scholarly journals Clinical Features and Treatment Outcomes of Angioimmunoblastic T-Cell Lymphoma: An Analysis from a Nationwide Multicenter Registry, Thailand

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 5064-5064
Author(s):  
Chittima Sirijerachai ◽  
Kanchana Chansung ◽  
Arnuparp Lekhakula ◽  
Jakrawadee Julamanee ◽  
Kitsada Wudhikarn ◽  
...  

Abstract OBJECTIVES: To analyze clinical features, treatment outcomes in Thai patients with angioimmunoblastic T cell lymphoma (AITL). PATIENTS AND METHODS: From a nationwide multicenter registry of 4,056 NHL patients in Thailand between 2007 and 2014, there were a total of 54 angioimmunoblastic T cell lymphoma (AITL) patients. The clinical features and treatment outcomes were analyzed. RESULTS: There were a total of 54 cases accounted to the prevalence of 1.2 % of NHL and 12% of peripheral T cell lymphoma. The median age was 59 years (range 35-81). Male: female was 1.5:1. Seventy-eight percent of patients had advanced disease (stage III, IV), 69% had B symptoms, 28% had poor performance status (ECOG > 2) and 61% had elevated serum LDH level. Extranodal involvement was 56 %; the most common sites of which were bone marrow (30%) and liver (19 %). Thirty-five percent of the patients had IPI score > 2 and 7% had PIT >2. Eighty-one percent of patients were treated with chemotherapy of which CHOP/CHOP-like was the main regimen. Of the 43 evaluable patients receiving chemotherapy, complete remission was achieved in 41.9%. Forty-four percent of patients with complete remission had disease progression. With the median follow-up time of 65 months, the 5-year overall survival was comparable to peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), (28% vs. 37%, p=0.7). On multivariate analysis, response to treatment (at least PR) was associated with better outcome (HR 0.13, 95%CI 0.05-0.33, p= 0.000). Patients with PIT > 2 or B symptoms trended to have inferior survival outcome, although statistical significant was not achieve (HR 3.2, 95%CI 0.36-27.95, p=0.3; HR 2.3, 95%CI 0.78-7.31, p=0.13). CONCLUSIONS: The prevalence of Thai patients with AITL was much less than data reported from the international T-cell lymphoma project (18% of T-NHLs) (Vose et al, JCO2008;26:4124-30). The long-term survival was not inferior to patients with PTCL, NOS. Disclosures Khuhapinant: Roche: Honoraria.

2007 ◽  
Vol 48 (4) ◽  
pp. 716-722 ◽  
Author(s):  
Byeong-Bae Park ◽  
Baek-Yeol Ryoo ◽  
Jae H. Lee ◽  
Hyuck Kwon ◽  
Sung H. Yang ◽  
...  

2010 ◽  
Vol 28 (15_suppl) ◽  
pp. 8048-8048
Author(s):  
B. Pro ◽  
G. F. Perini ◽  
L. Feng ◽  
J. E. Romaguera ◽  
M. A. Rodriguez ◽  
...  

2020 ◽  
Vol 12 ◽  
pp. 175883592092382 ◽  
Author(s):  
Yuanyuan Sun ◽  
Ling Li ◽  
Xin Li ◽  
Lei Zhang ◽  
Xinhua Wang ◽  
...  

