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1.
Medicine (Baltimore) ; 99(2): e18650, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31914048

RESUMO

RATIONALE: Peripheral T cell lymphoma, coexisting with Castleman's disease (CD), is rarely seen in clinical practice and is not frequently reported in the literature. PATIENT CONCERNS: A 68-year-old female was admitted to our hospital for the first time due to "multiple lumps in the neck that progressively enlarged over 7 months". 1.5 years later, the patient returned to our hospital complaining of " difficulty breathing and purulent blood in the mouth for more than 20 days". DIAGNOSIS: The postoperative pathology from the (right) cervical lymph node biopsy confirmed the diagnosis of Castleman Disease (Vascular follicular type). 1.5 years after the diagnosis of CD, the patient developed secondary peripheral T cell lymphoma of unspecified type (PTCL-U). INTERVENTIONS: The patient received 5 courses of chemotherapy: 2 courses of CHOP, Chidamide combined with GemOx, GDP and Hyper CVAD Bregimen. OUTCOMES: After 3 courses of treatment, the curative effect was partly remitted (PR). The patient was discharged in a good condition and the follow-up was uneventful. LESSONS: The mechanism responsible for CD concurrent or secondary lymphoma is not clear. Epstein-Barr virus (EBV) infection may be the most common reason of CD and PTCL-U. Further understanding the mechanisms of the condition is needed.


Assuntos
Hiperplasia do Linfonodo Gigante/complicações , Linfoma de Células T Periférico/complicações , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Humanos , Linfoma de Células T Periférico/tratamento farmacológico
2.
Ann Hematol ; 99(1): 83-91, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31807859

RESUMO

This observational study aimed to evaluate the prognostic significance of interim and final 18F-fluorodeoxyglucose positron emission tomography-computed tomography (PET/CT) responses to upfront autologous stem cell transplantation (ASCT) in patients with peripheral T cell lymphomas (PTCLs). A total of 118 patients, from two independent institutions, with newly diagnosed PTCLs were enrolled, and 96 of them were evaluated. PET/CT was assessed at diagnosis, and during and after the primary treatment. Clinical outcomes of interim and final PET/CT were compared between transplanted and non-transplanted patients. The responses of PET/CT were assessed based on visual analysis using the Deauville five-point scale (5-PS). Clinicopathological features of transplanted patients (n = 37) were similar to those of non-transplanted patients (n = 59). After a median follow-up of 60.8 months, only final PET/CT response based on 5-PS was the independent prognostic factor of survival outcome (P < 0.001; HR 8.215; 95% C.I. 2.97-22.72) in multivariate analysis. Interim PET/CT response did not have a differential potential for predicting progression-free survival (PFS). In 59 patients, with score 1 or 2 in final PET/CT, the PFS rate was not significantly different between transplanted and non-transplanted patients (P = 0.970). Moreover, among the 37 patients with final PET/CT response score of 3-4, the PFS rate was equally poor in both transplanted and non-transplanted patients (P = 0.178). Final PET/CT assessment, based on 5-PS, was an important prognostic parameter for primary treatment of PTCLs, regardless of upfront ASCT. Interim PET/CT response could not be an indicator to determine the requirement for upfront ASCT.


Assuntos
Fluordesoxiglucose F18/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Linfoma de Células T Periférico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X , Adulto , Autoenxertos , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Linfoma de Células T Periférico/diagnóstico por imagem , Linfoma de Células T Periférico/mortalidade , Linfoma de Células T Periférico/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
3.
Hematol Oncol ; 38(1): 59-66, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31834627

RESUMO

Galectin-1 (Gal-1) has been associated with adverse prognosis in several cancers including lymphoma entities with CD30 expression. However, Gal-1 expression has not been systematically assessed in peripheral T-cell lymphomas (PTCL). Specimens from 169 nodal PTCL were assessed for intratumoural Gal-1 expression by immunohistochemistry. Overall survival (OS) in groups exhibiting high and low Gal-1 expression was compared in the cohort and in a subset analysis of CD30-positive PTCL only. Gal-1 expression was also correlated with biomarkers of the tumour microenvironment. No significant difference in OS based on Gal-1 expression was observed in the entire PTCL cohort. However, in the CD30-positive cohort, patients with high Gal-1 levels had significantly poorer outcome (5 years OS 10%, 95% confidence interval CI, 1-36) than their low Gal-1 counterparts (5 years OS 48%, 95% CI, 30-64, P = .021). In univariate analyses age 60 or younger, non-elevated lactate dehydrogenase (LDH), and performance score less than 2 correlated with superior survival but high Gal-1 expression significantly predicted adverse outcome at both univariate (HR 2.5, 95% CI, 1.1-5.7, P = .026) and multivariate levels (HR 3.2, 95% CI, 1.2-8.5, P = .017). Tumours with high Gal-1 had few cytotoxic T cells in the tumour microenvironment. High intratumoural Gal-1 expression before therapeutic intervention correlates with adverse outcome in nodal CD30+ , ALK- PTCL patients.


