The NCCN-IPI Score Is Prognostic of Survival in Patients with Peripheral T-Cell Lymphoma, Not Otherwise Specified

Abstract Introduction: Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) accounts for 15-20% of NHL and is characterized by a poor survival. The IPI and PIT scores are prognostic factors in survival in B-cell and T-cell lymphomas, but not without limitations. The aim of this study is to evaluate the prognostic value of the NCCN-IPI score in patients with PTCL-NOS. Methods: We included patients with a pathological diagnosis of PTCL-NOS who were diagnosed and treated at our institution between 1997 and 2017. IRB approval was obtained prior to research. Pathological samples were reviewed by hematopathologists at our institution to confirm the diagnosis. Pertinent clinicopathological data were collected through chart review and are presented using descriptive statistics. Survival curves were estimated using the Kaplan-Meier method and compared using the log-rank test. Univariate and multivariate Cox proportional-hazard regression models were fitted to evaluate hazard ratios (HR) for overall survival (OS). Results: A total of 173 patients with diagnosis of PTCL-NOS were included in this analysis. The median age at diagnosis was 58 years (range 18-91 years) with a male predominance (58%). Clinically, 47% of patients were 60 or older, 52% presented with ECOG >1, 82% had elevated serum LDH, 74% had extranodal disease, and 34% had stage I/II and 66% had stage III/IV. IPI score distribution was low-risk in 55% of patients, low-intermediate in 36%, high-intermediate in 48% and high-risk in 33%. PIT score distribution was low-risk in 53%, low-intermediate in 67%, high-intermediate in 38% and high-risk in 14%. NCCN-IPI score distribution was low risk in 40%, low-intermediate in 56%, high- intermediate in 63% and high risk in 11%. 22% of patients received CHOEP, 23% received CHOP, and 55% received other regimens. The overall response rate was 59%; 47% had a complete response and 12% had a partial response. The 5-year overall survival (OS) rate was 32% with a median OS of 12 months. PTCL-NOS patients with low, low-intermediate, high-intermediate and high-risk NCCN-IPI had 5-year OS rates of 46%, 36%, 26% and 0%, respectively (p<0.001). When compared with patients with low-risk NCCN-IPI, patients with low-intermediate (HR 3.2, 95% CI,1.1-9.5; p=0.044) and high-intermediate /high risk NCCN-IPI (HR 6.0, 95% CI 2.1-17; p=0.001) had worse OS. Conclusions: We have validated the NCCN-IPI score as a prognostic tool in patients with PTCL-NOS. This work can serve to address future prospective designs that allow selection of groups of patients at greater risk and thus lead to more individualized therapy. Figure. Figure. Disclosures Castillo: Janssen: Consultancy, Research Funding; Genentech: Consultancy; Abbvie: Consultancy, Research Funding; Pharmacyclics: Consultancy, Research Funding; Millennium: Research Funding; Beigene: Consultancy, Research Funding.

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Nodal Peripheral T-Cell Lymphoma – a Clinical and Epidemiological Analysis at Medicine School of Sao Paulo University

Blood ◽

10.1182/blood.v124.21.1706.1706 ◽

2014 ◽

Vol 124 (21) ◽

pp. 1706-1706

Author(s):

Luis Alberto de Padua Covas Lage ◽

Marianne Castro Goncalves ◽

Rodrigo Santucci ◽

Renata Oliveira Costa ◽

Debora Levy ◽

...

Keyword(s):

