Clinicopathologic Analysis of Primary Gastrointestinal T-Cell Lymphoma with a Special Reference to Intestinal and Gastric Type in Japan.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1782-1782
Author(s):  
Hideko Yamamoto ◽  
Keitaro Matsuo ◽  
Hidemi Goto ◽  
Shigeo Nakamura
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Cell Leukemia ◽  
Clinical Factors ◽  
Intestinal Lymphoma ◽  
Total Protein Level ◽  
T Cell Lymphomas

Abstract Primary gastrointestinal T-cell lymphomas (PGITL) are very rare and heterogeneous diseases with poor prognosis. Their incidence and pathogenesis may vary with geographical locations. In this study, we aimed to define clinicopathologic and prognostic features of PGITL in Japan, non endemic area for celiac disease, with compared to those in Western countries. 79 patients with PGITL (41 gastric and 38 intestinal lymphoma cases) were enrolled. Epstein-Barr virus associated NK-/T-cell lymphoma and human T-cell leukemia virus type 1 associated adult T-cell leukemia/lymphoma cases were excluded from this study. Our intestinal cases (63%) were histopathologically characterized by monomorphic small-to medium-sized tumor cells, which appeared to be contrasted with higher incidence of pleomorphic appearance among Western patients, and by frequent expression of CD8, CD56, and cytotoxic molecules (CM) of those cells, which is consistent with immunohistochemical features of Western cases. Our gastric cases were unique in showing a wide range of histologic and immunophenotypical characteristics with a significantly higher inclusion of non-cytotoxic lymphoma than intestinal cases (33% vs. 9%, P=.0299). Approximately 62% of patients also showed pleomorphic appearance most frequently with CD4+CD8− and CD4–CD8-phenotype. In comparison with gastric patients, intestinal ones were significantly associated with several clinical factors to indicate poor prognosis such as poorer performance status (P=.0232), lower total protein level (P=.0018), and lower serum albumin level (P=.0303). Overall survival of intestinal cases were significantly inferior to that of gastric patients (P = 0.026), while no statistically significant differences were found in survival between CM-positive PGITL cases and –negative ones (P = 0.6). In univariate analysis, the factors that turned out to correlate significantly with survival were total protein level (<6.0g/dL) (HR, 3.696; 95% CI, 1.671 to 8.171; P=.0012), platelet level (<10.0 /μl) (HR, 10.706; 95% CI, 2.713 to 42.244; P=.0007), a higher than normal serum lactate dehydrogenase (LDH) level (HR, 2.574; 95% CI, 1.266 to5.231; P=.009), direct invasion or gastrointestinal perforation (HR, 2.079; 95% CI, 1.013 to 4.267; P=.046), bone marrow involvement of the disease (HR, 3.134; 95% CI, 1.136 to 8.651; P=.0274), performance status equal to or more than 2 (HR, 3.988; 95% CI, 1.763 to 8.882; P=.0007), the International Prognostic Index (IPI) more than low-intermediate (HR, 3.317; 95% CI, 1.304 to 8.441; P=.0119), Prognostic Index for T-cell lymphoma (PIT) scores more than 2 (HR, 8.166; 95% CI, 2.574 to25.904; P=.004), CD8 positivity (HR, 2.041; 95% CI, 1.032 to 4.037; P=.0404), and intestinal case (HR, 2.955; 95% CI, 1.493 to 5.849; P=.0019). Multivariate analysis with individual factors confirmed that PIT scores was a significant (HR, 6.622; 95% CI, 1.108 to 39.566; P=.0382) and platelet level (<10.0 /μl) was a marginally significant (HR, 5.343; 95% CI, .837 to 34.116; P=.0765) prognostic factors independent from other clinical factors. In conclusion, Japanese intestinal lymphoma patients were in keeping with characteristics of Enteropathy type T-cell lymphoma other than an association with celiac disease and monomorphic histology. However, gastric T-cell lymphomas were distinct from intestinal ones, and were separately considered.

