[Efficacy and safety analysis of P-GemDOx regimen and stratified prognosis in patients with early extranodal NK/T cell lymphoma].

Objective: To assess the efficacy, safety, and related prognostic factors associated with the P-GemDOx regimen as a first-line treatment for patients with early-stage extranodal natural killer (NK) /T cell lymphoma (ENKTL) . Methods: A retrospective analysis was performed on sixty early-stage ENKTL patients treated with the P-GemDOx regimen who were admitted to the First Affiliated Hospital of Nanjing Medical University between August 2015 and May 2021. The Chi-square test or Fisher's exact test was used to compare group differences, and the Log-rank test was used to compare the differences in survival. Survival outcomes and prognostic factors were examined. Results: After completing 4 to 6 cycles of P-GemDOx chemotherapy, the overall response rate (ORR) was 88.3%, with forty-six patients (76.7% ) achieving complete response (CR). The 4-year progression-free survival (PFS) and overall survival (OS) rates were (66.3±7.1) % and (79.5±6.0) %, respectively. According to the PINK/PINK-E model, there was no significant difference in survival outcomes among risk groups. 23.3% of patients experienced progression of disease within 24 months (POD<24). OS estimates differed significantly (P<0.001) between the POD<24 group (n=14) and the POD≥24 group (n=46). Analysis showed that SUVmax > 12.8 at diagnosis, non-single nasal cavity infiltration, and response less than CR after 4-6 cycles all had a significant association with POD24. We used these data as the basis for predicting POD<24 international prognostic index (POD24-IPI). Patients were stratified into low-risk (no risk factors), intermediate-risk (one risk factor), or high risk (two or three risk factors). These groups were associated with 4-year OS rate of 100%, (85.6±9.7) %, and (65.0±10.2) %, respectively (P=0.014). The P-GemDOx regimen was well tolerated, with hematological toxicity being the main side effect. Conclusion: This study demonstrated that the P-GemDOx regimen is effective and safe in the first-line treatment of early-stage ENKTL, and POD24-IPI is a promising prognostic model.

Wait and see: a case of EBV + cutaneous extranodal NK/T-type lymphoma with indolent behaviour.

Extranodal NK/T-cell lymphoma (ENKTL) is a rare lymphoma subtype associated with Epstein-Barr virus (EBV) infection, portending a poor prognosis despite systemic chemotherapy. We present the unusual case of an 85-year-old man receiving ibrutinib for mantle cell lymphoma, who developed a erythematous, subcutaneous nodule on the forehead, featuring a proliferation of pleomorphic CD8 + /CD56 - /EBV + cells. Given the negative staging and comorbidities, a watchful waiting strategy was performed, experiencing a benign course with self-resolution and complete remission over a 4-year follow-up. The literature on primary cutaneous ENKTL has been discussed, with particular attention to clinical and histological prognostic factors.

[Analysis of prognostic factors of extranodal NK/T-cell lymphoma treated with pegaspargase/L-asparaginase: a multicenter retrospective study].

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.

Diagnosis and management of rare paediatric Non-Hodgkin lymphoma.

Mature B-cell lymphomas, (B- or T-cell) lymphoblastic lymphomas (LBL), and anaplastic large cell lymphoma (ALCL) correspond to about 90% of all non-Hodgkin lymphoma (NHL) cases occurring in children and adolescents. The remaining 10% encompass a complex group of entities characterized by low/very low incidences, paucity of knowledge in terms of underlying biology in comparison to their adult counterparts, and consequent lack of standardization of care, information on clinical therapeutic efficacy and long-term survival. At the Seventh International Symposium on Childhood, Adolescent and Young Adult NHL, organized on October 20-23, 2022, in New York City, New York, US, we had the opportunity to discuss clinical, pathogenetic, diagnostic, and treatment aspects of certain subtypes of rare B- or T-cell NHL and they will be the topic of this review.

Clinical analysis of seven cases of hemophagocytic lymphohistiocytosis secondary to cutaneous T-cell lymphoma / 中华皮肤科杂志

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.

Efficacy and safety of SIMPLE regimen in treatment of extranodal NK/T-cell lymphoma / 白血病·淋巴瘤

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.

Analysis of prognostic factors of extranodal NK/T-cell lymphoma treated with pegaspargase/L-asparaginase: a multicenter retrospective study / 中华血液学杂志

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.

Gastric CD56-negative Extranodal Natural Killer/T-cell Lymphoma: A Case Report.

Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL-NT) is the most common subtype of Epstein-Barr virus-associated NK/T-cell lymphomas. ENKTCL-NT occurs infrequently in the gastrointestinal tract. In particular, reports of ENKTCL-on NT arising from the stomach are extremely rare. Several clusters of differentiation (CDs) have been useful in recognizing NK-cells, T-cells, and tumor cells of NK/T-cell lymphomas. Among them, the CD56 antigen is considered the most sensitive marker for ENKTCL-NT and is expressed in almost all cases of ENKTCL-NT. Thus, the development of CD56-negative ENKTCL-NT is highly atypical. This paper reports a case of a young Asian female who presented with gastric ulcer bleeding. The patient was histologically diagnosed with ENKTCL-NT. No tumor cells for CD56 were observed, whereas no monoclonality of the T-cell receptor gamma gene rearrangement was detected in the tumor cells. The patient was scheduled for systemic chemotherapy six times and achieved complete remission. Peripheral blood-hematopoietic stem cell transplantation was performed later.

Value of pretreatment albumin in prognostic assessment of extranodal nasal type NK/T cell lymphoma / 肿瘤研究与临床

Objective:To explore the prognostic value of pretreatment albumin in extranodal nasal type NK/T cell lymphoma (ENKTL).Methods:The clinical data of 184 ENKTL patients in Shanxi Province Cancer Hospital from January 2002 to December 2018 were retrospectively analyzed. The Contal-O'Quigley change point method was used to determine the optimal cut-off value of albumin for predicting the prognosis of patients. The propensity score matching (PSM) was used to minimize selection biases. The Kaplan-Meier method was used for survival analysis, and Cox proportional hazards model was used to determine the factors affecting survival. The time-dependent receiver operating characteristic curve, Akaike information criterion and integrated Brier score were used to evaluate the efficacy of international prognostic index (IPI), Korean prognostic index (KPI) and prognostic index of NK cell lymphoma (PINK) models incorporating albumin for predicting the prognosis of patients.Results:The optimal cut-off value of pretreatment albumin for predicting the prognosis of ENKTL patients was 37.5 g/L. The 3-year and 5-year overall survival (OS) rates in >37.5 g/L group (126 cases) were 66.2% and 60.3%, and the progression-free survival (PFS) rates were 58.8% and 49.6%; the 3-year and 5-year OS rates in ≤37.5 g/L group (58 cases) were 35.0% and 32.4%, and the PFS rates were 32.5% and 30.0%. The OS and PFS in > 37.5 g/L group were better than those in ≤37.5 g/L group (both P<0.001). After PSM, the OS and PFS in >37.5 g/L group were still better than those in ≤37.5 g/L group (both P = 0.002). Multivariate analysis showed that albumin was an independent influencing factor for OS ( RR = 0.419, 95% CI 0.266-0.660, P < 0.001) and PFS ( RR = 0.493, 95% CI 0.322-0.755, P < 0.001). After PSM, albumin was still an independent influencing factor for OS ( RR = 0.305, 95% CI 0.156-0.598, P = 0.001) and PFS ( RR = 0.341, 95% CI 0.185-0.627, P = 0.001). The prognostic prediction performance of the IPI, KPI and PINK models incorporating albumin were all improved. Conclusions:Pretreatment albumin is an important prognostic indicator for ENKTL.

Extranodal NK/T-cell lymphoma, nasal type with extensive cardiopulmonary involvement.

Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin's lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.

Extranodal NK/T-cell lymphoma, nasal type with extensive cardiopulmonary involvement

Extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT) is a rare type of Non-Hodgkin's lymphoma, which usually presents with extranodal involvement and affects the nasal/upper aerodigestive tract in the classical presentation. Herein, we report the case of a 31-year-old, previously healthy, male patient diagnosed with ENKTL-NT with the involvement of the lung parenchyma and heart. Unfortunately, due to the rapid disease progression, the diagnosis was performed only at the autopsy. The authors highlight the rare clinical presentation of this type of lymphoma, as well as the challenging anatomopathological diagnosis in necrotic samples.

Prognostic value of absolute lymphocyte count in patients with extranodal nasal NK/T cell lymphoma / 白血病·淋巴瘤