Overall Survival ◽

T Cell ◽

High Risk ◽

Cell Lymphoma ◽

Progression Free Survival ◽

Large Cell ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Free Survival ◽

Large Cell Lymphoma

Abstract Background: Peripheral T-cell lymphoma (PTCL) are a biologically and clinically heterogeneous group of rare diseases arising from mature or activated post-thymic T lymphocytes. Correspond to 10% to 15% of lymphoid malignancies with marked geographical variation in incidence. According to the WHO classification they are divided into nodal, extranodal, primary cutaneous and leukemic or disseminated and encompass 18 distinct entities. The nodal group involves the peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), angioimmunoblastic lymphoma (AITL), anaplastic large cell lymphoma ALK positive (ALCL-ALK+) and anaplastic large cell lymphoma ALK negative (ALCL-ALK-). The literature of PTCL is scarce, especially in our country where data of epidemiology, clinical features and outcomes are usually rarely available. So, to better understand PTCL we performed a retrospective study with patients treated in a reference service for cancer treatment in Brazil. Methods: Eight-seven nodal PTCL patients treated with anthracyclne-based regimen (CHOP or, CHOEP) from January 2000 to June 2014 were evaluated retrospectively at the Medicine School of Sao Paulo University, Brazil. All patients lower than 60 years were consolidated with autologous hematopoietic stem cell transplantation (ASCT) in first CR or PR except that with ALCL-ALK+ diagnosis. Refractory and relapsed patients were salvaged with 3-4 cycles of IVAC (Ifosphamide 1.5 g/m2 i.v D1-D5, etoposide 100mg/m2 i.v D1-D5, aracytin 2g/m2 i.v twice a day D1-D2) regimen and submitted to ASCT. It was performed a central histopathological review and clinical and epidemiological data were obtained from medical records. Patients were evaluated for overall response (OR) including complete response (CR) and partial response (PR), overall survival (OS) and progression free survival (PFS). Statistical analysis was performed using the STATA-3 program using and a p-value ≤ 0.05 was considered statistically significant. Results: Of the 87 patients, 34 (39.08%) cases were classified as ALCL-ALK-, 27 (31.03%) as PTCL-NOS, 16 (18.39%) as ALCL-ALK+, 6 (6.89%) as AITL and in 4 (4.1%) cases the diagnosis could not be performed and an expansion of the immunohistochemical is ongoing. Thirty-six (45.38%) cases were female and 51(54.62%) were male, 59(67.81%) patients were lower than 60 years. Seventy-six (87.35%) patients presented in advanced stage (III or IV) at diagnosis but 73(83.90%) patients presented an ECOG < 2 and 14(16.10%) ≥ 2. Eighteen (20.70%) patients were of low-risk, 26 (29.88%) of low-intermediate risk and 43(49.42%) of high-intermediate and high-risk of international prognostic index (IPI). The CR and PR was obtained for 44(50.57%) and 8(9.19%), respectively with 59.76% OR. Thirty (34.48%) patients were primary refractory and five remain under treatment. In a median of follow of 30 months, ALCL-ALK+ show higher OS (median 140.98 months) than ALCL-ALK- (44.20 months), PTCL-NOS (median 20.62 months) and AITL (median 7.24 months) (p=0.41) (Figure 1A). The median of PFS was 3.84 months for AITL, 23.44 months for ALCL-ALK+, 40.03 months for PTCL-NOS and was not yet reached for ALCL-ALK- (p=0.0006) (Figure 1B). Figure 1: Overall survival (1A) and Progression Free Survival (1B) of nodal PTCL Figure 1:. Overall survival (1A) and Progression Free Survival (1B) of nodal PTCL Figure 2 Figure 2. Conclusion: In this study we showed that ALCL-ALK+ as well as found in the literature presented a better OS in comparison to others nodal T-cell lymphoma as AITL, PTCL-NOS and ALCL-ALK-. Surprisingly the PFS of ALCL-ALK+ was statistically significant lower than of ALCL-ALK-. We thought that this result may be explained because in our service until to perform this analysis we did not indicate ASCT in first CR for ALCL-ALK+, but for all ALCL-ALK-. This hypothesis may be reinforced as the most of our cases presented high-intermediate and high-risk of IPI and that could equalize the favorable effect of ALK expression. In addition, we changed our approach and we are also indicating ASCT in first line for patients with ALCL-ALK+ with intermediate-high and high-risk of IPI . Disclosures No relevant conflicts of interest to declare.

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

Blood ◽

10.1182/blood.v128.22.1822.1822 ◽

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.

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Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project

Blood ◽

10.1182/blood-2010-09-310342 ◽

2011 ◽

Vol 117 (12) ◽

pp. 3402-3408 ◽

Cited By ~ 228

Author(s):

Dennis D. Weisenburger ◽

Kerry J. Savage ◽

Nancy Lee Harris ◽

Randy D. Gascoyne ◽

Elaine S. Jaffe ◽

...