International T-Cell Lymphoma Classification Project: Angioimmunoblastic T-Cell Lymphoma.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 2052-2052
Author(s):  
Thomas Ruediger ◽  
Bertrand Coiffier ◽  
Dennis D. Weisenburger ◽  
Massimo Federico
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Stage Disease ◽  
Classification Project ◽  
And Performance ◽  
Generalized Lymphadenopathy

Abstract Peripheral T-cell lymphomas are rare diseases: therefore, the International T-cell Lymphoma Project was undertaken to compare lymphomas at different sites throughout North America, Asia and Europe. Within this project, 243 angioimmunoblastic T-cell lymphomas (AILTs) were diagnosed which made up 21% of all peripheral T-cell lymphoma (PTCL). At presentation, generalized lymphadenopathy was noted in 76% of the patients. Interestingly, three patients presented with extranodal disease only. Among the skin symptoms, erythroderma was the most frequent (21% of patients). Hemolytic anemia was seen in 13% and dysproteinemia occurred in 50%, and among these monoclonal serum immunoglobulin was seen in 8% of the patients. Anemia, hypergammaglobulinemia and elevated LDH were significantly more frequent in AILT than in PTCL-unspecified. Similarly, patients with AILT had a significantly higher frequency of high stage disease (89% of the patients were stage 3 or 4), as well as worse prognostic indices. Despite this, their 5-year overall (33%) and failure-free survivals (18%) were similar to patients with PTCL-unspecified. Treatment was usually administered in combination with anthracycline. A few factors at presentation were prognostic for outcome, including the PIT (prognostic index for T-cell lymphoma; Gallamini et al.: Blood. 2004 Apr 1;103(7):2474–9), age, B-symptoms and performance status. The IPI, however, was not prognostic. Controlling for the PIT, a platelet count <150.000 μl was prognostic for overall survival whereas B-symptoms were prognostic for failure-free survival. In conclusion, AILT is an aggressive disease for which the optimum treatment has not yet been developed.

scholarly journals Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters

Blood ◽  
2012 ◽  
Vol 120 (7) ◽  
pp. 1466-1469 ◽  
Author(s):  
François Lemonnier ◽  
Lucile Couronné ◽  
Marie Parrens ◽  
Jean-Philippe Jaïs ◽  
Marion Travert ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
Prognostic Index ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas ◽  
Stage Disease ◽  
Peripheral T Cell Lymphomas

Abstract Inactivating mutations of the Ten-Eleven Translocation 2 (TET2) gene were first identified in myeloid malignancies and more recently in peripheral T-cell lymphomas (PTCLs). In the present study, we investigated the presence of TET2 coding sequence mutations and their clinical relevance in a large cohort of 190 PTCL patients. TET2 mutations were identified in 40 of 86 (47%) cases of angioimmunoblastic T-cell lymphoma (AITL) and in 22 of 58 (38%) cases of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), but were absent in all other PTCL entities, with the exception of 2 of 10 cases of enteropathy-associated T-cell lymphoma. Among PTCL-NOS, a heterogeneous group of lymphoma-comprising cases likely to derive from Th follicular (TFH) cells similarly to AITL, TET2 mutations were more frequent when PTCL-NOS expressed TFH markers and/or had features reminiscent of AITL (58% vs 24%, P = .01). In the AITL and PTCL-NOS subgroups, TET2 mutations were associated with advanced-stage disease, thrombocytopenia, high International Prognostic Index scores, and a shorter progression-free survival.

scholarly journals A Clinical Prognostic Index for Angioimmunoblastic T Cell Lymphoma in an Asian Multi-Centre Study

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 2453-2453
Author(s):  
Esther Chang ◽  
Valerie Shiwen Yang ◽  
Shin Yeu Ong ◽  
Hilda Kang ◽  
Ya Hwee Tan ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
White Cell Count ◽  
Performance Status ◽  
Bone Marrow Involvement ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Ann Arbor ◽  
Progression Of Disease ◽  
Advanced Stages

