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Objective To explore the changes in serum energy metabolites in patients with peripheral T-cell lymphoma,and investigate serum biomarkers for monitoring peripheral T-cell lymphoma from the perspective of energy metabolism.Methods Multiple/selected reaction monitoring(MRM/SRM)was used to detect the energy-related metabolites in the sera of 16 patients with newly diagnosed peripheral T-cell lymphoma admitted in the Hematology Medical Center of the Second Affiliated Hospital of Army Medical University from November 2020 to December 2021,as well as 10 recruited healthy volunteers.The corresponding clinical data including medical history,laboratory results and image data were collected and retrospectively analyzed.Results Significant differences were seen in the contents and expression profiles of serum energy metabolism-related products between the patients and the healthy volunteers.The patients had significantly reduced serum contents of cyclic AMP,succinate,citrate and cis-aconitate(P＜0.05),and elevated D-glucose 6-phosphate content(P＜0.05).The serum contents of citrate and succinate were negatively correlated with the risk stratification(low-,moderate-and high-risk)and clinical stage of the disease(P＜0.05).Meanwhile,there was a negative correlation between the contents of L-malic acid and citrate and the mid-term efficacy evaluation results,such as complete/partial response(CR/PR)or stable disease(SD)(P＜0.05).For patients with extranodal NK/T cell lymphoma(n=10),there were also significant reductions in the contents of cyclic AMP,succinate,citrate,isocitrate and cis-aconitate in the sera of patients compared with healthy volunteers(P＜0.05),and the contents of citrate and succinate were negatively correlated with the clinical stage(P＜0.05)and were rather correlated with mid-term efficacy evaluation results(CR/PR or SD)(P＜0.05).For patients with angioimmunoblastic T-cell lymphoma(n=6),the serum contents of cyclic AMP,citrate and succinate were significantly lower,while the content of D-glucose 6-phosphate was higher when compared with the healthy volunteers(P＜0.05),and the content of succinate was negatively correlated with both clinical stage and risk grade of the patients(P＜0.05).Conclusion There are 5 serum differential metabolites identified between patients with peripheral T-cell lymphoma and healthy controls,and succinate and citrate are expected to be serum biomarkers of peripheral T-cell lymphoma.
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Objective To investigate the clinicopathological characteristics of extranodal NK/T cell lymphoma(ENKTL)with B-lymphocytosis.Methods Two cases of ENKTL with B-lymphocytosis diagnosed in Shaanxi Provincial People's Hospital from June to September 2023 were collected.HE staining,immunohistochemistry,and in situ hybridisation Epstein-barr virus encoded small RNA(EBER)testing was used to observe the histological features,immunophenotypes,and results of the in situ hybridisation EBER testing.A review of the relevant literature was conducted.Results In two cases of elderly male patients,whose lesion sites were on both the right side of the nasal cavity,histological characteristics of the tumor cells were diffuse distribution.The cells were of different sizes,mainly medium and large cells,with irregular nuclei,stained or transparent cytoplasm,oval nuclei,granular chromatin and inconspicuous nucleoli.Nuclear schizophrenia was more common and coagulative necrosis and apoptosis were evident.Foci of small lymphocyte aggregates were seen in the background and lymphoid follicles were distributed in a scattered manner.Immunohistochemical CD2,CD3,CD56,TIA-1 and granzyme B(GrB)were positive.CD20,CD79a and PAX-5 were focal positive.CD21,CD23 and CD35 had residual FDC network,and CD5 was negative.Ki-67 proliferation index was approximately 30%.EBER tumor cells detected by in situ hybridisation were positive.Pathological diagnosis showed ENKTL with B-lymphocytosis.Conclusion NKTL with B-lymphocytosis was rare,especially when B-lymphocyte hyperplasia formed lymphoid follicles.Lack of experience can easily cause diagnostic difficulties,and comprehensive analysis and diagnosis should be combined with the clinical manifestations,histological morphology and immunophenotype.
