P1183: CENTRAL NERVOUS SYSTEM INVOLVEMENT AT DIAGNOSIS AND AT RELAPSE IN PATIENTS WITH PERIPHERAL T-CELL LYMPHOMA: A RETROSPECTIVE ANALYSIS OF THE “RETE EMATOLOGICA VENETA” (T-REV PROJECT).
Marcello RivaMaria Chiara TisiDavide FacchinelliGreta ScapinelloAlberto SchenaDario MarinoSilvia FinottoLeila RomanoMaurizio CavallariCarlo BorgheroRenato BassanMauro KramperaFilíppo GherlinzoniLivio TrentinAlberto TosettoPiero Maria StefaniFrancesco PiazzaCarlo Visco
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Topic: 19. Aggressive Non-Hodgkin lymphoma - Clinical Background: Peripheral T-cell lymphoma (PTCL), is a heterogeneous and rare entity (5-10% of all lymphomas). Central nervous system (CNS) involvement at diagnosis and at relapse in patients with PTCL ranged from 2-9%, with a well-known dismal prognosis. There are no clear recommendations for CNS prophylaxis in PTCL, with the exception of some entity. We retrospectively collected clinicopathologic and treatment data from 205 PTCL patients, diagnosed between 2000 and 2022, who underwent induction chemotherapy in six Italian centers. Aims: To characterize the rate and survival outcome of CNS involvement in newly diagnosed PTCL. Methods: Routine staging investigations and response was evaluated using the 2014 Lugano criteria. The diagnosis of PTCL was histologically confirmed and categorized according to the classification at the time of diagnosis. Patients with primary cutaneous T-cell lymphoma, T-cell lymphoblastic leukemia/lymphoma, T-cell prolymphocytic leukemia, adult T-cell leukemia/lymphoma and extranodal NK/T-cell lymphoma were excluded from this study. Only 6 patients received CNS prophylaxis, 3 with intrathecal therapy and 3 with high-dose methotrexate. Results: Baseline characteristics are shown in Figure 1. The median age of the entire population was 72 years (range 28-90), with a predominance of PTCL, not otherwise specified. With a median follow up of 24 months, 10 patients out of 205 had CNS disease: four patients (1.5%) had CNS involvement at initial diagnosis (1 ALCL-ALK+ and 3 ALCL-ALK-) and 6 patients (2.5%) experienced a CNS relapse (3 PTCL-NOS, 2 AITL, 1 EATL). At the end of first line treatment, ORR was 65% (with 55% CR and 10% PR) in patients without CNS involvement at diagnosis, and only one CR (25%) in patients with CNS involvement at diagnosis. Median overall survival (OS) of the entire population was 38 months; primary refractory and early relapsed were 69 (35%) and 33 (17%) patients, with a median OS of 8 and 21 months, respectively. Median OS for patients with CNS relapse were 8 months, and median OS for cases with CNS involvement at diagnosis were 13 months. As previously reported, patients relapsing to CNS were all in stage IV, had an elevated LDH, with extranodal involvement ≥ 1 site. CNS relapse had inferior survival, and it usually manifests as a terminal events in patients with refractory disease Summary/Conclusion: In this small multicenter retrospective analysys, we confirmed that patients with PTCL, developing a CNS relapse, had a higher burden of disease at baseline with a very poor prognosis. PTCL is a group of disease generally considered at low risk of CNS involvement at diagnosis and relapse; prophylaxis should be stratified according to histologic subtypes and risk factors. Further studies are needed to prevent and treat this condition, aiming to define a more individualized therapeutic approaches.Keywords: Peripheral T-cell lymphomaKeywords:
Peripheral T-cell lymphoma
Not Otherwise Specified
T-Cell Lymphoma
Peripheral T-cell lymphoma
Not Otherwise Specified
T-Cell Lymphoma