Keyword(s):

Overall Survival ◽

T Cell ◽

Cell Lymphoma ◽

International Prognostic Index ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Bulky Disease ◽

Free Survival ◽

Not Otherwise Specified ◽

Pathologic Features

Abstract The International Peripheral T-cell Lymphoma Project is a collaborative effort to better understand peripheral T-cell lymphoma (PTCL). A total of 22 institutions submitted clinical and pathologic material on 1314 cases. One objective was to analyze the clinical and pathologic features of 340 cases of PTCL, not otherwise specified. The median age of the patients was 60 years, and the majority (69%) presented with advanced stage disease. Most patients (87%) presented with nodal disease, but extranodal disease was present in 62%. The 5-year overall survival was 32%, and the 5-year failure-free survival was only 20%. The majority of patients (80%) were treated with combination chemotherapy that included an anthracycline, but there was no survival advantage. The International Prognostic Index (IPI) was predictive of both overall survival and failure-free survival (P < .001). Multivariate analysis of clinical and pathologic prognostic factors, respectively, when controlling for the IPI, identified bulky disease (≥ 10 cm), thrombocytopenia (< 150 × 109/L), and a high number of transformed tumor cells (> 70%) as adverse predictors of survival, but only the latter was significant in final analysis. Thus, the IPI and a single pathologic feature could be used to stratify patients with PTCL-not otherwise specified for novel and risk-adapted therapies.

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Bone Marrow Involvement Detected By Multi-Parameter Flow Cytometry Predicts Poor Outcome after Autologous Stem Cell Transplantation for Peripheral T-Cell Lymphoma

Blood ◽

10.1182/blood.v126.23.1972.1972 ◽

2015 ◽

Vol 126 (23) ◽

pp. 1972-1972

Author(s):

Jordan Gauthier ◽

Leona Holmberg ◽

David Wu ◽

William I. Bensinger ◽

Ajay K. Gopal ◽

...

Keyword(s):