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Objective:To compare and choose the best method for measuring metabolic tumor volume (MTV) of nasal extranodal natural killer/T-cell lymphoma (ENKTL), evaluate the prognostic value of 18F-FDG PET/CT metabolic parameters and clinical staging/scoring systems for patients with nasal ENKTL, and explore the added value of the two combinations for prognostic prediction. Methods:From January 2016 to September 2022, 44 patients (26 males, 18 females; age (47.5±13.6) years) pathologically diagnosed with nasal ENKTL who underwent 18F-FDG PET/CT imaging before treatment in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology were retrospectively collected. SUV 2.5, SUV 4.0 and 41%SUV max were used as thresholds to measure MTV and total lesion glycolysis (TLG), and the consistency was analyzed by Bland-Altman analysis. The ROC curve analysis was used to compare the prognostic efficiency of different methods and determine the best method. The prognostic values of different clinical factors and clinical staging/scoring systems between groups were evaluated by corrected χ2 test. The independent factors were screened by Cox-regression model, and the combined diagnosis model was constructed by logistic regression. Results:Of 44 patients, 6(13.6%) were dead, with the overall survival (OS) of 32.05(11.77, 64.43) months, and the 2-year and 5-year OS rates of 86.6% and 82.5%, respectively. The mortality of different groups in age (≥60 and <60 years), prognostic index of natural killer cell lymphoma (PINK) score (low- and high-risk), and international prognostic index (IPI) score (low- and high-risk) were significantly different ( χ2 values: 5.02, 4.12, 3.88, all P<0.05). The consistency of MTV measured by different thresholds was good. Among them, the MTV measured by threshold of SUV 2.5 had the highest predictive efficiency with the AUC of 0.737. Multivariate analysis showed that MTV (hazard ratio ( HR)=10.488, 95% CI: 1.864-59.026, P=0.008) was the independent influencing factor of OS. By removing other factors, minimization model was obtained, including MTV and PINK score ( P values: 0.006, 0.048). The prediction model of MTV combined with PINK score improved prognostic efficacy with the AUCs of MTV, PINK score and the combination model of 0.781, 0.741 and 0.912, respectively. Conclusions:MTV measured by threshold of SUV 2.5 has better prognostic predictive value. MTV is the independent prognostic factor for OS in nasal ENKTL patients. MTV combined with PINK score has better prognostic value.
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Abstract Objective We aimed to assess the significance of rENE and creat a predictive tool (nomogram) for estimating Overall Survival (OS) in locoregionally advanced Nasopharyngeal Carcinoma (NPC) patients with Lymph Node Metastasis (LNM) based on their clinical characteristics and Radiologic Extranodal Extension (rENE). Methods Five hundred and sixty-nine NPC patients with LNM were randomly divided into training and validation groups. Significant factors were identified using univariate and multivariate analyses in the training cohort. Then, the nomogram based on the screening results was established to predict the Overall Survival (OS). Calibration curves and the Concordance index (C-index) gauged predictive accuracy and discrimination. Receiver Operating Characteristic (ROC) analysis assessed risk stratification, and clinical utility was measured using Decision Curve Analysis (DCA). The nomogram's performance was validated for discrimination and calibration in an independent validation cohort. Results A total of 360 (63.2%) patients were present with radiologic extranodal extension at initial diagnosis. Patients with rENE had significantly lower OS than other patients. Multivariate analysis identified the five factors, including rENE, for the nomogram model. The C-index was 0.75 (0.71-0.78) in the training cohort and 0.76 (0.69-0.83) in the validation cohort. Notably, the nomogram outperformed the 8th TNM staging system, as evident from the higher AUC values (0.77 vs. 0.60 for 2 year and 0.75 vs. 0.65 for 3 year) and well-calibrated calibration curves. Decision curve analysis indicated improved Net Benefit (NB) with the nomogram for predicting OS. The log-rank test confirmed significant survival distinctions between risk groups in both training and validation cohorts. Conclusions We demonstrated the prognostic value of rENE in nasopharyngeal carcinoma and developed a nomogram based on rENE and other factors to provide individual prediction of OS for locoregionally advanced nasopharyngeal carcinoma with lymph node metastasis. Level of evidence: III.
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NK/T cell leukemia/lymphoma is a type of malignancy originating from T cells or natural killer cells with low incidence and poor clinical prognosis. There is still no effective treatment strategy. In recent years, targeted therapy has made great progress in the treatment of hematological malignancies, including monoclonal antibody and chimeric antigen receptor T cells (CAR-T), among which CD30, CD7, CD5, CD52, CCR4 and other target antigens are effective in the treatment of NK/T cell leukemia/lymphoma, but its widespread application still faces a great challenge. This article reviews the progress of immunotherapy for NK/T cell leukemia/lymphoma.
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Extranodal NK/T cell lymphoma (ENKTCL) is a group of highly aggressive non-Hodgkin lymphoma associated with epstein-barr virus infection. Asparaginase-based chemoradiotherapy regimens are not effective in advanced patients. In recent years, anti-programmed-death receptor 1 (PD-1)/programmed-death receptor ligand 1 (PD-L1) immunotherapy has developed rapidly, which can effectively improve the prognosis of ENKTCL patients. But some patients with ENKTCL still have low response rate. This article reviews the mechanisms of resistance to anti-PD1/PD-L1 therapy and other immune targets in ENKTCL recently to change the traditional treatment mode of ENKTCL through the combination of different targeted drugs.
