were classified in group 1, 41 (25.2%) in group 2, 58 (35.6%) in group 3, and 46 (28.2%) in group 4. Upfront autologous hematopoietic stem cell transplantation (n=17) improved OS and PFS (P=0.001 and P<0.001, respectively). In univariate analysis, poor performance status (ECOG ¡Ã2) (P <0.001 and P <0.001, respectively), low absolute lymphocyte counts (<1000/mm3) (P=0.022 and P=0.038, respectively), high ferritin (¡Ã1,000/mm3) (P =0.002 and P =0.002, respectively), EBV viremia in the whole blood (positive) (P=0.016 and P <0.001, respectively), low protein level (<6.3 g/dL) (P <0.001 and P <0.001, respectively) and low albumin level (<3.5 g/dL) (P =0.001 and P =0.001, respectively) were related with inferior OS and PFS. High international prognostic index (IPI) and prognostic index for PTCLu (PIT) were related with inferior OS and PFS (P<0.001, P=0.029 and P=0.019, P=0.278, respectively) (Figure 1A, 1B, 2A, 2B). In multivariate analysis, poor performance status, extranodal involvement more than one site and EBV viremia were related with OS and PFS in multivariate analysis. (P <0.001, P =0.024, P =0.001 and P =0.001, P=0.002, P=0.031, respectively). We made a new prognostic score model using statistically significant 3 variables in multivariate analysis: low, no adverse factors; intermediate, one factor; high, two or three factors. This model could identify three groups of patients for OS and PFS (Figure 3A,3B.) This study suggests that prognostic models including EBV for PTCL showed good risk classification. There will be need to investigate the mechanism of EBV and specific treatment strategy for EBV-related patients. These patients will be need to consider more effective therapeutic strategy to improve the poor survival in PTCL. Figure 1. Overall survival and progression free survival according to international prognostic index Figure 1. Overall survival and progression free survival according to international prognostic index Figure 2. Overall survival and progression free survival according to prognostic index for PTCLu Figure 2. Overall survival and progression free survival according to prognostic index for PTCLu Figure 3. Overall survival and progression free survival according to new prognostic model Figure 3. Overall survival and progression free survival according to new prognostic model Disclosures No relevant conflicts of interest to declare.

A Retrospective Analysis on Prognostic Factors of Angioimmunoblastic T-Cell Lymphoma: a Multicenter Cooperative Study In Japan

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3117-3117
Author(s):  
Takashi Tokunaga ◽  
Kazuyuki Shimada ◽  
Kazuhito Yamamoto ◽  
Dai Chihara ◽  
Takuji Ichihashi ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
T Cell ◽  
Research Funding ◽  
Cell Lymphoma ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Rank Test ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
Log Rank Test ◽  
Number Of Patients