Flow Cytometry ◽

Overall Survival ◽

T Cell ◽

Cumulative Incidence ◽

Research Funding ◽

Residual Disease ◽

Cell Lymphoma ◽

Multivariable Analysis ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma

Abstract BACKGROUND: Peripheral T-cell lymphomas (PTCL) encompass a heterogeneous group of neoplasms accounting for 10 to 15% of non-Hodgkin lymphomas worldwide. Prognosis for PTCL patients is poor and consolidation in first remission with autologous stem cell transplantation (ASCT) is widely used. Most patients though still relapse after transplant. We hypothesized that pre-ASCT bone marrow (BM) involvement detected by multi-parameter flow cytometry (FC) would identify patients with inferior outcome after ASCT. METHODS: We retrospectively analyzed the outcome of 29 consecutive PTCL patients who underwent ASCT at the Fred Hutchinson Cancer Research Center from April 2004 through July 2014. Pre-ASCT BM involvement by flow cytometry (FC) was defined as the presence of an abnormal T-cell population detected by multi-parameter FC analysis in a BM aspirate obtained within 30 days prior to ASCT. An abnormal T-cell population accounting for a percentage equal or greater than 0.01% of total leukocytes after red blood cell lysis was considered significant. RESULTS: Ten patients (34%) with angioimmunoblastic T cell lymphoma (AITCL), 8 (27%) with ALK-negative anaplastic large cell lymphoma (ALCL), 8 (27%) with peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) and 3 (12%) with other PTCL sub-types were included. Median age at transplant was 54 (range: 29-71). Twenty patients (76%) presented at ASCT in complete remission (CR) per 1999 Cheson criteria and 9 (31%) were in first CR (CR1). Fifteen patients (50%) underwent ASCT upfront. Pre-ASCT BM involvement was detected by FC analysis in 7 patients (24%, 3 patients with AITCL, 1 patient with ALCL and 3 patients with PTCL-NOS) and by morphology in 2 patients (7%, 2 patients with AITCL). The 7 patients with pre-ASCT BM involvement detected by FC experienced a significantly higher 4-year cumulative incidence of relapse (CIR) (85% versus 36%, p < 0.001) and lower overall survival (OS) (19% versus 89%, p < 0.001) with a median follow-up of 51 months. These findings were confirmed in multivariable analysis for CIR (HR = 7.37, CI = 1.14 - 47.61, p = 0.03) and OS (HR = 7.04, CI = 1.29 - 38, p = 0.024). The IPI score, absence or presence of CR1 and the number of prior therapies were included in a multivariable model for CIR, while age and the IPI score at diagnosis were taken into account for OS. ASCT performed in CR1 was associated with lower CIR (HR = 0.11, CI = 0.03-0.041, p < 0.001). Histologic subtypes did not impact CIR (p = 0.97) nor OS (p = 0.91) and they did not significantly differ between the groups with and without pre-ASCT BM involvement detected by FC (p = 0.60) after applying Fisher's exact test. Further analysis of the 22 patients presenting in CR at ASCT revealed that in this subgroup, pre-ASCT BM involvement by FC (n = 4) was also associated with higher CIR (75% versus 32%, p = 0.002) and lower OS (25 versus 94%, p <0.001). The prognostic impact of pre-ASCT BM involvement by FC persisted in multivariable analysis for CIR (HR = 16, CI = 1.11-228.70, p = 0.042, IPI score at diagnosis and number of prior therapies considered as covariables) but not for OS (HR = 0.15, CI = 0.65-65.40, p = 0.10, multivariable model including age and the IPI score at diagnosis). CONCLUSION: Pre-ASCT BM involvement by FC correlated with dramatically higher relapse rates and an inferior OS in PTCL patients after ASCT. Furthermore, we demonstrate that residual disease, detected only in patients meeting Cheson 1999 criteria for CR at ASCT is capable of predicting a higher risk for relapse. These findings should encourage further evaluation of minimal residual disease in PTCL patients achieving complete remission as defined per Cheson 1999 and 2007 criteria. Figure 1. Cumulative incidence of relapse Figure 1. Cumulative incidence of relapse Figure 2. Overall survival Figure 2. Overall survival Disclosures Gopal: Merck: Research Funding; Emergent/Abbott: Research Funding; Cephalon/Teva: Research Funding; BioMarin: Research Funding; Sanofi-Aventis: Honoraria; Millenium: Honoraria, Research Funding; Seattle Genetics: Consultancy, Honoraria, Research Funding; Janssen: Consultancy, Research Funding; Pfizer: Consultancy, Research Funding; Spectrum: Consultancy, Research Funding; Gilead: Consultancy, Research Funding; Piramal: Research Funding; Biogen Idec, BMS: Research Funding. Maloney:Juno Therapeutics: Research Funding; Roche/Genentech: Honoraria; Janssen Scientific Affairs: Honoraria; Seattle Genetics: Honoraria. Till:Roche-Genentech: Research Funding.

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Clinical Characteristics and Survival in Patients with EBV Positive Diffuse Large B- Cell Lymphoma, Not Otherwise Specified: Report of 60 Cases

Blood ◽

10.1182/blood-2018-99-117185 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 2986-2986

Author(s):

Brady E. Beltrán ◽

Denisse A. Castro ◽

Yesenia M. Huerta- Collado ◽

Jose Manuel Malaga ◽

Mauricio Postigo ◽

...

Keyword(s):