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To study the clinicopathologic features of various types of mature T-cell and natural killer (NK)/T-cell lymphoma in Guangdong, China, with respect to the 2008 WHO classification of lymphoid neoplasms.Eleven hundred and thirty-seven (1137) cases of mature T-cell or NK/T-cell lymphoma diagnosed during the period from 2002 to 2006 in Guangzhou area were retrieved. The clinical data, histologic features and immunohistochemical findings were reviewed by a panel of experienced hematopathologists. Additional immunostaining was performed if indicated. The cases were re-classified according to the 2008 WHO classification of lymphoid neoplasms.Nine hundred and sixty-three (963) cases fulfilled the diagnostic criteria of mature T-cell or NK/T-cell lymphoma and accounted for 20.1% of all cases of lymphoma encountered during the same period (963/4801). A predominance of extranodal involvement was noted in 644 cases (66.9%), while 319 cases (33.1%) showed mainly nodal disease. The prevalence of various lymphoma subtypes was as follows: peripheral T-cell lymphoma, unspecified (PTCL, NOS) 293 cases (30.4%), extranodal NK/T-cell lymphoma, nasal type 281 cases (29.2%), anaplastic large cell lymphoma (ALCL) 198 cases (20.6%), and angioimmunoblastic T-cell lymphoma (AILT) 46 cases (4.8%). The male-to-female ratio was 1.99. The median age of the patients was 44 years, with the peak age of PTCL, NOS, extranodal NK/T-cell lymphoma, nasal type and AILT being 55 to 64 years, 25 to 54 years and 65 to 74 years, respectively. ALK-positive ALCL occurred more frequently in young age, while the ALK-negative ALCL cases occurred mainly in the elderly.Extranodal lesions predominate in mature T-cell and NK/T-cell lymphomas occurring in Guangzhou area. There is a male predominance and the overall incidence shows no increasing trend with age of the patient. The peak age of various subtypes however varies. The most common subtype was PTCL, NOS, followed by extranodal NK/T-cell lymphoma, nasal type, ALCL and AILT. The relatively frequent occurrence of extranodal NK/T-cell lymphoma, nasal type in Guangdong area is likely associated with the high incidence of Epstein-Barr virus infection there.
Anaplastic large-cell lymphoma
T-Cell Lymphoma
Not Otherwise Specified
Peripheral T-cell lymphoma
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Objective To study the relationship of Epstein Barr virus (EBV) and T cell lymphoma.Methods Sixty cases of T cell lymphomas were examined for the presence of EBV using in situ hybridization for EBV encoded RNA (EBERs).Results EBERs were detected in tumor cells in 37(69.8%) of 53 cases with peripheral T cell lymphoma,but in none of seven cases of precusor T lymphoblastic lymphoma.The total detected EBERs were 37(61.6%) in 60 cases of T cell lymphomas.By Revised European Amercan Lymphoma(REAL) classification,EBERs were detected in 2/2 angioimmuno blastic T cell lymphoma,17/18 angiocentric lymphoma, 4/6 anaplastic large cell lymphoma and 14/27 peripheral T cell lymphoma,unspecified (51.9%).The frequency of EBERs among the extranodal peripheral T cell lymphoma was higher than the nodal ( P 0.01),there was no significent correlation with the sex,age and clinical stage.Conclusion This study indicated that high incidence of EBV was observed in peripheral T cell lymphoma,with predilection for angiocentric lymphoma and extranodal presentation.
Peripheral T-cell lymphoma
T-Cell Lymphoma
Anaplastic large-cell lymphoma
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Peripheral T-cell lymphoma
Anaplastic large-cell lymphoma
T-Cell Lymphoma
Not Otherwise Specified
Large cell
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To study the relationship of Epstein-Barr virus (EBV) and T cell lymphoma.Sixty cases of T cell lymphomas were examined for the presence of EBV using in situ hybridization for EBV encoded RNA (EBERs).EBERs were detected in tumor cells in 37(69.8%) of 53 cases with peripheral T cell lymphoma, but in none of seven cases of precursor T lymphoblastic lymphoma. The total detected EBERs were 37(61.6%) in 60 cases of T cell lymphomas. By Revised European-American Lymphoma(REAL) classification, EBERs were detected in 2/2 angioimmuno-blastic T cell lymphoma,17/18 angiocentric lymphoma, 4/6 anaplastic large cell lymphoma and 14/27 peripheral T cell lymphoma, unspecified (51.9%). The frequency of EBERs among the extranodal peripheral T cell lymphoma was higher than the nodal (P less than 0.01) there was no significant correlation with the sex, age and clinical stage.This study indicated that high incidence of EBV was observed in peripheral T cell lymphoma, with predilection for angiocentric lymphoma and extranodal presentation.