Assuntos
Quinase do Linfoma Anaplásico/metabolismo , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Galectina 1/metabolismo , Antígeno Ki-1/metabolismo , Linfoma Anaplásico de Células Grandes/mortalidade , Linfoma de Células T Periférico/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Seguimentos , Humanos , Linfoma Anaplásico de Células Grandes/tratamento farmacológico , Linfoma Anaplásico de Células Grandes/metabolismo , Linfoma Anaplásico de Células Grandes/patologia , Linfoma de Células T Periférico/tratamento farmacológico , Linfoma de Células T Periférico/metabolismo , Linfoma de Células T Periférico/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Linfócitos T Citotóxicos/imunologia , Microambiente Tumoral , Adulto Jovem
4.
Hematol Oncol ; 38(1): 51-58, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31872891

RESUMO

The peripheral T-cell lymphomas (PTCL) are rare and heterogeneous diseases characterized by an unfavorable prognosis. Chemotherapy is standard upfront treatment in most patients, but responses are short-lived with few FDA-approved "novel" agents available. We sought to define the impact of these novel agents as single agents or in clinical trials on the outcomes of patients with PTCL. From January 1994 to May 2019, adult patients with PTCL who were managed at our institution were included in this analysis. In addition to patients with incomplete data, those diagnosed with large granular lymphocytic leukemia and cutaneous T-cell lymphoma (CTCL) except for transformed mycosis fungoides were excluded. Statistical analyses were performed using SAS version 9.4. There were 219 patients included in the analysis. The median age at diagnosis was 56 years (range, 18-90 years). First line therapies mostly consisted of combination chemotherapy (75%). There was a statistical difference among patients who received chemotherapy, novel agents alone and in chemotherapy-free combinations, other, and no treatment (P < .0001). In patients who were treated with second line chemotherapy, novel agents alone and in combination without chemotherapy, or other, there was a still a survival benefit favoring novel agents (P = .0417). In the third line, there was no statistical difference among the three groups (P = .569). All patients who received novel therapies and underwent autologous stem cell transplant (autoSCT) achieved a complete response (CR) and had a better survival compared with patients who underwent chemotherapy who had a 70% CR rate prior to autoSCT (P = .046). Exposure to FDA-approved novel agents, immunoepigenetic trials, and clinical trials in general was associated with an overall survival (OS) benefit (P = .003, P = .04, and P = .006, respectively). These data suggest that patients who receive novel agents have superior outcomes compared with patients without exposure to novel therapies who receive chemotherapy-predicated treatments.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Células-Tronco Hematopoéticas/mortalidade , Linfoma de Células T Periférico/mortalidade , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Ensaios Clínicos como Assunto , Terapia Combinada , Feminino , Seguimentos , Humanos , Linfoma de Células T Periférico/patologia , Linfoma de Células T Periférico/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Adulto Jovem
5.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 27(6): 1973-1978, 2019 Dec.
Artigo em Chinês | MEDLINE | ID: mdl-31839069

RESUMO

OBJECTIVE: To explore the efficacy and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of relapsed or refractory peripheral T-cell lymphoma(PTCL). METHODS: The clinical data of 6 patients with relapsed or refractory PTCL undergoing allo-HSCT from Sep. 2014 to Sep. 2018 in the department of hematology, aerospace center hospital were retrospectively analyzed. Complications and disease-free survival after HSCT were observed. RESULTS: All the patients could well tolerate the conditioning regimen and acquired hematopoietic recon-struction. Following up till December 2018, with a median time of 11.5 months (1-51); acute GVHD developed in 2 cases and chronic GVHD developed in 5 cases, Among 6 cases one case died of viral pheumonia and the other 5 patients remained disease-free survival. The longest disease-free survival time has reached 51 months. CONCLUSION: allo-HSCT is a safe and effective method for relapsed or refractory peripheral T-cell lymphoma, which can be chosen as salvage treatment method for patients with primary resistance. Optimization of the conditioning regimen may result in better efficacy of allo-HSCT.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Linfoma de Células T Periférico , Humanos , Linfoma de Células T Periférico/terapia , Estudos Retrospectivos , Condicionamento Pré-Transplante , Transplante Homólogo
6.
Zhonghua Yi Xue Za Zhi ; 99(48): 3786-3791, 2019 Dec 24.
Artigo em Chinês | MEDLINE | ID: mdl-31874515