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Extranodal NK/T cell lymphoma, nasal type（ENKTL） is a highly aggressive malignant tumor derived from NK cells. This article reports a case of ENKTL invading the larynx and digestive tract. The clinical clinical manifestations include hoarseness and intranasal masses.
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Humans , Lymphoma, Extranodal NK-T-Cell/pathology , Nose/pathology , Nose Neoplasms/pathology , Larynx/pathology , Gastrointestinal Tract/pathologyABSTRACT
Objective: To explore the prognostic factors of extracellular NK/T cell lymphoma (ENKTL) treated with pegaspargase/L-asparaginase. Methods: The clinical data of 656 ENKTL patients diagnosed at 11 medical centers in the Huaihai Lymphoma Working Group from March 2014 to April 2021 were retrospectively analyzed. The patients were randomly divided into two groups: a training set (460 cases) and a validation set (196 cases) at 7∶3, and the prognostic factors of the patients were analyzed. A prognostic scoring system was established, and the predictive performance of different models was compared. Results: Patients' median age was 46 (34, 57) years, with 456 males (69.5% ) and 561 nasal involvement (85.5% ). 203 patients (30.9% ) received a chemotherapy regimen based on L-asparaginase combined with anthracyclines, and the 5-year overall survival rate of patients treated with P-GEMOX regimen (pegaspargase+gemcitabine+oxaliplatin) was better than those treated with SMILE regimen (methotrexate+dexamethasone+cyclophosphamide+L-asparaginase+etoposide) (85.9% vs 63.8% ; P=0.004). The results of multivariate analysis showed that gender, CA stage, the Eastern Cooperative Oncology Group performance status (ECOG PS) score, HGB, and EB virus DNA were independent influencing factors for the prognosis of ENKTL patients (P<0.05). In this study, the predictive performance of the prognostic factors is superior to the international prognostic index, Korean prognostic index, and prognostic index of natural killer lymphoma. Conclusion: Gender, CA stage, ECOG PS score, HGB, and EB virus DNA are prognostic factors for ENKTL patients treated with pegaspargase/L-asparaginase.
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Male , Humans , Middle Aged , Asparaginase/therapeutic use , Prognosis , Retrospective Studies , Lymphoma, Extranodal NK-T-Cell/drug therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Etoposide , Cyclophosphamide , Methotrexate/therapeutic use , DNA/therapeutic use , Treatment OutcomeABSTRACT
Objective:To investigate the predictive value of MRI radiologic extranodal extension (rENE) for distant metastasis of prostate cancer (PCa).Methods:The data of 107 patients of initial visit with clinically diagnosed N1 PCa who underwent MRI and 68Ga-prostate specific membrane antigen (PSMA) PET/CT examinations were retrospectively analyzed at Xijing Hospital, Air Force Medical University from January 2017 to April 2022. The rENE was evaluated with MRI. According to the results of 68Ga-PSMA PET/CT, the patients were divided into the distant metastasis group (group M1, 87 cases) and the non-distant metastasis group (group M0, 20 cases). Independent sample t test, Mann-Whitney U test or χ 2 test were used to compare the differences in clinical indicators and rENE between the two groups. The multivariate logistic regression analysis was used to screen the independent risk factors affecting distant metastasis. The receiver operating characteristic (ROC) curve was used to evaluate the predictive efficacy of independent risk factors for PCa distant metastasis. Results:In group M1, 72 cases (82.8%) were rENE positive and 15 cases (17.2%) were rENE negative, and in group M0, 7 cases (35.0%) were rENE positive and 13 cases (65.0%) were rENE negative, and there was a statistically significant difference in rENE between the two groups (χ 2=19.20, P<0.001). There were significant differences in total prostate specific antigen level, International Society of Urological Pathology grade and T stage between the group M1 and the group M0 ( P<0.05). Multivariate logistic regression analysis showed that rENE (OR=6.248, 95%CI 1.807-21.600, P=0.004) was an independent risk factor for distant metastasis of PCa, and the area under the ROC curve of rENE in the diagnosis of distant metastasis of PCa was 0.739 (95%CI 0.607-0.871), the sensitivity was 82.8%, and the specificity was 65.0%. Conclusion:rENE is an independent predictor of distant metastasis of PCa, which has a high efficacy. Compared with patients with rENE negative, PCa patients with rENE positive have a higher degree of invasion and are more likely to have distant metastasis.