Peripheral T-cell lymphoma
T-Cell Lymphoma
Anaplastic large-cell lymphoma
Lymphoblastic lymphoma
Large cell
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Introduction: Composite lymphoma is defined as coexistence of two or more morphologically and phenotypically distinct lymphomas in the same anatomical site. Composite lymphoma may include combinations of Hodgkin lymphoma (HL) and B- or T-cell non-Hodgkin lymphoma (NHL); B-cell NHL and T-cell NHL; or two distinct B-cell or T-cell NHLs. The exact pathogenesis of composite lymphoma is unknown. Most cases demonstrate poor outcomes with a median survival of 12 months. The treatment is usually directed toward the higher-grade component. Case Report: Here, we report an extraordinarily rare case of a composite lymphoma composed of peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS) and follicular B-cell lymphoma (FBCL) coexisting in a single axillary lymph node in a 66-year-old female. Conclusion: The medical literature lacks significant information regarding this type of composite lymphoma, thus creating a challenge for management. Currently, only one other case of this type of composite lymphoma has been reported in the English medical literature, with this case reporting the first female patient.
Follicular lymphoma
T-Cell Lymphoma
B-cell lymphoma
Not Otherwise Specified
Peripheral T-cell lymphoma
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Anaplastic large-cell lymphoma
Peripheral T-cell lymphoma
T-Cell Lymphoma
International Prognostic Index
Not Otherwise Specified
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The association of Epstein-Barr virus (EBV) with human immunodeficiency virus-negative T-cell lymphoma was examined in 68 patients using the polymerase chain reaction (PCR) with DNA obtained from formalin-fixed paraffin-embedded tissues and an in situ hybridization technique. EBV-encoded RNA (EBER) was detected in 43 of 68 cases (63%) of peripheral T-cell lymphoma: in 100% (11 of 11 cases) of NK/T-cell lymphomas, 70% (14 of 20 cases) of angioimmunoblastic T-cell lymphomas (AILT) and 49% (18 of 37 cases) of other types of peripheral T-cell lymphoma. A positive band was also detected at high incidence (36 of 65 cases; 55%) in a PCR analysis using primers to detect the Bam HI-W fragment of EBV. In the immunohistochemical analysis using a monoclonal antibody to latent membrane protein 1 (LMP-1) of EBV, one of the EBV-encoded latent gene products, LMP-1, was found to be expressed in 13 of 64 cases (20%), but EBNA-2 was not expressed in all the cases examined (0 of 59 cases; 0%). The 5-yr survival rate was 28% for peripheral T-cell lymphomas overall, 0% for NK/T-cell lymphomas, 38% for AILTs and 28% for other types of peripheral T-cell lymphoma. The difference in the overall survival rate between NK/T-cell lymphoma and non-NK/T-cell lymphoma was significant (P = 0.0498 by Log-rank test). Among peripheral T-cell lymphoma patients overall, the group severely infected with EBV (EBER-ISH ++) had a lower 5-yr survival rate (8%) than the group slightly (EBER-ISH +) or not infected (38%; P = 0.0013).
Peripheral T-cell lymphoma
T-Cell Lymphoma
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Peripheral T cell lymphomas (PTCLs) are a heterogeneous group of non-Hodgkin's lymphomas (NHLs), with universally poor outcome. This study was undertaken to provide data on demographics and outcomes of patients with PTCL who underwent treatment in a single tertiary care centre in southern India.
Anaplastic large-cell lymphoma
Peripheral T-cell lymphoma
Not Otherwise Specified
T-Cell Lymphoma
Large cell
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Objective
To investigate the diagnosis and treatment of sequential diffuse large B-cell lymphoma (DLBCL) after peripheral T-cell lymphoma (PTCL).
Methods
A case with sequential DLBCL after PTCL was reported, and the characteristics and responses of this case were analyzed. The previous literature was reviewed in order to explain the mechanism and prognosis of such type of disease.
Results
This patient was diagnosed as PTCL not otherwise specified (PTCL-NOS) definitely, but after a period of treatment, DLBCL was developed as a second tumor. The characteristics and onset interval were just similar to those described in the literature, in which the mechanisms were mentioned as common effects of tumor cell, microenviroment and therapies. This patient got effects through the initial treatment, but considering the poor outcome by former researchers, the prognosis needed to be closely followed up.
Conclusion
Sequential development of EBV-unrelated DLBCL after PTCL-NOS is very rare, and the mechanism, therapy and prognosis need further investigation.
Key words:
Peripheral T-cell lymphoma, not otherwise specified; Lymphoma, large cell, diffuse; EB virus
Peripheral T-cell lymphoma
Not Otherwise Specified
T-Cell Lymphoma
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