RESUMO

Objective: To evaluate the clinical outcomes in patients with relapsed or refractory peripheral T-cell lymphoma (PTCL) who had undergone allogeneic hematological stem cell transplantation (allo-HSCT). Methods: From June 2007 to June 2017, the clinical data of PTCL patients who underwent HSCT from eight hospitals were assessed retrospectively. Results: There were 23 patients diagnosed as relapsed or refractory PTCL with chemoresistance who underwent allo-HSCT. Among these patients, 18 were identified as progressive disease (PD) status and 5 patients as stable disease (SD) status before allo-HSCT. Seventeen patients received allo-HSCT from matched sibling donor (MSD),2 patients from matched unrelated donor and 4 patients from related haplo-identical donor (HD). After a median follow-up of 29 months, 21 patients survived longer than 28 days after allo-HSCT. Hematopoietic reconstitution was achieved in 20 of the 21 patients. The median time of myeloid and platelet engraftment were+13 (9-22) d and+16(10-38) d, respectively. The 100-d treatment-related mortality rate was 13.1%. Acute GVHD occurred in 11(47.8%) patients at a median time of 22(6-82) d after transplantation. Grade Ⅱ~Ⅳ aGVHD occurred in 6 patients. Chronic GVHD occurred in 10 patients at a median of 7.9 (3.5-27) months. After a median follow-up of 29 months, 13 patients died after HSCT. Four of them died of complications associated with allo-HSCT, and other 9 patients died of the primary lymphoma. The 3-years cumulative overall survival (OS) and progress-free survival (PFS) were 43.03% (95%CI: 29.79-69.16) and 39.13% (95%CI: 23.50-65.14), respectively. No significant difference was found in the 3-year PFS between patients with PD status and SD status before allo-HSCT (P=0.133). Conclusion: Allo-HSCT can be a promising treatment for relapsed or refractory PTCL with chemoresistance.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Linfoma de Células T Periférico , Resistencia a Medicamentos Antineoplásicos , Humanos , Linfoma de Células T Periférico/tratamento farmacológico , Recidiva Local de Neoplasia , Estudos Retrospectivos
7.
BMC Neurol ; 19(1): 266, 2019 Nov 04.
Artigo em Inglês | MEDLINE | ID: mdl-31684908

RESUMO

BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder in the central nervous system (CNS) with distinct clinical, radiological, and pathological characteristics. The pathophysiology of CLIPPERS still remains unclear. Because a few cases about lymphoma mimicking the manifestations of CLIPPERS were reported and the prognosis of lymphoma is much worse, early identification of lymphoma is very important. CASE PRESENTATION: A 31-year-old woman was admitted with 3 months' history of diplopia, dizziness, gait ataxia, and right facial numbness. The diagnosis of CLIPPERS was established based on the finding of punctate enhancing lesions in the cerebellum, thalamus, pons, medulla, and midbrain region in magnetic resonance imaging (MRI), together with the favorable clinical and radiological responses to corticosteroids. However, she was diagnosed as peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) by the pulmonary nodular and the skin biopsy almost 10 years later, and she got complete remission within 1 year after chemotherapy. CONCLUSION: We report the first case of CLIPPERS developing PTCL-NOS. This case proposes that when brain biopsy was difficult to achieve, biopsies in extra-cerebral lesions under the assisting examination of positron emission tomography-computed tomography (PET-CT) can be helpful in further identification.