Abstract Background and aims: Angioimmunoblastic T cell Lymphoma (AITL) is a subtype of peripheral T cell lymphoma that is generally felt to be aggressive and of poor prognosis. It is characterized as a lymphoma associated with inflammatory and immune conditions, typically seen in the older population and presenting at more advanced stages. The International T-Cell Lymphoma project recently reported a novel AITL score comprising of age, ECOG performance status, serum CRP level and serum B2-microglobulin level; the latter 2 variables suggesting a pro-inflammatory state. They also found that progression of disease within 24 months (POD24) to be strongly prognostic. In our Asian multicenter study, we aim to investigate the clinical prognostic factors affecting the outcomes of our AITL patients and attempt to identify a prognostic index that would be relevant to our Asian population. Methods: Patients who were consecutively diagnosed with AITL and seen at National Cancer Centre Singapore and Singapore General Hospital between June 1999 and Dec 2019 were retrospectively analyzed. Relevant demographical and clinical characteristics were collected. Median duration of follow up was 19.7 months. Outcomes of interest were that of 5-year overall survival (OS) and 5-year progression free survival (PFS). POD24 as defined by progression of disease within 24 months was also analyzed for its prognostic significance. Kaplan meier curves were plotted to estimate survival for each individual clinical parameter. Parameters found to be significant on univariate analysis were subsequently used in generation of multivariate cox regression models. Results: A total of 166 patients were included. The median age was 62.1 years. The majority of our patients (92.8%) had good performance status of ECOG 0-1 and 77.7% presented at advanced stages (Ann Arbor stage 3-4). The median PFS and OS was 1.5 years and 5.5 years respectively. The estimated 5-year PFS and OS was 40% and 53% respectively. Univariate analyses of various parameters were significant for age &gt;60 years, presence of B symptoms, ECOG &gt;1, Ann Arbor stage 3-4, bone marrow involvement, elevated serum lactate dehydrogenase &gt; upper limit normal, elevated total white cell count &gt; 12 x 10 9/L and low platelet count &lt; 150,000/mm 3. In the multivariate analyses, age &gt;60 years, bone marrow involvement, elevated total white cell count and low platelet count were associated with poorer PFS and OS. This allowed for a prognostic index (AITL-PI) differentiating patients into low (0-1 factors, n=62), moderate (2 factors, n=54) and high (3-4 factors, n=48) risk subgroups with 5-year OS of 83%, 41% and 26% respectively. The corresponding 5-year PFS of the low, moderate and high risk subgroups are 69%, 29% and 14%. Likewise, POD24 proved to be strongly prognostic in our cohort as patients with POD24 had a 5-year OS of 24%, whereas those without POD24 had a 5-year OS of 90% (p&lt;0.0001). Conclusion: We validated POD24 as a strong prognostic factor. Our AITL-PI was able to identify 3 different subgroups of patients with disparate outcomes based on their presenting clinical parameters. Further work can be done to elucidate if there are unique pathological or molecular characteristics in these individual risk groups that can further guide treatment choices. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

Multicenter, phase II study of bendamustine in refractory or relapsed T-cell lymphoma: The BENTLY trial.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 8026-8026 ◽  
Author(s):  
Remy Gressin ◽  
Gandhi Laurent Damaj ◽  
Kamal Bouabdallah ◽  
Guillaume Cartron ◽  
B Choufi ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
International Prognostic Index ◽  
Single Agent ◽  
Prognostic Index ◽  
Progression Free Survival ◽  
Complete Response ◽  
T Cell Lymphoma ◽  
Previous Response ◽  
T Cell Lymphomas

8026 Background: T-cell lymphomas have a poor prognosis with few options of effective treatment. This study determined the efficacy and safety of bendamustine as a single agent in the treatment of refractory or relapsed T-cell lymphomas. Methods: Patients with histologically confirmed peripheral T-cell lymphoma (PTCL) or cutaneous T-cell lymphoma (CTCL), who had previously received at least one line of chemotherapy were selected. Bendamustine was administered IV at the dosage of 120 mg/m2 on days 1 and 2 every 3 weeks, for 6 cycles. Treatment response was assessed using the IWC for non-Hodgkin's lymphoma. The primary end point was overall response rate (ORR). Secondary end points were duration of response (DoR), progression-free survival (PFS), and overall survival (OS), NCT00959686. Results: Twenty two female and 38 male were included. The median age was 66 years with more 1/4 of them > 75. Histology was predominantly angio-immunoblastic lymphadenopathy (n=32) and PTCL-nos (n=23). The median previous line of chemotherapy was 1 (1-3). Nearly one half (45%) of the patients was refractory to the last previous chemotherapy and the median duration of the best previous response was 6.6 (1.5-67) months. The disease was disseminated in the majority of case (87%) and the international prognostic index (IPI) was high (3–5) in 68% of the patients. Twenty patients (33%) received less than 3 cycles of bendamustine. The major reason for early discontinuation was disease progression. In the Intent-To-Treat (ITT) population, the best ORR was 50%, including complete response (CR) in 28% and partial response (PR) in 22 %. Bendamustine showed a consistency in the efficacy as a function of major disease characteristics. The median values for DoR, PFS and OS were 3.5, 4 and 6 months respectively. The most frequent grade 3/4 AEs were neutropenia (30%), thrombocytopenia (24%) and infections (20%). Conclusions: Bendamustine is active in high risk refractory and relapsed T-cell lymphoma with manageable toxicity.