High Risk ◽

B Cell ◽

Research Funding ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Low Risk ◽

Stage Iii ◽

Score Distribution ◽

Not Otherwise Specified ◽

Large B Cell Lymphoma

Abstract Introduction: Epstein Barr virus-positive (EBV+) diffuse large B- cell lymphoma (DLBCL), not otherwise specified (NOS) is a new entity recognized in the 4th edition of the 2016 classification of the WHO. Prevalence rates range from 5% in Western countries to 10-15% in Asia and South America. The IPI and Oyama score are prognostic factors for survival in this entity. The aim of this study is to evaluate the clinical characteristics and survival in EBV+ DLBCL, NOS. Methods: The study was retrospective, reviewing clinical records of patients treated at Rebagliati Martins Hospital between years 2002 - 2017. Patients of both sexes greater than or equal to eighteen years of age with the diagnosis of EBV+DLBCL, NOS were included. IRB approval was obtained prior to research. Pathological samples were reviewed by hematopathologists at our institution to confirm the diagnosis. Pertinent clinicopathological data were collected through chart review and are presented using descriptive statistics. Overall survival (OS) was determinate according to the Kaplan -Meier method, the comparison of the survival curves were made with the log-rank test. Univariate and multivariate Cox proportional-hazard regression models were fitted to evaluate hazard ratios (HR) for OS. Results: A total of 60 patients with a diagnosis of EBV+DLBCL, NOS were included in this analysis. The median age at diagnosis was 70 years (range 25-95 years), 62 % of patients were older than 60 and there was a male predominance (65%). Activated B-cell phenotype (ABC) was present in 57% of patients. Clinically, 62% presented ECOG >1, 52% had elevated serum LDH, 57% had B symptoms, 67% had an extranodal disease and 55% had stage III/IV. IPI score distribution was low-risk in 28% of patients, low-intermediate in 16%, high-intermediate in 30% and high-risk in 26% of patients. The Oyama score distribution was low risk in 20 % of patients, intermediate risk 45 % and high risk in 35% of patients. NCCN-IPI score distribution was low-risk in 5% of patients, low-intermediate in 25%, high-intermediate in 42% and high-risk in 28% of patients. 51% of patients received R-CHOP, 29% received CHOP, and 20% received other regimens. The overall response rate was 56%; 44% had a complete response and 12% had a partial response. The 5-year OS rate was 38% with a median of 9 months. In the univariate analysis, ECOG 2-4 (p=0.001), stage III/IV (p=0.001), CHOP and others regimens (p=0.025) were associated with a poor prognosis. EBV+ DLBCL, NOS patients with low, low-intermediate, high-intermediate and high-risk IPI had 5-year OS rates of 47%, 66%, 29% and 27%, respectively (p=0.006). In the NCCN-IPI score, EBV+ DLBCL, NOS patients with low/ low-intermediate, high-intermediate and high-risk had 5-year OS rates of 55%, 41%, 21%, respectively (p=0.014). In the Oyama score, EBV+ DLBCL, NOS patients with low, intermediate and high-risk had 5-year OS rates of 68%, 47%, 27%, respectively (p=0.004) In the multivariate analysis, patients with stage III/IV had a worse outcome (HR 3.8, 95% 1.6-9.2; p=0.003). The IPI score (HR 2.6, 95%CI, 1.1-6.3), NCCN-IPI score (HR3.4, 95%CI, 1.3-8.6, and Oyama score (HR 6.8, 95%CI, 1.5-31.2) were significant in the high-risk group. Conclusions: This is the largest Latin American cohort with EBV positive DLBCL, NOS reported so far. This entity is an aggressive tumor with a worse prognosis. IPI, NCCN-IPI and Oyama score are good prognostic index models for this disease. Rituximab appears to improve OS. Figure. Figure. Disclosures Castillo: Janssen: Consultancy, Research Funding; Pharmacyclics: Consultancy, Research Funding; Millennium: Research Funding; Beigene: Consultancy, Research Funding; Genentech: Consultancy; Abbvie: Consultancy, Research Funding.

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Decreased Peripheral Blood Lymphocyte-Monocyte Ratio and Platelet-Monocyte Ratio and the Peripheral Blood Score Model Predict Poor Survival in Peripheral T-cell Lymphoma Patients

10.21203/rs.3.rs-130878/v1 ◽

2020 ◽

Author(s):

Yan Zhang ◽

Yuanfei Shi ◽

Huafei Shen ◽

Lihong Shou ◽

Qiu Fang ◽

...