Assuntos
Doenças do Sistema Nervoso Central , Inflamação , Linfoma de Células T Periférico , Adulto , Biópsia , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Encéfalo/fisiopatologia , Feminino , Humanos , Imagem por Ressonância Magnética , Esteroides/uso terapêutico
8.
Hematol Oncol ; 37(5): 578-585, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31702065

RESUMO

Event free survival at 24 months (EFS24) has been described as a powerful predictor for outcome in several subtypes of B cell lymphoma. However, it was limitedly described in T cell lymphoma. We explored the implication of EFS24 as a predictor marker for peripheral T cell lymphoma (PTCL). We reviewed 293 systemic PTCL patients at 13 nationwide major university hospitals in Thailand from 2007 to 2014. The median event free survival (EFS) and overall survival (OS) of PTCL patients in our cohort was 16.3 and 27.7 months with corresponding 2-year EFS and 2-year OS of 45.8% and 51.9%, respectively. A total of 118 patients achieved EFS24 (no events during the first 24 mo). Patients who achieved EFS24 had better OS than patients who did not (2-y OS 92% vs 18.8%; HR, 0.1; P < .001). The standardized mortality ratio of patients achieving EFS24 was 18.7 (95% CI, 14.6-22.8). Multivariable analysis demonstrated performance status, histologic subtype, remission status, and EFS24 achievement as independent predictors for OS. Our study affirmed the value of EFS24 as a powerful prognostic factor for PTCL. Further validation in prospective study setting is warranted.


Assuntos
Linfoma de Células T Periférico/mortalidade , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfoma de Células T Periférico/epidemiologia , Linfoma de Células T Periférico/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Intervalo Livre de Progressão , Vigilância em Saúde Pública , Tailândia/epidemiologia , Resultado do Tratamento
9.
Hematol Oncol ; 37(5): 569-577, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31674027

RESUMO

Romidepsin is a class I selective histone deacetylase (HDAC) inhibitor approved by the Food and Drug Administration (FDA) for relapsed/refractory (R/R) cutaneous T-cell lymphoma (CTCL) and peripheral T-cell lymphoma (PTCL), treated with at least one prior systemic therapy. Currently, there is paucity of real-life data on the efficacy and safety of romidepsin in R/R T-cell lymphoma. This national, multicenter study presents real-life data on the efficacy and safety of romidepsin in R/R T-cell lymphoma. Patients diagnosed and treated with romidepsin for R/R CTCL or PTCL between 2013 and 2018 were retrospectively reviewed. Outcomes included overall survival (OS), event-free survival (EFS), overall response rate (ORR), complete response (CR), and adverse events. Fifty-three patients with R/R PTCL (n = 42) or CTCL (n = 11) were included. Among CTCL patients, median OS was not reached, ORR was 25%, and none achieved CR. Among PTCL patients, median OS was 7.1 months, EFS was 1.9 months, ORR rate was 33%, and 12.5% achieved CR. In a univariate analysis, predictors for longer EFS include any response to therapy, number of previous lines, and PTCL subclass (with better results for angioimmunobalstic T-cell lymphoma). In a univariate and multivariate analysis for OS, treatment response was the only factor predicting OS (OR 4.48; CI 95%, 1.57-12.79; P = .005). Most grade 3 and 4 adverse events were hematological (35%). Infections were reported in 34% of patients. This real-life experience with romidepsin confirms the results of the pivotal phase II trials. PTCL subtype and the number of previous lines of therapy have an impact on EFS. In addition, patients who had good response to romidepsin benefited most in terms of both EFS and OS. Efforts should be done to identify those patients.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Depsipeptídeos/uso terapêutico , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma de Células T Periférico/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Antibióticos Antineoplásicos/administração & dosagem , Antibióticos Antineoplásicos/efeitos adversos , Depsipeptídeos/administração & dosagem , Depsipeptídeos/efeitos adversos , Resistencia a Medicamentos Antineoplásicos , Feminino , Humanos , Estimativa de Kaplan-Meier , Linfoma Cutâneo de Células T/mortalidade , Linfoma Cutâneo de Células T/patologia , Linfoma de Células T Periférico/mortalidade , Linfoma de Células T Periférico/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Recidiva , Retratamento , Resultado do Tratamento
10.
Blood ; 134(17): 1367-1368, 2019 10 24.
Artigo em Inglês | MEDLINE | ID: mdl-31698429
11.
Indian J Pathol Microbiol ; 62(4): 586-588, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31611445