Serum Ferritin Level As a Prognostic Marker For Peripheral T-Cell Lymphoma.

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4326-4326
Author(s):  
Satoshi Koyama ◽  
Naoto Tomita ◽  
Reina Watanabe ◽  
Megumi Itabashi ◽  
Daisuke Ishibashi ◽  
...  
Keyword(s):  
T Cell ◽  
Serum Ferritin ◽  
Serum Ferritin Level ◽  
Cell Lymphoma ◽  
Ferritin Level ◽  
Performance Status ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Ann Arbor ◽  
Group 3

Abstract Background Peripheral T-cell lymphoma (PTCL) is known to have an aggressive clinical course and be associated with poor survival. The International Prognostic Index (IPI) score and the Prognostic Index for T-cell lymphoma (PIT) have been suggested as methods to predict the prognosis of PTCL. Ferritin, the iron storage protein, is associated with chronic inflammation. Although higher levels of serum ferritin are detected in many cancer patients, the significance of elevated serum ferritin as a prognostic factor for lymphoma has yet to be established. Thus, our retrospective study aimed to examine the prognostic value of serum ferritin levels in PTCL. Patients and Methods Serum ferritin levels were evaluated in 78 patients with PTCL, who were treated with anthracycline-containing regimens in 8 institutions affiliated to the Yokohama City University Hematology Group between 1998 and 2011. Fourteen patients received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP); 44 receivedpirarubicin, cyclophosphamide, vincristine, and prednisone (THP-COP); 3 received THP-COP followed by radiotherapy; 3 received up-front autologous peripheral blood stem cell transplantation; and 14 received THP-COP at 2-week intervals in a clinical trial. Results The study population comprised 50 male and 28 female patients with a median age of 64 years at the time of diagnosis (range, 16–83 years). With regard to the PTCL subtype, 39 patients had PTCL, not otherwise specified, and 39 had angioimmunoblastic T-cell lymphoma. Twelve patients had localized disease and 66 patients had advanced Ann Arbor stage lymphoma. Twenty-three patients had a poor Eastern Cooperative Oncology Group performance status (PS) of 2–4. B symptoms were present in 34 patients. Risk stratification according to the IPI was as follows: low risk, 9 patients; low–intermediate risk, 20 patients; high–intermediate (HI) risk, 30 patients; and high (H) risk, 19 patients. According to the PIT, 4 patients were categorized into group 1, 25 into, group 2 , 28 into, group 3, 21 into, as group 4. The median observation period for the surviving patients was 50 months. The median serum ferritin level was 183 ng/ml (range, 5–14,622 ng/ml). Factors associated with a poor overall survival (OS) in univariate analysis were HI and H risk status with regard to IPI (P = 0.024), assignment to group 3 or 4 with regard to PIT (P = 0.017), poor performance status (P< 0.001), and ferritin levels ≥ 300 ng/ml (P< 0.001). The 4-year OS rate of all 78 patients was 54%. The 4-year OS rate was poorer in patients with serum ferritin levels ≥300 ng/ml (n = 21) than in those with serum ferritin levels< 300ng/ml (n = 57; 22% vs. 65%; P< 0.001) (Figure). Multivariate analysis including each factor comprising the IPI (age, lactate dehydrogenase level, PS, Ann Arbor stage, and number of extranodal lesions), gender, bone marrow involvement, and serum ferritin level showed that poor PS (P = 0.002, relative risk [RR] 3.6) and a serum ferritin level ≥300 ng/ml (P = 0.014, RR 2.7) were independent risk factors for poor OS. Conclusion The serum ferritin level is a useful prognostic marker for PTCL. Disclosures: No relevant conflicts of interest to declare.

Report of a Phase II Study of Proteasome Inhibitor Bortezomib (Velcade) in Patients with Relapsed or Refractory T-Cell Lymphoma.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4764-4764
Author(s):  
Pier Luigi Zinzani ◽  
Monica Tani ◽  
Vittorio Stefoni ◽  
Lapo Alinari ◽  
Enrica Marchi ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Vital Role ◽  
T Cell Lymphoma ◽  
26S Proteasome ◽  
Cell Trafficking ◽  
Ecog Performance Status ◽  
T Cell Lymphomas ◽  
Cardiac Functions