Aim: To compare the outcomes of GDPT [gemcitabine (G), cisplatin (D), prednisone (P), thalidomide (T)] versus CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) in treating newly diagnosed PTCL (peripheral T-cell lymphoma). Methods: An open-label prospective clinical trial with 153 newly diagnosed PTCL patients conducted between January 2010 and December 2018 was designed. Patients were randomly assigned to the GDPT (77 cases) and CHOP (76 cases) groups. Patients in each group were further divided into four subgroups: PTCL, not otherwise specified (PTCL-NOS); anaplastic large cell lymphoma (ALCL), angioimmunoblastic T cell lymphoma (AITL), and other types subgroup, in accordance with pathological patterns. Based on expression of RRM1, TOP2A, TUBB3, and ERCC1, patients were divided into groups with high and low gene expression levels. Clinical characteristics, side effects, efficacy, progression-free survival (PFS), and overall survival (OS) were compared. Results: There were no significant differences in the basic clinical features or side effects between the GDPT and CHOP groups. The overall response rate (ORR) of the GDPT group was better than that of the CHOP group (66.3% versus 50.0%, p = 0.042), as was the complete remission (CR) rate (42.9% versus 27.6%, p = 0.049). Patients in the GDPT group had a longer PFS and OS than the CHOP group. The 4-year PFS and OS rates in the GDPT group were both superior to those in the CHOP group (63.6% versus 53.0% for PFS, p = 0.035; 66.8% versus 53.6% for OS, p = 0.039). In the GDPT group, the difference in CR between the four subgroups was statistically significant ( p = 0.046). In the CHOP group, differences in both CR and ORR among the four subgroups were statistically significant ( p < 0.001 and p = 0.005, respectively). There were also statistically significant differences in CR between patients treated with CHOP and GDPT in the PTCL-NOS subgroup, AITL subgroup, and the other types subgroup ( p = 0.015; p = 0.003; p = 0.005, respectively). The data also showed a significant difference in OS among the four subgroups within the GDPT group ( p = 0.001). The OS of AITL was shorter than that of the other three subgroups. Four subgroups of CHOP showed a significant difference in PFS ( p = 0.019). There was no statistical association between responses and the gene expression levels of RRM1, ERCC1, TUBB3, and TOP2A. Conclusion: The GDPT group had better response rates and prolonged patient PFS and OS. As a promising new regimen, GDPT is expected to become the first-line therapy for PTCL. New agents should be applied to patients who do not achieve good responses with previous treatment, such as those diagnosed with angioimmunoblastic T cell lymphoma. Trial registration: This open randomized prospective clinical trial was registered at ClinicalTrials.gov (NCT01664975).


Blood ◽  
2007 ◽  
Vol 109 (11) ◽  
pp. 4952-4963 ◽  
Author(s):  
Laurence de Leval ◽  
David S. Rickman ◽  
Caroline Thielen ◽  
Aurélien de Reynies ◽  
Yen-Lin Huang ◽  
...  

Abstract The molecular alterations underlying the pathogenesis of angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma, unspecified (PTCL-u) are largely unknown. In order to characterize the ontogeny and molecular differences between both entities, a series of AITLs (n = 18) and PTCLs-u (n = 16) was analyzed using gene expression profiling. Unsupervised clustering correlated with the pathological classification and with CD30 expression in PTCL-u. The molecular profile of AITLs was characterized by a strong microenvironment imprint (overexpression of B-cell– and follicular dendritic cell–related genes, chemokines, and genes related to extracellular matrix and vascular biology), and overexpression of several genes characteristic of normal follicular helper T (TFH) cells (CXCL13, BCL6, PDCD1, CD40L, NFATC1). By gene set enrichment analysis, the AITL molecular signature was significantly enriched in published TFH-specific genes. The enrichment was higher for sorted AITL cells than for tissue samples. Overexpression of several TFH genes was validated by immunohistochemistry in AITLs. A few cases with molecular TFH-like features were identified among CD30− PTCLs-u. Our findings strongly support that TFH cells represent the normal counterpart of AITL, and suggest that the AITL spectrum may be wider than suspected, as a subset of CD30− PTCLs-u may derive from or be related to AITL.


Blood ◽  
2010 ◽  
Vol 115 (5) ◽  
pp. 1026-1036 ◽  
Author(s):  
Javeed Iqbal ◽  
Dennis D. Weisenburger ◽  
Timothy C. Greiner ◽  
Julie M. Vose ◽  
Timothy McKeithan ◽  
...  

Abstract Peripheral T-cell lymphoma (PTCL) is often challenging to diagnose and classify. Gene expression profiling was performed on 144 cases of PTCL and natural killer cell lymphoma and robust molecular classifiers were constructed for angioimmunoblastic T-cell lymphoma (AITL), anaplastic lymphoma kinase-positive (ALK+) anaplastic large-cell lymphoma (ALCL), and adult T-cell leukemia/lymphoma. PTCL-unclassifiable was molecularly heterogeneous, but we were able to identify a molecular subgroup with features of cytotoxic T lymphocytes and a poor survival compared with the remaining PTCL–not otherwise specified cases. Many of the pathologic features and substantial components of the molecular signature of AITL are contributed by the follicular dendritic cells, B-cell, and other stromal components. The expression of Th17-associated molecules in ALK+ ALCL was noted and may represent aberrant activation of Th17-cell differentiation by abnormal cytokine secretion. Adult T-cell leukemia/lymphoma has a homogeneous molecular signature demonstrating high expression of human T-lymphotropic virus type 1–induced genes. These classifiers reflect the biology of the tumor cells as well as their microenvironment. We also constructed a molecular prognosticator for AITL that appears to be largely related to the microenvironmental signature, and the high expression of 2 immunosuppressive signatures are associated with poor outcome. Oncogenic pathways and tumor-host interactions also were identified, and these findings may lead to better therapies and outcome in the future.


Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4035-4035
Author(s):  
Sang Eun Yoon ◽  
Seok Jin Kim ◽  
Tsai-Yun Chen ◽  
Yong Park ◽  
Li Mei Poon ◽  
...  

Introduction T-cell lymphoma is a group of heterogeneous diseases with various clinical behaviors and treatment outcomes, representing 10-15% of non-Hodgkin lymphomas. Owing to its rarity and heterogeneity, the standard treatment approach for T-cell lymphoma is still not established. Accordingly, conventional chemotherapy regimens adapted from B-cell lymphoma treatment has been used for T-cell lymphoma. However, their outcome is still not satisfactory, and there are limited data representing the real-world situation in terms of clinical features and treatment outcomes. Given the incidence of T-cell lymphoma is relatively higher in Asian than Western countries; a comprehensive registry study focusing on Asian patients with T-cell lymphoma could be helpful for better understanding of T-cell lymphoma as well as the development of more effective treatment strategy. Methods We performed a multi-national, multi-center, prospective registry study for patients with T-cell lymphoma and enrolled patients between 01-March-2016 and 31-January-2019. All patients received chemotherapy with curative intent after diagnosis, and were pathologically diagnosed with T-cell lymphoma according to the 2008 World Health Organization classification of lymphoid neoplasms. Patients belonged to any one of following clinical situations could be enrolled: (1) newly diagnosed, treatment-naïve patients; (2) patients who started treatment or completed treatment; (3) relapsed or refractory patients. After we enrolled the planned number of patients (n = 500), we analyzed clinical features and treatment outcomes. Results Out of 500 patients enrolled from nine Asian countries (Korea, China, Taiwan, Singapore, Indonesia, Bangladeshi, Vietnam, Malaysia, and Philippines), 490 patients were analyzed because 10 patients with insufficient information were excluded. The median age was 59 years (range, 20-85), male patients (59%) were predominant compared to female patients (41%). Extranodal NK/T-cell lymphoma (ENKTL) was the most common (28%) and angioimmunoblastic T-cell lymphoma (AITL) was the second common (24%). Peripheral T-cell lymphoma, not-otherwise specified (PTCL-NOS, 20%) and ALK+/- anaplastic large cell lymphoma (ALCL, 16%) were also major subtypes of T-cell lymphoma. The proportion of stage IV was 40%, however, the distribution of stage was different between ENKTL and nodal T-cell lymphomas such as PTCL-NOS. The CHOP (Cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOP-like regimens accounted for the mainstay of primary treatment for nodal T-cell lymphoma whereas non-anthracycline-based chemotherapy regimens such as SMILE (steroid, methotrexate, ifosfamide, L-asparaginase, and etoposide) and GemOx-L (gemcitabine, oxaliplatin, and L-asparagainase) were mainly used for ENKTL. The overall survival of ENKTL was not significantly different from that of PTCL-NOS, AITL and ALK+/- ALCL. Conclusions Our study showed the distribution of T-cell lymphoma subtypes and tumor burdens at the time of diagnosis in Asian countries. Although clinical features of ENKTL are different from that of nodal T-cell lymphomas consisting of PTCL-NOS, AITL and ALK+/- ALCL, and the different types of treatment were used, survival outcome of patients were not significantly different. This finding might be associated with improved treatment outcomes of ENKTL compared to the past. However, considering a substantial number of patients experienced treatment failure in patients with PTCL-NOS as well as ENKTL, more effective treatment strategy should be warranted. Figure Disclosures Kim: F. Hoffmann-La Roche Ltd: Research Funding; Celltrion: Research Funding; Novartis: Research Funding; Donga: Research Funding; Kyowa-Kirin: Research Funding; Novartis: Research Funding; J + J: Research Funding.


Author(s):  
Xiaohan Gao ◽  
Jing Li ◽  
Lin Kang ◽  
Qiang Wei ◽  
Jie Li ◽  
...  

Angioimmunoblastic T-cell lymphoma (AITL) is a special subtype of peripheral T-cell lymphoma, Histopathological examination is the main basis for the diagnosis of the disease, but the diagnosis is challenging. The study has shown that combining immunohistochemistry and gene rearrangement can increase the diagnostic accuracy


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