RESUMO

The follicular variant of peripheral T-cell lymphoma, not otherwise specified, is very rare. Primary epiglottic follicular variant of peripheral T-cell lymphoma is extremely rare in clinical practice. Here, we report the first case of a follicular variant of peripheral T-cell lymphoma not otherwise specified in a 44-year-old Chinese man, who presented with a tumor in the middle of the epiglottis tongue surface. Microscopically, the tumor had a vague nodular growth pattern and the morphology of the nodules was different from each other at low power. Atypical lymphoid cells were medium to large in size and had round nuclei, with an irregular nuclear membrane, distinct nucleoli, and rapid mitotic activity. Plasma cells were found surrounding the nodules. The tumor cells were positive for follicular helper T-cell markers (CD10, PD-1, CXCL13, and BCL-6). The EBER was negative by in situ hybridization. Polymerase chain reaction-based analysis showed monoclonal rearrangements of TCRß, TCRγ, and polyclonal rearrangements of IgH, IgK, and IgL. The clinical and imaging features and the prognostic factors of FV PTCL-NOS remain poorly understood. Thus, investigation of more cases and longer follow-up is necessary to understand the disease and to identify the best treatment to improve prognosis.


Assuntos
Epiglote/patologia , Linfoma Folicular/diagnóstico , Linfoma Folicular/patologia , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/patologia , Adulto , Quimiocina CXCL13/genética , Epiglote/diagnóstico por imagem , Humanos , Masculino , Prognóstico , Proteínas Proto-Oncogênicas c-bcl-6/genética , Linfócitos T/citologia , Tomografia Computadorizada por Raios X
12.
Best Pract Res Clin Haematol ; 32(3): 253-261, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31585625

RESUMO

NK/T-cell lymphomas are extranodal EBV-related malignancies, mostly of NK-cell and occasionally of T-cell lineage. They are divided into nasal, non-nasal, and disseminated subtypes. Nasal NK/T-cell lymphomas involve the nose, nasopharynx and the upper aerodigestive tract. Non-nasal NK/T-cell lymphomas involve the skin, gastrointestinal tract, testis and other sites. Disseminated NK/T-cell lymphoma involves multiple organs, and may present with a leukemic phase. Initial evaluation requires positron emission tomography computed tomography (PET/CT) and quantification of circulating EBV DNA. Radiotherapy alone is inadequate with frequent relapses. Anthracycline-containing regimens are ineffective. Regimens incorporating asparaginase are currently the standard. For stage I/II disease, combined chemotherapy and radiotherapy is recommended. For stage III/IV disease, asparaginase-containing regimens are needed. Autologous hematopoietic stem cell transplantation (HSCT) is of limited efficacy, whereas allogeneic HSCT may be useful in patients with stage III/IV and relapsed diseases. Immunotherapy with antibodies against CD30, programmed cell death protein 1 and CD38 is promising.


Assuntos
Antineoplásicos Imunológicos/uso terapêutico , Asparaginase/uso terapêutico , Transplante de Células-Tronco Hematopoéticas , Linfoma Extranodal de Células T-NK , Linfoma de Células T Periférico , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Humanos , Linfoma Extranodal de Células T-NK/diagnóstico por imagem , Linfoma Extranodal de Células T-NK/imunologia , Linfoma Extranodal de Células T-NK/patologia , Linfoma Extranodal de Células T-NK/terapia , Linfoma de Células T Periférico/diagnóstico por imagem , Linfoma de Células T Periférico/imunologia , Linfoma de Células T Periférico/patologia , Linfoma de Células T Periférico/terapia , Transplante Autólogo
13.
Dtsch Med Wochenschr ; 144(20): 1400-1404, 2019 10.
Artigo em Alemão | MEDLINE | ID: mdl-31594013

RESUMO

NEW WHO CLASSIFICATION 2016 FOR LYMPHOMA AND MOLECULAR MARKER: The new WHO 2016 Classification for Lymphomas included more detailed definitions of individual entities, such as: the Anaplastic Large Cell Lymphoma, ALK; the terminus of enteropathy-associated T-cell lymphoma (EATCL, type 1) now is restricted to celiac-associated form. The integration of next Generation sequencing (NGS) enables better differential diagnostics, e. g. angioimmunoblastic lymphoma (AITL) can be characterized by the presence of TET2, RHOA, IDH2 or DNMT3A mutations. Furthermore, certain mutations also have prognostic impact. In ALCL/ALK-, evidence of a DUSP22 / IRF4 re-arrangement is associated with a better and detection of TP63 is associated with a worse prognosis. CLINICAL COURSE AND PROGNOSIS: In addition to B symptoms and nodal manifestation, extranodal manifestations such as skin infiltrates can be observed in up to 20 %. In AITL, polyclonal hypergammaglobulinemia, Coombs-positive hemolytic anemia and immunodeficiency can occur, as a sign of a dysregulated immune system. ADVANCES IN THERAPY: By combining the immunoconjugate brentuximab-vedotin with chemotherapy, improvement in disease-free and overall survival in CD30+-PTCL (especially in ALCL) was observed. FUTURE PERSPECTIVE: At present demethylating agent 5-azacytidine is explored in AITL and PTCL with a T-follicle helper type (TFH).