Abstract Abstract 3117 Background: Angioimmunoblastic T-cell lymphoma (AITL) is one of the major types of peripheral T-cell lymphoma (PTCL), with T follicular helper cells (TFH) reported to be the normal counterpart cell type. The disease generally presents with poor prognosis following conventional chemotherapy treatments. Furthermore, existing prognostic factors or predictive models for non-Hodgkin lymphoma are not useful in the prognostification of AITL. Identification of novel prognostic factors is therefore vital. Unfortunately, the number of studies using a large cohort of patients with AITL has so far been limited. Patients and Method: To elucidate the clinicopathological characteristics of AITL in Japan, we retrospectively analyzed 213 patients who were diagnosed with AITL between January 1990 and September 2008 from 31 participating hospitals. Patients with AITL were eligible for analysis only if their diagnosis was confirmed by histopathological and immunohistochemical criteria in accordance with the WHO classification. For immunohistochemical analysis, we evaluated CD10, CXCL13, PD-1 and EBER-ISH in addition to routine immunostaining. Clinical data was retrospectively collected from case reports. Patients received treatment for AITL according to the respective institutional protocols. Overall survival (OS) and progression free survival (PFS) were analyzed by using the log-rank test, and results expressed as Kaplan-Meier plots. Cox proportional hazard regression analysis with OS and PFS was performed to identify potential independent prognostic factors. This study was approved by the institutional review board of participating hospitals and complied with the provisions of the Declaration of Helsinki. Result: The median patient age was 67 years (range: 34–89 years), with 74% of patients older than 60 years. The female:male ratio was 1:1.8. Ninety percent of patients displayed Stage III or IV disease, and 23% of patients involved more than 1 extranodal site. B-symptoms and bone marrow involvement were present in 60% and 30% of patients, respectively. Laboratory findings showed anemia (male: Hb <13.0 g/dl, female: Hb <11.0 g/dl) in 61% of patients, a positive Coombs test in 47%, hypergammaglobulinemia (IgG >1700 mg/dl) in 54%, IgA >400 mg/dl in 37%, and elevated serum LDH levels in 75% of patients, respectively. According to the international prognostic index (IPI) and prognostic index for PTCL-NOS (PIT) score, patients were categorized as follows; IPI: Low (L), 10% (22/199); Low-intermediate (LI), 20% (39/199); High-intermediate (HI), 39% (77/199); and High (H), 31% (61/199), respectively, and PIT: Group1 (G1), 4% (8/201); Group2 (G2), 19% (38/201); Group3, 42% (85/201); and Group4, 35% (70/201), respectively. In terms of the initial series of treatments, 84% of patients received anthracycline-based chemotherapies. With a median follow-up duration of 42 months in surviving patients, 3-year OS and PFS were 54% and 39%, respectively. IPI was predictive for OS (3-years OS: L, 84%; LI, 65%; HI, 54%; H, 38%; Log-rank test, p<0.001), however, PIT was less predictive than IPI according to the distribution of the number of patients and survival in each group (3-years OS: G1, 88%; G2, 65%; G3, 57%; G4, 42%; Log-rank, p=0.014). Immunohistochemical staining revealed positivity for CD10 in 31% (40/130), EBER-ISH in 68% (108/160), CXCL13 in 92% (76/83), and PD-1 in 61% of patients (51/83), respectively. Multivariate analysis revealed total protein (TP) (<6.5 g/dl), {hazard ratio (HR), 2.12; 95% confidence interval (CI), 1.20–3.72; p=0.010}, IgA (>400 mg/dl) (HR, 2.00; 95% CI, 1.19–3.34; p=0.009), anemia (male, Hb <13.0 g/dl; female, Hb <11.0 g/dl) (HR, 1.95; 95% CI, 1.11–3.52; p=0.020), CRP (>1.0 mg/dl) (HR, 1.84; 95% CI, 1.05–3.35; p=0.033), and performance status (>2) (HR, 1.73; 95% CI, 1.03–2.92; p=0.040) were identified as significant prognostic factors for OS. IgA (HR, 1.94; 95% CI, 1.25–2.98; p=0.003), and anemia (HR, 1.65; 95% CI, 1.03–2.66; p=0.036) were significant prognostic factors for PFS. Conclusion: Prognosis of patients with AITL in Japan is poor. Although IPI was useful in prognostification of AITL, other factors including those not adopted in IPI, such as IgA, anemia, TP and CRP, significantly affected the prognosis in this analysis. Further validation studies of these criteria should be performed. Disclosures: Naoe: Chugai Pharmaceutical Co.,Ltd.: Research Funding; Zenyaku Kogyo Co.: Research Funding; Kyowa Hakko Kirin Co.,Ltd.: Research Funding; Dainippon Sumitomo Pharma Co.,Ltd.: Research Funding; Novartis Pharma K.K.: Research Funding; Janssen Pharmaceutical K.K.: Research Funding. Kinoshita:Chugai Pharmaceutical Co.,Ltd.: Research Funding; Zenyaku Kogyo Co.: Research Funding; Kyowa Hakko Kirin Co.,Ltd.: Research Funding.

scholarly journals A Clinically-Indolent Variant of Extranodal NK/T Cell Lymphoma with Unique Immunophenotypic Profile and Superior Outcome