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Objective:To analyze the diagnostic and prognostic value of routine bone marrow examination in patients with extranodal NK/T-cell lymphoma (ENKTCL) based on PET-CT staging.Methods:Clinical data of 186 patients who received bone marrow biopsy and bone marrow aspiration in Fujian Medical University Union Hospital from 2013 to 2021 were retrospectively analyzed. All patients were divided into bone marrow biopsy + bone marrow aspiration group ( n=186) and PET-CT + bone marrow biopsy group ( n=139). The sensitivity, specificity, positive and negative predictive values were compared between two groups. The data were analyzed and plotted. Survival analysis was performed using Kaplan-Meier method and log-rank test. Results:In the whole cohort, 45 patients were positive for bone marrow biopsy, and 30 of them were positive for bone marrow aspiration. A total of 141 patients who were negative for bone marrow biopsy also achieved negative results for bone marrow aspiration. A total of 139 patients completed PET-CT staging and bone marrow biopsy. And 30 patients were diagnosed with positive bone marrow by PET-CT, in which 22 of them were confirmed positive by bone marrow biopsy. Among 109 patients diagnosed with negative bone marrow by PET-CT, 5 of them were confirmed positive by bone marrow biopsy. All these cases were classified as stage Ⅳ due to distant metastases. PET-CT had a diagnostic sensitivity of 81.5%, a specificity of 92.9%, a positive predictive value of 73.3%, and a negative predictive value of 95.4%. Among early stage (Ⅰ-Ⅱ stage) patients diagnosed with PET-CT, all of them were negative for bone marrow biopsy (the negative predictive value was 100%). In stage Ⅳ patients ( n=55), the 1-year overall survival of patients with bone marrow involvement by bone marrow biopsy or PET-CT ( n=35) compared with their counterparts with the involvement of other organs ( n=20) was 28.7% vs.42.0% ( P=0.13), and 1-year progression free survival rates was 23.2% vs. 23.3% in ( P=0.94). Conclusions:Routine bone marrow biopsy does not change the original staging of patients with early stage ENKTCL based on PET-CT staging. Advanced stage patients with positive bone marrow biopsy tend to obtain worse prognosis, indicating that bone marrow biopsy still has certain value.
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Objective:To investigate clinical and laboratory characteristics of secondary hemophagocytic lymphohistiocytosis (sHLH) associated with secondary cutaneous T-cell lymphoma (CTCL) .Methods:CTCL patients with clinically suspected sHLH were collected from Department of Hematology, Wuhan No.1 Hospital from January 2016 to October 2021, and were evaluated according to the HLH-2004 diagnostic criteria and HScore.Results:Seven CTCL patients were confirmedly diagnosed with sHLH, including 2 with primary cutaneous γδT-cell lymphoma (PC-GDTCL) , 3 with cutaneous extranodal natural killer/T-cell lymphoma (C-ENKTCL) , and 2 with primary cutaneous anaplastic large cell lymphoma (PC-ALCL) . All the 7 patients received chemotherapy, but 6 died finally, and the median overall survival duration was 26.5 days (range: 14 - 60 days) after the confirmed diagnosis of CTCL complicated by sHLH. HLH-related gene mutations, which were located in the PRF1 and LYST genes, were identified in 2 patients; lymphoma-related gene mutations were identified in the KRAS and KMT2D genes in 1 PC-GDTCL patient,and in the JAK3 and SAMHD1 genes in another PC-GDTCL patient.Conclusions:CTCL complicated by sHLH usually progresses rapidly, so early diagnosis and treatment are needed. Bone marrow biopsy and mutation screening of lymphoma- and HLH-related genes at initial diagnosis and during disease progression may facilitate early diagnosis.