Assuntos
Linfoma de Células T Periférico , Antineoplásicos/uso terapêutico , Análise Mutacional de DNA , Diagnóstico Diferencial , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Imunoconjugados/uso terapêutico , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/genética , Linfoma de Células T Periférico/terapia , Mutação/genética
15.
Ann Hematol ; 98(11): 2541-2550, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31493002

RESUMO

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a provisional entity in the 2017 World Health Organization classifications. To further elucidate the clinicopathologic features of this new disease, we carried out a retrospective, multicenter analysis of 42 patients with MEITL. The median age of the patients was 59 years (range, 20-84 years), and 27 patients (64 %) were male. Thirty-two patients (76 %) were Ann-Arbor stages I-II and 28 (67 %) were Lugano stages I-II1&2. The most frequent site of involvement was the jejunum (N = 21). Most cases expressed CD8 (79 %) and CD56 (95 %) and did not express CD30 (5 %) or EBER (0 %). The median progression-free survival was 6.9 months (95 % CI 4.3-9.6); the median OS was 14.8 months (2.4-27.2). Thirty-two patients (76 %) underwent surgery and 37 (88 %) received chemotherapy. A complete response (CR) rate was 38 %. Sixteen patients had undergone autologous stem cell transplantation (ASCT). Relapse or progression was documented in 24 cases, most frequently in the primary site (N = 23). Four cases showed central nervous system relapse. Age over 55 years, poor performance scale, advanced Lugano stage (IIE-IV), not achieving CR, and not receiving ASCT were associated with inferior OS. While the optimal management of MEITL remains undetermined, achieving CR and consolidative ASCT seem essential. As CHOP might be insufficient for achieving CR, more efficient combinations should be investigated. Additionally, considering the frequent local failure and CNS relapse, novel therapeutic approaches are required to improve survival.


Assuntos
Antígenos CD/biossíntese , Neoplasias do Jejuno , Linfoma de Células T Periférico , Proteínas de Neoplasias/biossíntese , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Feminino , Humanos , Neoplasias do Jejuno/metabolismo , Neoplasias do Jejuno/mortalidade , Neoplasias do Jejuno/patologia , Neoplasias do Jejuno/terapia , Linfoma de Células T Periférico/metabolismo , Linfoma de Células T Periférico/mortalidade , Linfoma de Células T Periférico/patologia , Linfoma de Células T Periférico/terapia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Taxa de Sobrevida
16.
J Cancer Res Clin Oncol ; 145(10): 2595-2604, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31410605

RESUMO

PURPOSE: Peripheral T cell lymphomas (PTCL) are a rare and heterogeneous group of aggressive non-Hodgkin lymphomas, showing a generally poor prognosis. In this retrospective analysis, we aimed to investigate the impact of autologous stem cell transplantation (autoSCT) in PTCL. METHODS: A retrospective analysis of 58 consecutive unselected PTCL patients aged 21-71 years undergoing autoSCT as first-line consolidation as well as in the relapse setting was performed. RESULTS: The median follow-up time was 67 months. A 5-year overall survival (OS) of 53% and a 5-year progression-free survival (PFS) after autoSCT of 44% was achieved. The overall relapse rate after autoSCT was 50%. On multivariate analysis, standard baseline characteristics such as age, disease stage and international prognostic index (IPI) score failed to predict outcome in our cohort. First-line treatment with autoSCT was not associated with a benefit in OS when compared to patients receiving autoSCT at relapse. Notably, autoSCT seemed to be a suitable approach even for older transplant-eligible patients (aged ≥ 60 years), with a similar 5-year OS of 49% when compared to younger patients. CONCLUSIONS: Our study suggests that autoSCT can achieve long-term survival in PTCL patients even after relapse and should also be considered for eligible older patients.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Linfoma de Células T Periférico/terapia , Adulto , Idoso , Terapia Combinada , Feminino , Seguimentos , Transplante de Células-Tronco Hematopoéticas/métodos , Humanos , Estimativa de Kaplan-Meier , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/mortalidade , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Fatores de Tempo , Transplante Autólogo , Resultado do Tratamento , Adulto Jovem
17.
Medicine (Baltimore) ; 98(34): e16932, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31441883

