scholarly journals Peripheral T-cell lymphoma: Molecular profiling recognizes subclasses and identifies prognostic markers

Author(s):  
Marta Rodríguez ◽  
Ruth Alonso-Alonso ◽  
Laura Tomás-Roca ◽  
Socorro Maria Rodriguez Pinilla ◽  
Rebeca Manso ◽  
...  

Peripheral T-cell lymphoma (PTCL) is a clinically aggressive disease, with a poor response to therapy and a low overall survival rate of around 30% after 5 years. We have analyzed a series of 105 cases with a diagnosis of PTCL using a customized NanoString platform that includes 208 genes associated with T-cell differentiation, oncogenes and tumor suppressor genes, deregulated pathways and stromal cell subpopulations. A comparative analysis of the various histological types of PTCL (angioimmunoblastic T-cell lymphoma, AITL; PTCL-with T follicular helper phenotype, PTCL-TFH; PTCL-not otherwise signified, PTCL-NOS) showed that specific sets of genes were associated with each of the diagnoses. These included TFH markers, cytotoxic markers and genes whose expression was a surrogate for specific cellular subpopulations, including follicular dendritic cells, mast cells and genes belonging to precise survival (NF-κB) and other pathways. Furthermore, the mutational profile was analyzed using a custom panel that targeted 62 genes in 76 cases distributed in AITL, PTCL-TFH and PTCL-NOS. The main differences between the three nodal PTCL classes involved the RHOAG17V mutations (p<0.0001), which were approximately twice as frequent in AITL (34.09%) as in PTCL-TFH (16.66%) cases, but were not detected in PTCL-NOS. A multivariate analysis identified gene sets that allowed the series of cases to be stratified into different risk groups. This study supports and validates the current division of PTCL into these three categories, identifies sets of markers that can be used for a more precise diagnosis, and recognizes the expression of B-cell genes as an IPI-independent prognostic factor for AITL.

1984 ◽  
Vol 2 (7) ◽  
pp. 788-798 ◽  
Author(s):  
J P Greer ◽  
J C York ◽  
J B Cousar ◽  
R T Mitchell ◽  
J M Flexner ◽  
...  

Clinical and histopathologic material from 42 patients with peripheral T-cell lymphoma (PTCL) was reviewed. The median age was 63.5 years (range, 11-97 years). The male:female ratio was 2.8:1. Prior immune or lymphoproliferative diseases occurred in 36% of the patients. PTCL was advanced at presentation with B symptoms (67%), generalized adenopathy (69%), and stage III/IV disease (79%). Suspected lung or pleural involvement (21%), hepatomegaly (29%), and splenomegaly (43%) were common; marrow involvement was documented in 37% of the patients at presentation and in 51% of patients during the illness. Hypercalcemia and eosinophilia occurred in 19% and 29% of patients, respectively. Among patients receiving combination chemotherapy (BCOP, CHOP, BACOP, COMLA), eight (24%) of 33 achieved a complete remission and only four (12%) of 33 had a sustained complete remission. The median survival for PTCL was 11 months. Because of the poor response to standard therapy, clinical trials should identify cases of PTCL and evaluate newer regimens in this subset of aggressive lymphoma.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 31-32
Author(s):  
Hua Wang

Introduction Glasgow prognosis score (GPS) is an inflammation-based prognostic scoring system that combines clinical tumor markers including C-reactive protein and albumin. However, its role in predicting the prognosis of angioimmunoblastic T-cell lymphoma (AITL) is unclear. The prognosis index for peripheral T-cell lymphoma (PIT) has been widely used to evaluate the prognosis of various types of T-cell non-Hodgkin's lymphoma. In this study, we conducted a retrospective analysis involving 106 patients newly diagnosed with AITL to determine the prognostic value of GPS and compare with PIT model in patients with AITL. Methods A total of 106 patients newly diagnosed with AITL received standardized chemotherapy at Sun Yat-Sen University Cancer Center and the First Affiliated Hospital of Hainan University between July 2009 and October 2019 were enrolled in our study. The baseline patient characteristics were collected, including Eastern Cooperative Oncology Group performance status, B symptoms, lactate dehydrogenase level, albumin level (ALB), CRP level, extra nodal invasion, bone marrow involvement, and Ann Arbor stage . GPS was calculated according to serum CRP and ALB values. Patients with elevated CRP (>10 mg/L) and hypoalbuminemia (<35 g/L) were rated with a score of 2; patients with CRP >10 mg/L or hypoalbuminemia (<35 g/L) were rated with a score of 1; and patients with neither of these abnormalities were rated with a score of 0. Four variables were used to build up PIT score: age (≤60 versus >60), performance status (ECOG ≤1 versus >2), LDH level (low versus high) and BM involvement (negative versus positive). Depending on the number of adverse prognostic factors (0, 1, 2 or ≥3), patients were classified into low-(0,1) or high-risk (2, ≥3) groups, respectively. Progression-free survival (PFS) and overall survival (OS) rates were the primary endpoints of this study. Results Patients were divided into three groups by GPS: GPS=0, GPS=1 and GPS=2. Among them, 74 (69.8%), 22 (20.8%), and 10 (9.40%) received cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP or similar CHOP regimens); etoposide, doxorubicin, vincristine, cyclo-phosphamide and prednisone (EPOCH); gemcitabine and oxaliplatin (GEMOX) regimens, respectively. The results showed that the survival outcomes among the three groups were significantly different. The 5-year PFS rate (61.0% vs. 38.7% vs.0.00%; P<0.001) and 5-year OS rate (76.0% vs. 43.4% vs. 0.00%; P<0.001) were significantly better in the patients with GPS=0 than in those with GPS=1 and GPS=2. The OS and PFS of the patients with GPS=1 were significantly lower than those of patients with GPS=0 (P=0.003, 0.007). In addition, the OS of patients with GPS=1 was significantly better than that of patients with GPS=2 (P=0.021). However, no significant difference was found in their PFS (P=0.144). Furthermore, the proportion of patients with GPS=2 receiving GEMOX and EPOCH was significantly higher than that of patients with GPS=0 or GPS=1. (P=0.001). GEMOX and EPOCH regimens may produce better outcome than CHOP regimen in aggressive peripheral T-cell lymphoma. However, the long-term survival of patients with GPS=2 was significantly worse than those of patients with GPS=0 and GPS=1(P<0.001, =0.021), further confirming the beneficial effect of the GPS model in distinguishing the prognosis of patients. The GPS prognostic model also could effectively identify patients with poor prognosis in the low-risk group by PIT score. According to the PIT prognostic model, 54.7% of the patients were assigned to the low risk group. In those patients, approximately 28.3%, 44.3%, and 27.4% of patients exhibited GPS=0, 1, 2, respectively. In the low-risk group by PIT score, we get similar results of the GPS model mentioned above to evaluate the prognosis. Univariate and multivariate analysis were conducted to determine the prognostic value of GPS in AITL. The results showed that GPS≥1 (P<0.001) were an independent adverse prognostic factor for predicting PFS (95% CI: 1.668-6.626) and OS (95% CI: 2.214-14.686). Conclusion Our study reveals that GPS is an effective prognostic model for patients with AITL. It tends to balance the distribution of patients in the three risk groups and has better prognostic significance than PIT in low-risk groups. Further studies are needed to explain the underlying mechanism of the relationship between high GPS and low survival outcomes in AITL. Figure Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2812-2812
Author(s):  
Heather L. McArthur ◽  
Mukesh Chhanabhai ◽  
Gascoyne D. Randy ◽  
Connors M. Joseph ◽  
Savage J. Kerry