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5278-5278
Author(s):  
Fabiola Valvert ◽  
Elizabeth Solorzano ◽  
Edward Briercheck ◽  
Marcos Mauricio Siliézar Tala ◽  
Yasodha Natkunam ◽  
...  
Keyword(s):  
T Cell ◽  
Nk Cell ◽  
Cell Lymphoma ◽  
Elevated Serum ◽  
T Cell Lymphoma ◽  
Nasal Type ◽  
Aggressive Disease ◽  
Ebv Infection ◽  
Nk T Cell ◽  
Indolent Disease

Introduction Extranodal NK/T-cell lymphoma, nasal type (ENKTL), is the most frequent NK-cell malignancy. It is typically associated with a highly aggressive course and extensive local tissue destruction. ENKTL, nasal type, is most common among East Asians and indigenous persons in Latin America, which may result from genetic predisposition, shared strains of EBV infection or other factors. We noted that a subset of patients with ENKTL in Guatemala present with more indolent disease. The clinical and histologic features of these indolent cases, including outcomes after treatment, have not been defined. Methods We reviewed clinical data from 68 patients with ENKTL at INCAN, the largest public cancer hospital in Guatemala, who underwent evaluation between 2006-2018. We confirmed the diagnosis of ENKTL using available paraffin-embedded biopsies based on immunohistochemistry and in situ hybridization for 46 markers at Stanford University (O.S., Y.N.). We defined indolent cases as those lacking macroscopic necrosis, palate perforation, distant lesions (i.e. Stage II or greater), hemophagocytic lymphohistiocytosis (HLH) and B symptoms. Aggressive cases had one or more of these characteristics. Statistical analysis on categorical data was performed using Fisher's exact test. Results Fifty-three patients were confirmed to have ENKTL. The median age at the time of diagnosis was 43 years (range: 11-83) and 36 patients were male (68%). 75.7% of patients self-identified as Mayan ancestry and 85% were born or lived in central or western Guatemala. As outlined in the Table, 14 cases were classified as indolent and 39 were aggressive. Patients with indolent NKTCL were older (mean, 51 years vs. 41.5 years in the aggressive group; p=0.04). Patients with aggressive disease more commonly had anemia, lymphocytopenia and elevated serum LDH. Both indolent and aggressive cases typically had NK cell immunophenotype, including positivity for CD56, granzyme, perforin and TIA-1. All 53 NKTCLs expressed EBER, consistent with EBV infection, with a subset in each group also expressing EBV LMP1. In contrast, greater than 40% of aggressive cases expressed CXCL13 compared to 0% of indolent cases (p=0.005). Aggressive cases were more commonly BCL2 positive (67% versus 31%, p = 0.048). A subset of aggressive cases had Ki67 >50% (6/39 versus 0/14 indolent cases) but there were also aggressive cases with Ki67 <10%. A multiple correspondence analysis using 14 clinical and 18 IHC markers was performed on 33 patients with complete data available. Variables contributing to categorization of aggressive versus indolent ENKTL included palate perforation, peripheral blood lymphocyte count < 0.8 K/uL, B symptoms, anemia, cachexia and macroscopic necrosis. Median survival was markedly better for patients with indolent disease compared to those with aggressive disease (median not reached vs. 2 years, p<0.05). Twelve of (92.9%) thirteen treated patients in the indolent group achieved a complete response compared to only 8 (40%) of 22 treated for aggressive disease (p=0.04). In fact, 9 patients with aggressive disease died before receiving treatment compared to 0 with indolent disease (23.0% vs. 0%; p=0.04). Three of the deaths in patients with indolent disease were due to toxicity from chemotherapy (infection, pancytopenia). Conclusion Approximately one-quarter of patients with extranodal NK/T cell lymphoma, nasal type, in our cohort have a unique variant associated with the absence of aggressive clinical features. These patients have a more indolent clinical course, better outcome with treatment, have less frequent expression of BCL2, and lack CXCL13 expression. Patients with the indolent variant may benefit from less aggressive therapeutic approaches to minimize unnecessary treatment-associated toxicity. Efforts to define genetic and transcriptional characteristics of these cases are underway. Table Disclosures Weinstock: Celgene: Research Funding.

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