Abstract The 26S proteasome plays a vital role in degrading regulatory proteins such as p53, p21, p27, NF-kB, I-kB, and bcl-2, that govern cell cycle, transcription factors activation, apoptosis and cell trafficking. Thus, the mechanisms by which bortezomib elicits its antitumor activity may vary among tumor types, and the extent to which each affected pathway is critical to the inhibition of tumor growth could also differ. The aim of this study was to determine the efficacy and toxicity of bortezomib in previously pretreated patients with peripheral T-cell lymphoma unspecified (PTCLU) and cutaneous T-cell lymphomas (CTCL). Each patient had to meet the following inclusion criteria to be enrolled in the study: histologically confirmed PTCLU or CTCL (according to REAL/WHO classification); any stage, any IPI, any bone marrow status; second or more relapse or refractory disease; age ≥ 18; ECOG performance status ≤ 2; Hb ≥ 10 g/dL, ANC ≥ 1.5x109/L and platelets ≥ 100 x 109/L; normal hepatic, renal and cardiac functions; and voluntary written informed consent. Bortezomib was given at 1.3 mg/m2 IV push on days 1, 4, 8 and 11 every 21 days. Restaging was done every 2 cycles. Patients were treated for up to a total of 6 cycles unless removed from study for failure to respond or toxicity. The response criteria were those recommended by NCI sponsored Working Group. A total of 30 patients will be enrolled; so far 10 patients entered and preliminary results of this trial will be presented.

scholarly journals Outcome of Allogeneic Transplantation for Mature T-cell Lymphomas: Impact of Donor Source and Disease Characteristics

2021 ◽  
Author(s):  
Mehdi Hamadani ◽  
Maud Ngoya ◽  
Anna Sureda ◽  
Qaiser Bashir ◽  
Carlos Alejandro Litovich ◽  
...  
Keyword(s):  
T Cell ◽  
Hematopoietic Cell Transplantation ◽  
Cell Lymphoma ◽  
Performance Status ◽  
Progression Free Survival ◽  
T Cell Lymphoma ◽  
Unrelated Donor ◽  
Marrow Graft ◽  
T Cell Lymphomas

Mature T-cell lymphomas constitute the most common indication of allogeneic hematopoietic cell transplantation (allo-HCT) in lymphomas. Large studies evaluating contemporary outcomes of allo-HCT in mature T-cell lymphomas, relative to commonly used donor sources are not available. Included in this registry study were adult patients who had undergone allo-HCT for anaplastic large cell lymphoma, angioimmunoblastic T-cell lymphoma (AITL), or peripheral T-cell lymphoma-NOS (PTCL-NOS) between 2008 and 2018. HCT platforms compared were post-transplant cyclophosphamide-based haploidentical (haplo-) HCT, matched sibling donor (MSD) HCT, matched unrelated donor HCT with in-vivo T-cell depletion (MUD TCD+), and MUD HCT without TCD (MUD TCD-). Co-primary endpoints were overall survival (OS) and progression-free survival (PFS); secondary endpoints included non-relapse mortality (NRM), and relapse/progression incidence (RI). 1942 patients were eligible (haplo-HCT 237; MSD 911; MUD-TCD+ 468; MUD TCD- 326). Cohorts were comparable for baseline characteristics except higher proportions of patients with decreased performance status (PS) and marrow graft recipients in the haplo-HCT group. On univariate and multivariate comparisons, OS and PFS, RI, and NRM were not significantly different between haplo-HCT, MSD, MUD-TCD+, and MUD-TCD- cohorts, with 3-year OS and PFS of 60%, 63%, 59%, and 64%; and 50%, 50%, 48%, and 52%, respectively. Significant predictors of inferior OS and PFS on multivariate analysis were active disease status at HCT and decreased PS. AITL was associated with significantly reduced relapse risk and better PFS compared to PTCL-NOS. Allo-HCT can provide durable PFS in patients with mature T-cell lymphoma. Outcomes of haplo-HCT were comparable to that of matched donor allo-HCT.

scholarly journals Extranodal NK/T-cell lymphoma, nasal type presenting as primary intestinal lymphoma vs intestinal T-cell lymphoma: A borderline diagnostic category in the revised WHO classification

2021 ◽  
Vol 25 ◽  
pp. 200534
Author(s):  
Brady E. Beltrán ◽  
Mario L. Marques-Piubelli ◽  
M. Pilar Quiñones ◽  
Esther Cotrina ◽  
Eugenio A. Palomino ◽  
...  
Keyword(s):  
T Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
Diagnostic Category ◽  
T Cell Lymphoma ◽  
Intestinal Lymphoma ◽  
Nasal Type ◽  
Nk T Cell
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