Keyword(s):

T Cell ◽

High Risk ◽

Peripheral Blood Lymphocyte ◽

Peripheral Blood ◽

Blood Lymphocyte ◽

Cell Lymphoma ◽

Risk Group ◽

Low Risk ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma

Abstract Background. Peripheral T-cell lymphoma(PTCL) is a group of lymphoproliferative tumors originated from post-thymic T cells or mature natural killer (NK) cells. It shows highly aggressive clinical behaviour, resistance to conventional chemotherapy, and a poor prognosis. Its incidence rate in China is 1 to 2 times higher than in western countries. Therefore, optimal strategies for identifying high-risk patients are urgently needed. Methods. We retrospectively studied 347 newly diagnosed PTCL patients from January 2011 to October 2019 and analyzed the relationship between peripheral blood lymphocyte-monocyte ratio (LMR) and platelet-monocyte ratio (PMR) and prognosis. The model of Peripheral Blood Score was established to screen out high-risk patients.Results. The receiver operating characteristic (ROC) curve was used to determine the optimal cut-off value based on survival rate. It was found that patients with PTCL with LMR ≤ 1.68 and PMR ≤ 300 had inferior overall survival (OS) and the difference was significant in both low-risk (P＜0.001) and medium high-risk (P＜0.001) groups of IPI score. In multivariate analysis, LMR ≤ 1.68 (HR=1.751, 95% CI 1.158-2.647, p=0.006), PMR ≤ 300 (HR=1.762, 95% CI 1.201-2.586, p=0.002), stage III-IV (HR=3.276, 95% CI 1.512-7.099, p=0.003), Eastern Cooperative Oncology Group (ECOG) score 3-5 (HR=2.351, 95% CI 1.647-3.356, p＜0.001) and extra-nodal invasion more than one site (HR=1.659, 95% CI 1.125-2.445, p=0.039) were independently associated with short survival. LMR and PMR were integrated into "Peripheral Blood Score (PBS)" model. PTCL patients were divided into three risk groups: low-risk group, medium risk group and high-risk group. The 1-year OS was 86%, 55.3% and 22.6%, and the 3-year OS was 43.4%, 20% and 13.1%, respectively.Conclusion. Overall, LMR and PMR can be used as early prognostic indicators in PTCL patients. Moreover, we can easily detect the complete blood cell count (CBC), and use PBS model to preliminarily screen and stratify patients. It is simple, convenient and accurate to screen out patients with short lives, and formulate personalized treatment strategies.

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RDW Is a Prognostic Marker for Patients with Aggressive Peripheral T-Cell Lymphoma

Blood ◽

10.1182/blood-2018-99-113236 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 5364-5364

Author(s):

Brady E. Beltrán ◽

Denisse A. Castro ◽

Yesenia M. Huerta- Collado ◽

Eduardo Sotomayor ◽

Jorge J. Castillo

Keyword(s):

Overall Survival ◽

T Cell ◽

Research Funding ◽

Cox Regression ◽

De Novo ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Curative Intent ◽

The Impact

Abstract Introduction: Red blood cell distribution width (RDW) has emerged as a potential prognostic factor in solid tumors and lymphomas. Previous studies have shown an association between increased levels of RDW and inflammatory diseases, being a surrogate marker of inflammation and a predictor of poor outcome. Data on the impact of RDW in outcomes of patients with aggressive peripheral T-cell lymphoma (PTCL) are scarce. Methods: We performed a retrospective study on consecutive patients with a de novo diagnosis of aggressive PTCL diagnosed and treated at our institution between 2010 to 2016. All patients included were treated with chemotherapy with a curative intent. The RDW was collected from the hemogram of PTCL patients at diagnosis. RDW-CV >14% and RDW-SD >49 fL were our cut-offs for the categorical analysis. We fitted univariate and multivariate Cox proportional hazard regression models for overall survival (OS). P<0.05 were considered statistically significant. Results: A total of 101 patients with de novo aggressive PTCL were included. This study included 53 patients (52%) with PTCL, not otherwise specified, 16 patients (16%) with extranodal natural killer/T-cell lymphoma,15 (15%) with adult T-cell lymphoma/leukemia,14 patients (14%) with anaplastic large cell lymphoma, 2 patients (2%) with angioimmunoblastic T-cell lymphoma, and 1 patient (1%) with enteropathy-associated T-cell lymphoma. Median age was 57 with a male predominance (69%). Clinically, 45 patients (45%) were 60 years or older, 41 patients (34%) had ECOG ≥2, 63 patients (65%) had increased LDH levels, 68 patients (67%) had > 1 extranodal site, 65 patients (64%) had stage III/IV disease, and 55 patients (54%) had B symptoms. RDW-CV was >14% in 64 patients (64%). RDW-SD was >49 fL in 35 patients (35%). 38% of patients received CHOEP, 29% received CHOP, and 33% received other regimens. The overall response rate was 68%; 50% had a complete response and 18% had a partial response. At 5 years, median overall survival (OS) was 46%. In the univariate Cox regression analysis, ECOG ≥2 (p<0.001), high RDW-CV >14% (p<0.001), high RDW-SD >49 fL (p=0.007) and high neutrophil-lymphocyte ratio (NLR >4) (p=0.002) were associated with a worse survival. In the multivariate Cox regression analyses, ECOG ≥2 (HR 3.1 CI 1.6-6.0; p=0.001) and high RDW-CV >14% (HR 2.7, 95% CI 1.2-6.0; p=0.01) were independent predictors of poor survival. RDW-CV was an independent factor of survival when was compared with IPI and NCCN-IPI score. Conclusions: RDW is an independent marker for adverse prognosis in aggressive patients with aggressive PTCL treated with curative intent. Figure. Figure. Disclosures Castillo: Genentech: Consultancy; Pharmacyclics: Consultancy, Research Funding; Janssen: Consultancy, Research Funding; Beigene: Consultancy, Research Funding; Abbvie: Consultancy, Research Funding; Millennium: Research Funding.