Abstract Background: Peripheral T-cell lymphoma, unspecified (PTCL-US) represents the largest subtype of PTCLs among Western populations. The prognosis of this heterogeneous group is poor with a 5-year overall survival (OS) of approximately 30%. The biological diversity of this subtype has prompted various attempts at refining clinical risk groups. For instance, the International Prognostic Index (IPI), first validated in DLBCL, has also been validated in PTCL-US in several studies. Recently, however, a new Prognostic Index for PTCL-US (PIT) has been proposed (Gallamini et al Blood103 (7): 2004). The purpose of this study was to apply the PIT to all patients with PTCL-US diagnosed and treated at the British Columbia Cancer Agency (BCCA) and to compare its prognostic utility with the IPI. Methods: The BCCA Lymphoid Cancer Database was screened to identify all patients over 18 y diagnosed with PTCL-US by the World Health Organization classification system between January 1981 and June 2004. Patients were excluded if the diagnosis occurred outside of British Columbia or if the pertinent prognostic information was incomplete. Five year OS estimates were calculated for each variable in both the IPI (age >60 y, LDH> normal, PS ≥2, Stage III/IV and >1 extranodal sites) and PIT models (age, LDH> normal, PS ≥2 and bone marrow involvement). Five year OS estimates were then calculated for IPI groups 1, 2 and 3 (0/1, 2/3 and 4/5 factors, respectively) as well as PIT groups 1 through 4 (0, 1, 2 and 3/4 factors, respectively). Results: Of the 134 patients identified, the median age was 61 y and the male to female ratio was 1.6. The predominant sites of extranodal involvement were bone marrow (10%), bone (6%), liver (4%), skin (4%), soft tissue (4%), lung (3%) and GI (3%). As demonstrated in figure 1, 5 year OS estimates were 73%, 24% and 22% for IPI groups 1, 2 and 3 (p=10−4), respectively and 76%, 35%, 25% and 19% for PIT groups 1 through 4, respectively (p=10−4 ). There was no difference between the prognostic models in the subset of 107 (80%) patients treated with CHOP-based chemotherapy. Conclusions: Although the PIT was as effective as the IPI in defining clinical risk groups among PTCL-US patients, it did not provide any additional information in this study. Since the IPI is a well-established, familiar tool with similar prognostic capacity, it is reasonable to continue to apply this model in PTCL-US. With ongoing advances in gene expression profiling, it is likely that new biological models will emerge which may be used in combination with the IPI to better prognosticate this heterogeneous group. Figure Figure


1988 ◽  
Vol 117 (4_Suppl) ◽  
pp. S245
Author(s):  
H. DÖHNER ◽  
M. HÜFNER ◽  
J. SCHMIDT ◽  
P. MÖLLER ◽  
A.D. Ho

2020 ◽  
Vol 2020 ◽  
Author(s):  
MOUNIA BENDARI ◽  
Wafaa Matrane ◽  
Maryam Qachouh ◽  
Asmaa Quessar ◽  
Nisrine Khoubila

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.


2019 ◽  
Vol 81 (1) ◽  
pp. 22-25
Author(s):  
Haruka KOBASHI ◽  
Issei KIDO ◽  
Hideki NAKAJIMA ◽  
Shigetoshi SANO

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