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Objective:To investigate the efficacy and safety of SIMPLE regimen in the treatment of extranodal NK/T-cell lymphoma (ENKTCL).Methods:The clinical data of 11 patients with ENKTCL who were admitted to the University of Hong Kong-Shenzhen Hospital from January 2012 to January 2022 were retrospectively analyzed. The patients received 4-6 courses of SIMPLE (cisplatin, gemcitabine, ifosfamide, etoposide, dexamethasone, and pegasparaginase) regimen chemotherapy, and stage Ⅰ and Ⅱ patients who also received local radiotherapy after 2 or 3 courses of chemotherapy. Patients were evaluated for mid-treatment and end-of-treatment outcomes, and the adverse effects of patients were evaluated in each treatment cycle. The Kaplan-Meier method was used to analyze the progression-free survival (PFS) and overall survival (OS) of the 11 patients.Results:All 11 patients were nasal type, with the median age of 41 years old (26-67 years old), including 5 males and 6 females, 3 relapsed cases and 8 newly treated cases. Of the 10 patients evaluated for efficacy, 9 achieved complete remission and 1 achieved at least partial remission (efficacy was assessed based on follow-up). All 11 patients were followed up for a median time of 50 months (15-72 months) and 2 relapsed patients died due to disease progression. The expected 5-year PFS rate and OS rate of 11 patients were both 90.0%, and the expected 5-year OS rate was 100.0% and 66.6% in newly treated and relapsed patients, respectively. Common adverse effects were hematologic adverse reactions, infections, gastrointestinal symptoms, elevated transaminases, and hypofibrinogenemia, all of which were curable. There is no treatment-related death.Conclusions:The SIMPLE regimen for the treatment of ENKTCL has a high remission rate, the patients have long survival time, and the regimen is moderately well tolerated.
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OBJECTIVE@#To explore the influence of lymphocyte-to-monocyte ratio (LMR) and neutrophil-to-lymphocyte ratio (NLR) on the prognosis of patients with extranodal NK/T cell lymphoma (ENKTL).@*METHODS@#The clinical data of 203 patients with ENKTL admitted to the First Affiliated Hospital of Zhengzhou University from January 2011 to January 2020 were retrospectively analyzed. The ROC curve determined the limit values of LMR and NLR; Categorical variables were compared using a chi-square test, expressed as frequency and percentage (n,%). Continuous variables were expressed as medians and extremes and compared with the Mann-Whitney U test; Progression-free survival (PFS) and overall survival (OS) of different grouped LMR and NLR patients were analyzed using Kaplan-Meier curves and compared with log-rank tests. The COX proportional risk regression model was used to perform one-factor and multi-factor analysis of PFS and OS.@*RESULTS@#The optimal critical values of LMR and NLR were determined by the ROC curve, which were 2.60 and 3.40, respectively. LMR≤2.60 was more likely to occur in patients with bone marrow invasion (P=0.029) and higher LDH (P=0.036), while NLR≥3.40 was more likely to occur in patients with higher ECOG scores (P=0.002), higher LDH (P=0.008), higher blood glucose (P=0.024), and lower PLT (P=0.010). Kaplan-Meier survival analysis showed that PFS and OS of patients in the high LMR group were significantly better than the low LMR group, while PFS and OS in the low NLR group were significantly better than the high NLR group. The results of multivariate COX analysis showed that EBV-DNA positive (P=0.047), LMR≤2.60 (P=0.014), NLR≥3.40 (P=0.023) were independent risk factors affecting PFS in patients with ENKTL. LMR≤2.60 (P<0.001), NLR≥3.40 (P=0.048), and high β2-MG (P=0.013) were independent risk factors affecting OS in patients with ENKTL.@*CONCLUSION@#Low LMR and high NLR before treatment are associated with poor prognosis in patients with ENKTL, which also can be used as an easily testable, inexpensive, and practical prognostic indicator in the clinic.