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Primary T-Cell Lymphoma of The Testis Presenting In A Young Adult

Health Sciences ◽

10.15342/hs.2020.214 ◽

2020 ◽

Vol 2020 ◽

Author(s):

MOUNIA BENDARI ◽

Wafaa Matrane ◽

Maryam Qachouh ◽

Asmaa Quessar ◽

Nisrine Khoubila

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Clinical Stage ◽

T Cell Lymphoma ◽

Second Line ◽

Peripheral T Cell Lymphoma ◽

Not Otherwise Specified ◽

Ann Arbor ◽

Prophylactic Chemotherapy ◽

Line Chemotherapy

We report the case of a 40-year-old male presented with a painless right testicular swelling. Right radical orchidectomy was performed. The pathological diagnosis was peripheral T-Cell lymphoma-not otherwise specified (PTCL-NOS). According to Ann Arbor staging, the initial clinical stage was IEa. Treating him with four courses of the CHOEP protocol and intrathecal prophylactic chemotherapy was unsuccessful; with the appearance of orbital infiltration and a loco-regional extension. Although the patient started a second line chemotherapy, he unfortunately succumbed to death.

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Fusion transcripts FYN-TRAF3IP2 and KHDRBS1-LCK hijack T cell receptor signaling in peripheral T-cell lymphoma, not otherwise specified

Nature Communications ◽

10.1038/s41467-021-24037-4 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Koen Debackere ◽

Lukas Marcelis ◽

Sofie Demeyer ◽

Marlies Vanden Bempt ◽

Nicole Mentens ◽

...

Keyword(s):

T Cell ◽

T Cell Receptor ◽

Cell Lymphoma ◽

Ex Vivo ◽

Cell Receptor ◽

T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Fusion Transcripts ◽

Not Otherwise Specified

AbstractPeripheral T-cell lymphoma (PTCL) is a heterogeneous group of non-Hodgkin lymphomas with poor prognosis. Up to 30% of PTCL lack distinctive features and are classified as PTCL, not otherwise specified (PTCL-NOS). To further improve our understanding of the genetic landscape and biology of PTCL-NOS, we perform RNA-sequencing of 18 cases and validate results in an independent cohort of 37 PTCL cases. We identify FYN-TRAF3IP2, KHDRBS1-LCK and SIN3A-FOXO1 as new in-frame fusion transcripts, with FYN-TRAF3IP2 as a recurrent fusion detected in 8 of 55 cases. Using ex vivo and in vivo experiments, we demonstrate that FYN-TRAF3IP2 and KHDRBS1-LCK activate signaling pathways downstream of the T cell receptor (TCR) complex and confer therapeutic vulnerability to clinically available drugs.

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