RESUMO

INTRODUCTION: Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of mature peripheral T-cell lymphoma and accounts for approximately 1% to 2% of non-Hodgkin lymphomas. Although the B symptoms with generalized lymphadenopathy are the most frequent manifestations of AITL, its diagnosis remains a challenge as clinical manifestations and pathological features are frequently misleading. PATIENT CONCERNS: We report herein the case of a 70-year-old man with intermittent fever, pulmonary infection, and skin rash developed for 1 month before admission. Previously, he had undergone thyroidectomy for thyroid papillary carcinoma. Fever occurred on the day of discharge and occurred again during the next month. Symptoms worsened despite treatment with antibiotics and papular rash appeared. The local hospital diagnosed it as drug fever and stopped all antibiotics. Fever and rash were controlled temporarily; however, both relapsed 2 days before admission. On the night of admission, the patient developed fever again. Blood culture showed Staphylococcus epidermidis and Staphylococcus haemolyticus infection. INTERVENTIONS: Taking into account the recent history of surgery, the patient was diagnosed with septicemia and was treated with anti-infective treatment. On 13th day after admission, the patient developed fever again accompanied by generalized lymphadenopathy. However, multiple blood cultures were negative and bone marrow aspiration cytology, biopsy, immunohistochemistry, and gene rearrangement results were normal. DIAGNOSIS: The patient was finally subjected to cervical lymph node biopsy and was diagnosed with AITL. OUTCOMES: The patient was transferred to the Department of Hematology for further treatment. CONCLUSION: This case highlights the complex diagnostic challenges of AITL. AITL accompanied by thyroid carcinoma may not be a mere coincidence and administration of antibiotics may be a rare cause of AITL.


Assuntos
Linfonodos/patologia , Linfoma de Células T Periférico/diagnóstico , Linfoma de Células T Periférico/patologia , Idoso , Diagnóstico Diferencial , Exantema/etiologia , Febre/etiologia , Humanos , Pulmão/diagnóstico por imagem , Linfadenopatia/diagnóstico por imagem , Masculino , Infecções Estafilocócicas/diagnóstico , Tireoidectomia/efeitos adversos
18.
Am J Dermatopathol ; 41(10): 754-756, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31436576

RESUMO

Peripheral T-cell lymphomas expressing follicular helper T-cell (TFH) markers have recently been identified. Although this type of lymphomas consist of malignant proliferation of T-cells, they may also exhibit B-cell clonality. We report a case of a 72-year-old woman with multiple erythematous to violaceous nonscaling plaques and tumors on her trunk. Histopathological analysis revealed a dense infiltration of medium-to-large-sized atypical cells throughout the entire dermis. The result of immunohistochemical analysis showed that the infiltrating T-cells expressed programmed death-1 (PD-1), CD10, Bcl-6, CD3, CD4, CD2, and CD5. The infiltrate also contained scattered atypical large B-cells. Based on the clinical appearance and the histopathological findings, we diagnosed the patient with secondary cutaneous dissemination of peripheral T-cell lymphoma with expression of a T-follicular helper phenotype.


Assuntos
Linfoma de Células T Periférico/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Cutâneas/patologia , Idoso , Neoplasias da Mama/patologia , Carcinoma/patologia , Carcinoma Ductal de Mama/patologia , Neoplasias do Endométrio/patologia , Feminino , Humanos , Linfoma Difuso de Grandes Células B/patologia , Linfócitos T Auxiliares-Indutores/patologia
19.
mSphere ; 4(4)2019 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-31292228