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Humans , Monocytes/pathology , Neutrophils , Lymphoma, Extranodal NK-T-Cell/pathology , Retrospective Studies , Lymphocytes , PrognosisABSTRACT
OBJECTIVE@#To investigate the effect of baicalin on the growth of extranodal NK/T cell lymphoma (ENKTCL) cells and its related mechanism.@*METHODS@#Normal NK cells and human ENKTCL cells lines SNK-6 and YTS were cultured, then SNK-6 and YTS cells were treated with 5, 10, 20 μmol/L baicalin and set control. Cell proliferation and apoptosis was detected by Edu method and FCM method, respectively, and expressions of BCL-2, Bax, FOXO3 and CCL22 proteins were detected by Western blot. Interference plasmids were designed and synthesized. FOXO3 siRNA interference plasmids and CCL22 pcDNA overexpression plasmids were transfected with PEI transfection reagent. Furthermore, animal models were established for validation.@*RESULTS@#In control group and 5, 10, 20 μmol/L baicalin group, the proliferation rate of SNK-6 cells was (56.17±2.96)%, (51.92±4.63)%, (36.42±1.58)%, and (14.60±2.81)%, respectively, while that of YTS cells was (58.85±2.98)%, (51.38±1.32)%, (34.75±1.09)%, and (15.45±1.10)%, respectively. In control group and 5, 10, 20 μmol/L baicalin group, the apoptosis rate of SNK-6 cells was (5.93±0.74)%, (11.78±0.34)%, (28.46±0.44)%, and (32.40±0.37)%, respectively, while that of YTS cells was (7.93±0.69)%, (16.29±1.35)%, (33.91±1.56)%, and (36.27±1.06)%, respectively. Compared with control group, the expression of BCL-2 protein both in SNK-6 and YTS cells decreased significantly (P<0.001), and the expression of Bax protein increased in SNK-6 cells only when the concentration of baicalin was 20 μmol/L (P<0.001), while that in YTS cells increased in all three concentrations(5, 10, 20 μmol/L) of baicalin (P<0.001). The expression of FOXO3 protein decreased while CCL22 protein increased in ENKTCL cell lines compared with human NK cells (P<0.001), but the expression of FOXO3 protein increased (P<0.01) and CCL22 protein decreased after baicalin treatment (P<0.001). Animal experiments showed that baicalin treatment could inhibit tumor growth. The expression of CCL22 protein in ENKTCL tissue of nude mice treated with baicalin decreased compared with control group (P<0.01), while the FOXO3 protein increased (P<0.05). In addition, FOXO3 silencing resulted in the decrease of FOXO3 protein expression and increase of CCL22 protein expression (P<0.01, P<0.001).@*CONCLUSION@#Baicalin can inhibit proliferation and promote apoptosis of ENKTCL cell lines SNK-6 and YTS, up-regulate the expression of Bax protein, down-regulate the expression of BCL-2 protein, and down-regulate the expression of CCL22 protein mediated by FOXO3. Animal experiment shown that the baicalin can inhibit tumor growth. Baicalin can inhibit the growth and induce apoptosis of ENKTCL cells through FOXO3/CCL22 signaling pathway.
Subject(s)
Animals , Mice , Humans , Lymphoma, Extranodal NK-T-Cell/pathology , Forkhead Box Protein O3/metabolism , bcl-2-Associated X Protein/pharmacology , Mice, Nude , Signal Transduction , Apoptosis , Proto-Oncogene Proteins c-bcl-2/metabolism , Chemokine CCL22/pharmacologyABSTRACT
Background: Non-Hodgkin抯 Lymphoma is a common hematological malignancy. Its incidence and distribution in India are slightly different from developed nations. The study was initiated to obtain the epidemiological, clinical and histopathological spectrum of lymphoma in a single tertiary care hospital of North India. Methods: 104 eligible consecutive cases of lymphoma were retrospectively analyzed from January 2016 to March 2020. Morphology and immunohistochemistry were performed and clinical details were captured from hospital information system. Cases were reclassified according to WHO classification of Tumors of Hematopoietic and Lymphoid Tissue 2017 and also clinically as nodal and extra nodal lymphomas and analyzed. Results: Among the included patients 14.4% patients are diagnosed as Hodgkin抯 lymphoma (HL) while 85.5% belonged to non-Hodgkin抯 lymphoma (NHL). Median age for NHL is 52.5 years. Male outnumbered females in both NHL and HL with ratio of 1.87 and 1.5. Majority of NHL are mature B-cell neoplasm (83.1%), while mature T-cell and NK cell neoplasm are less (14.6%). Most common subtype found is Diffuse large B-cell lymphoma (52.8%) followed by Primary diffuse large B cell lymphoma of CNS (15.7%). Primarily nodal Vs extranodal involvement is seen in 51.9% and 48% of cases. Most common extranodal site is CNS followed by GIT and spine. Conclusion: In the present study DLBL is the most common subtype seen in 52.8% of cases in concordance with other studies but the sticking difference here is low incidence of follicular lymphoma and CLL/SLL and a much higher incidence of Primary DLBCL of CNS. There is an Inherited selection bias because the cases are collected from single institution; however, causes and reasons should be more extensively investigated.
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ABSTRACT Introduction: Involvement of the peritoneum occurs very rarely and is exceptional as an exclusive extranodal presentation of lymphomas. In most cases lymphomas associated with this rare entity are high-grade ones. PL secondary to high-grade nodal lymphoma is more frequent than primary peritoneal lymphoma, and there are only a few cases of the latter described in the literature. Description of the case: We present the case of a patient with constitutional syndrome and imaging findings suggestive of peritoneal carcinomatosis who was finally diagnosed with a Diffuse Large B-cell Lymphoma (DLBCL) by an ultrasound-guided core needle biopsy (CNB) of peritoneum. The patient received one polychemotherapy cycle; however tumor lysis syndrome occurred with death of the patient in the following days. This case tries to show the existence of a PL without other radiological findings of lymphoma, a fact that is very exceptionally described in the literature. Discussion: The differential diagnosis between PL and others peritoneum diseases such as peritoneal carcinomatosis, malignant primary peritoneal mesotheliomas, tuberculous peritonitis, sarcomatosis, diffuse peritoneal leiomyomatosis or benign splenosis, constitutes a major problem in imaging techniques. An exhaustive analysis of the radiological characteristics as well as a clinical-analytical context allows the differential diagnosis against peritoneal carcinomatosis and the rest of the entities previously referred although the final diagnosis will always be a biopsy. Conclusion: PL usually manifests as an aggressive histological subtype of high-grade lymphomas leading to a rapid progression and deterioration of the patient. It is crucial for the radiologist and the clinician to be aware of this rare entity providing the earliest possible diagnosis and optimal treatment to prolong the patient's life.
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Humans , Male , Aged , Lymphoma, Non-Hodgkin , Lymphoma, Large B-Cell, Diffuse , Peritoneal NeoplasmsABSTRACT
Background: Primary extra-nodal lymphomas (ENLs) are rare neoplasm but can affect many organ systems of body. Proper and timely diagnosis and justified treatment of primary ENL are often affected due to variety of lymphoma types and the relative rarity of many of these tumor types. They are mainly of Non-Hodgkin lymphoma which further consists of many subtypes having variation of epidemiology and clinical presentation along with geographic distribution. Aim and Objectives: Aim and objectives of the study were to find out topographical distribution and histological patterns of ENL along with their immunohistochemical character in a tertiary care hospital of Eastern India. Materials and Methods: All the cases from April 2015 to March 2018 diagnosed as ENL by histopathological examination were included in this descriptive cross-sectional study for further immunohistochemical analysis for CD45 Life-cycle assessment (LCA), CD3 and CD 20 to identify the cell of origin (B cell vs. T cell). Results: Out of total 31 cases, 67.74% were male and the most common age group was 51–60 years. Gastrointestinal tract was the most common organ system (61.29% of cases) affected and stomach (29.03%) followed by Ileum (9.67%) were the most common sites affected by ENL. Histomorphologically diffuse large cell lymphoma was the most common pattern while immunohistochemistry study revealed all the cases were B cell type with LCA and CD 20 positive and CD3 negative. Conclusion: This study revealed the topographical pattern along with predominant histomorphological and immunohistochemical character of ENL in study population. However, further studies with meta- analysis can enlighten these characters more comprehensively.
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Background: The eighth edition of the American Joint Committee on Cancer (AJCC) for oral cancer has incorporated additional pathological features like depth of invasion (DOI) and extranodal extension (ENE) into T and N staging. The incorporation of these two factors will impact the staging and, hence, the treatment decisions. The aim of the study was to clinically validate the new staging system in predicting the outcome in patients treated for carcinoma oral tongue. The study also examined the correlation of pathological risk factors with survival. Methods: We studied 70 patients with squamous cell carcinoma of the oral tongue who underwent primary surgical treatment at a tertiary care center in the year 2012. All these patients were restaged pathologically according to the new AJCC eighth staging system. The 5-year overall survival (OS) and disease-free survival (DFS) were calculated using the Kaplan–Meier method. Akaike information criterion and concordance index were calculated between both staging systems to identify a better predictive model. Log-rank test and univariate Cox regression analysis were conducted to find out the significance of different pathological factors on outcome. Results: Incorporation of DOI and ENE resulted in 47.2% and 12.8% stage migration, respectively. DOI less than 5 mm was associated with a 5-year OS and DFS of 100% and 92.9%, respectively, compared to 88.7% and 85.1%, respectively, when the DOI was more than 5 mm. Presence of lymph node involvement, ENE, and perineural invasion (PNI) were associated with inferior survival. The eighth edition had lower Akaike information criterion and improved concordance index values compared with the seventh edition. Conclusion: The eighth edition of AJCC allows better risk stratification. Restaging of cases based on the eighth edition AJCC staging manual resulted in significant upstaging with difference in survival.