RESUMO

Certain peripheral T-cell lymphomas (PTCLs) have been associated with viral infection, particularly infection with Epstein-Barr virus (EBV). However, a comprehensive virome analysis across PTCLs has not previously been reported. Here we utilized published whole-transcriptome RNA sequencing (RNA-seq) data sets from seven different PTCL studies and new RNA-seq data from our laboratory to screen for virus association, to analyze viral gene expression, and to assess B- and T-cell receptor diversity paradigms across PTCL subtypes. In addition to identifying EBV in angioimmunoblastic T-cell lymphoma (AITL) and extranodal NK/T-cell lymphoma (ENKTL), two PTCL subtypes with well-established EBV associations, we also detected EBV in several cases of anaplastic large-cell lymphoma (ALCL), and we found evidence of infection by the oncogenic viruses Kaposi's sarcoma-associated herpesvirus and human T-cell leukemia virus type 1 in isolated PTCL cases. In AITLs, EBV gene expression analysis showed expression of immediate early, early, and late lytic genes, suggesting either low-level lytic gene expression or productive infection in a subset of EBV-infected B-lymphocyte stromal cells. Deconvolution of immune cell subpopulations demonstrated a greater B-cell signal in AITLs than in other PTCL subtypes, consistent with a larger role for B-cell support in the pathogenesis of AITL. Reconstructed T-cell receptor (TCR) and B-cell receptor (BCR) repertoires demonstrated increased BCR diversity in AITLs, consistent with a possible EBV-driven polyclonal response. These findings indicate potential alternative roles for EBV in PTCLs, in addition to the canonical oncogenic mechanisms associated with EBV latent infection. Our findings also suggest the involvement of other viruses in PTCL pathogenesis and demonstrate immunological alterations associated with these cancers.IMPORTANCE In this study, we utilized next-generation sequencing data from 7 different studies of peripheral T-cell lymphoma (PTCL) patient samples to globally assess viral associations, provide insights into the contributions of EBV gene expression to the tumor phenotype, and assess the unique roles of EBV in modulating the immune cell tumor microenvironment. These studies revealed potential roles for EBV replication genes in some PTCL subtypes, the possible role of additional human tumor viruses in rare cases of PTCLs, and a role for EBV in providing a unique immune microenvironmental niche in one subtype of PTCLs. Together, these studies provide new insights into the understudied role of tumor viruses in PTCLs.


Assuntos
Herpesvirus Humano 4/genética , Linfoma de Células T Periférico/imunologia , Linfoma de Células T Periférico/virologia , Microambiente Tumoral/genética , Linfócitos B/imunologia , Microambiente Celular , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/imunologia , Regulação Viral da Expressão Gênica , Herpesvirus Humano 8/genética , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Análise de Sequência de RNA , Linfócitos T/imunologia , Microambiente Tumoral/imunologia
20.
Cancer Biomark ; 25(3): 259-273, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31282408

RESUMO

BACKGROUND: The expression of neuropilin-1 (NRP-1) in Epstein-Barr virus (EBV)-associated lymphomas and its relationships with clinicopathological parameters was investigated. METHODS: The researchers compared 111 cases of patients with lymphoma to 20 cases of reactive lymphoid hyperplasia. In situ hybridization was applied to observe the expression of EBV-encoded RNA (EBER) in lymphomas, and immunohistochemistry was used to detect the NRP-1 expression in lymphoma tissues and lymph node tissues with reactive hyperplasia. RESULTS: In these 111 cases, the EBER of 62 cases (55.9%) appeared positive. NRP-1 was relatively highly expressed in lymphomas (P= 0.019). Further, NRP-1 showed higher expression in lymphomas with positive EBER than in negative ones. A comprehensive analysis revealed that NRP-1 was differently expressed in NK/T-cell lymphoma, Hodgkin's lymphoma, diffuse large B-cell lymphoma, and anaplastic large cell lymphoma (P= 0.027). Moreover, highly expressed NRP-1 was found to be a useful independent prognostic factor in assessing overall survival and progression-free survival rates in cases of non-Hodgkin's lymphoma (NHL). CONCLUSIONS: NRP-1 exhibited higher expression in lymphomas, and it was positively expressed in EBV-positive lymphomas. Moreover, highly expressed NRP-1 can be used as an undesirable independent prognostic factor in NHL.


Assuntos
Biomarcadores Tumorais/genética , Infecções por Vírus Epstein-Barr/genética , Linfoma/genética , Neuropilina-1/genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Infecções por Vírus Epstein-Barr/patologia , Infecções por Vírus Epstein-Barr/virologia , Feminino , Regulação Neoplásica da Expressão Gênica/genética , Herpesvirus Humano 4/genética , Herpesvirus Humano 4/patogenicidade , Humanos , Imuno-Histoquímica , Linfoma/classificação , Linfoma/patologia , Linfoma/virologia , Linfoma Extranodal de Células T-NK/genética , Linfoma Extranodal de Células T-NK/patologia , Linfoma Extranodal de Células T-NK/virologia , Linfoma Difuso de Grandes Células B/genética , Linfoma Difuso de Grandes Células B/patologia , Linfoma Difuso de Grandes Células B/virologia , Linfoma de Células T Periférico/genética , Linfoma de Células T Periférico/patologia , Linfoma de Células T Periférico/virologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Adulto Jovem
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