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Resumen ANTECEDENTES: La afectación primaria del aparato genital femenino por un linfoma no Hodgkin es excepcional, se reporta en 0.2 a 1.1% de los casos. Los órganos afectados con más frecuencia son los ovarios, seguidos del cuello uterino; el endometrio y la vagina son los menos aquejados. Debido a su baja frecuencia aún no se llega a un consenso del tratamiento más adecuado de estos linfomas; hasta ahora, por lo general, se ha individualizado para cada caso en concreto. CASO CLÍNICO: Paciente de 29 años que consultó por sangrado genital anómalo e incapacidad para la inserción de tampones vaginales. En la exploración se encontró una masa pélvica, palpable a través de la vagina, que impresionaba al infiltrar toda la luz vaginal y la parte media e izquierda de la vulva. En los estudios de imagen se objetivó una gran masa pélvica de hasta 10 centímetros que parecía depender del cuello del útero y que se extendía e infiltraba el canal vaginal, la vulva y el tercio inferior de la vejiga. El reporte anatomopatológico de la biopsia fue: infiltración de pared vaginal por un linfoma no Hodgkin B difuso de células grandes. Se le indicaron seis ciclos de quimioterapia con ciclofosfamida, vincristina, adriamicina y prednisona con los que se consiguió la remisión metabólica completa. CONCLUSIÓN: El diagnóstico del linfoma genital primario puede resultar complejo por la posibilidad de simular una neoplasia ginecológica. En casos de enfermedad avanzada, la manifestación clínica más frecuente es el sangrado genital anómalo. El esquema de tratamiento más aceptado en la actualidad es con rituximab-ciclofosfamida, vincristina, adriamicina, prednisona seguido de radioterapia de consolidación.
Abstract BACKGROUND: Primary involvement of the female genital tract by non-Hodgkin's lymphoma is exceptional, reported in 0.2 to 1.1% of cases. The most frequently affected organs are the ovaries, followed by the cervix; the endometrium and vagina are the least affected. Due to their low frequency, there is still no consensus on the most appropriate treatment of these lymphomas; until now, it has generally been individualized for each specific case. CLINICAL CASE: A 29-year-old female patient consulted for abnormal genital bleeding and inability to insert vaginal tampons. On examination a pelvic mass was found, palpable through the vagina, which impressed by infiltrating the entire vaginal lumen and the middle and left side of the vulva. Imaging studies showed a large pelvic mass of up to 10 centimeters that appeared to be dependent on the cervix and that extended and infiltrated the vaginal canal, the vulva and the lower third of the bladder. The anatomopathological report of the biopsy was: infiltration of the vaginal wall by diffuse large cell non-Hodgkin's B lymphoma. She was prescribed six cycles of chemotherapy with cyclophosphamide, vincristine, adriamycin and prednisone with which complete metabolic remission was achieved. CONCLUSION: The diagnosis of primary genital lymphoma can be complex because of the possibility of simulating a gynecologic neoplasm. In cases of advanced disease, the most frequent clinical manifestation is abnormal genital bleeding. The most accepted treatment scheme at present is rituximab-cyclophosphamide, vincristine, adriamycin, prednisone followed by consolidation radiotherapy.
ABSTRACT
Objective:To investigate the quality of life in patients with early-stage extra-nodal NK/T cell lymphoma of nasal type (ENKTL) arising from the upper aerodigestive tract, who had remained progression-free survival (PFS) for over 3 months after definitive intensity-modulated radiation therapy (IMRT), and to analyze the factors related to main adverse symptoms.Methods:276 patients who received IMRT from March, 2012 to June, 2021 were included. There were 201 males and 75 females with a median age of 41.5 years (range: 13-81 years) upon diagnosis. Consistent target delineation schemes and similar dose gradients were adopted for IMRT, with a median prescribed dose of 54.6 Gy/26F. Cross-sectional investigation was performed with a modified EORTC QLQ-H&N35 questionnaire, the incidence and severity of adverse symptoms, severity of disease and their influencing factors at each time-point during their survival were statistically analyzed.Results:The median age of patients at the investigation was 46.2 years, and the median PFS after IMRT was 47.2 months (range: 3.1-115.7 months). The most common adverse symptoms included nasal symptoms (incidence rate 63.8%), dry mouth (50%), tooth diseases (47.1%), smell and taste alteration, and sexual apathy, etc. Most symptoms were mild (the average standardized score was 5.50, the full score of 100 indicating the most severe), and could be relieved remarkably over survival time, but some symptoms, such as tooth diseases and sexual apathy, were more obvious and recurred for several years. Age and anti-PD-1 immune therapy influenced the symptom scores, and tooth diseases were closely correlated with dry mouth. Conclusion:The quality of life in patients with early-stage ENKTL after definitive IMRT is high, and the most significant symptoms include nasal symptoms, tooth diseases, and sexual apathy, etc. , which need to be mitigated with more studies.