Objective:To investigate the relationship between absolute lymphocyte count (ALC) and prognosis of extranodal nasal NK/T cell lymphoma (ENKTL).Methods:The data of 184 patients with ENKTL who were admitted to Shanxi Provincial Cancer Hospital and the Second People's Hospital of Datong in Shanxi Province from January 2002 to December 2018 were retrospectively analyzed. Contal-O'Quigley variable point method was used to calculate the optimal cut-off value of ALC for predicting the prognosis of ENKTL, and the patients were divided into two groups based on this value. Kaplan-Meier method was used to analyze the survival of the two groups of patients. Cox proportional hazard regression model was used for univariate and multivariate survival analyses.Results:According to the Contal-O'Quigley variable point method, the best cut-off value of ALC for predicting ENKTL prognosis was 1.5×10 9/L. According to this value, patients were divided into ALC>1.5×10 9/L group (98 cases) and ALC≤1.5×10 9/L group (86 cases). Compared with the ALC≤1.5×10 9/L group, the proportions of patients with Eastern Cooperative Oncology Group (ECOG) score ≥2 points, serum lactate dehydrogenase (LDH)> 245 U/L, Korean prognostic index (KPI) score 2-4 points and international prognostic index (IPI) score 2-5 points were lower in the ALC>1.5×10 9/L group, but the proportion of patients with hemoglobin > 120 g/L was higher (all P < 0.05). The overall survival (OS) and progression-free survival (PFS) in the ALC>1.5×10 9/L group were better than those in the ALC≤1.5×10 9/L group (3-year OS rate: 65.7% vs. 45.4%, 5-year OS rate: 61.0% vs. 36.6%; 3-year PFS rate: 61.7% vs. 41.0%, 5-year PFS rate: 51.9% vs. 32.2%; all P < 0.05). Multivariate Cox regression analysis showed that ECOG score, serum LDH level, ALC, radiotherapy and L-asparaginase-containing chemotherapy were independent influencing factors of OS and PFS (all P < 0.05), and for OS, when ALC>1.5×10 9/L compared with≤1.5×10 9/L, RR was 0.634 (95% CI 0.398-1.008, P=0.050), and for PFS, RR was 0.625 (95% CI 0.406-0.962, P=0.033). Conclusion:Patients with high ALC have good prognosis, and therefore ALC may be a powerful prognostic factor of ENKTL.

[Expression of pSTAT3 and PD-L1 in extranodal NK/T cell lymphoma and its clinical significance].

Objective: To study the expression of phosphates signal transducer and activator of transcription 3 (pSTAT3) and programmed death ligand-1 (PD-L1) in extranodal NK/T cell lymphomas (ENKTCL) and the relationships of pSTAT3 and PD-L1 expression with the clinicopathological characteristics and prognosis of ENKTCL. Methods: Fifty-one cases of ENKTCL diagnosed at Guangdong Provincial People's Hospital from June 2015 to February 2019 were included in the study. The expression of pSTAT3 and PD-L1 was examined using immunohistochemistry. Results: There were 35 males and 16 females, ranging from 18 to 85 years old with a median age of 47 years. The positive rates of pSTAT3 and PD-L1 expression were 68.6% (35/51) and 76.5% (39/51), respectively. pSTAT3 expression was correlated with PD-L1 expression (P=0.033,R=0.322), while there were no associations of pSTAT3 and PD-L1 expression with the clinicopathological characteristics of ENKTCL, including age, sex, clinical site, B symptom, Ann Arbor stage, LDH value, EBV DNA load of peripheral blood and international proliferation index score. Kaplan-Meier survival analysis showed the prognoses of the pSTAT3 and PD-L1 positive groups were slightly better than the respective negative groups, but the differences were not significantly (P>0.05). Conclusions: pSTAT3 is highly expressed in extranodal NK/T cell lymphoma and related to the expression of PD-L1, which provides a potential target and rationale for combinations of targeted therapies and immune checkpoint blockade inhibitors in the treatment of ENKTCL.

Sepsis por escherichia coli de inicio infrecuente en paciente con linfoma de células T y NK / Sepsis By Escherichia Coli Of Uncommon Starting In Patient With T And NK Cell Lymphoma

La sepsis se da en presencia de un foco infeccioso; en este caso por Escherichia coli (E.coli), las cuales poseen toxinas que pueden causar daños severos en la funcionalidad y morfología del organismo, viéndose empeorado en pacientes con enfermedades que afecten al sistema inmunitario; como es este caso en presencia de neoplasia hematológica. Paciente femenina de 42 años , con diagnóstico de Linfoma no Hodgkin de células T-NK presenta una placa necrótica en el paladar, temperatura por encima de 38°C, frecuencia respiratoria superando las 20 respiraciones por minutos y frecuencia cardíaca superando los 90 latidos por minuto, al examen de cultivo faríngeo positivo a E.coli, urocultivo negativo, confirmando punto partida infeccioso faríngeo; tratada con amikacina vía endovenosa durante 7 días con buena respuesta a la antibioticoterapia . Se reporta un caso infrecuente por este tipo de bacteria en zona bucal y revisión del tema.

Sepsis occurs in the presence of an infectious focus; in this case by Escherichia coli (E. coli), that toxins can cause severe damage to the organism's functionality and morphology, being worsened in patients with diseases that affect the immune system; as is this case in the presence of hematological malignancy. A 42-year-old female patient with a diagnosis of T-NK non-Hodgkin lymphoma presented with a necrotic plaque on the palate, temperature above 38 ° C, respiratory rate exceeding 20 breaths per minute, and heart rate exceeding 90 beats per minute. , on examination of a positive pharyngeal culture for E. coli, negative urine culture, confirming a pharyngeal infectious starting point; Treat with amikacin endovenously for 7 days with good response to antibiotic therapy. An infrequent case is reported for this type of bacteria in the oral area and a review of the subject.

[Advances in immunotherapy of extranodal NK/T cell lymphoma].

Extranodal NK/T-cell lymphoma (ENKTCL) is a relatively rare group of highly aggressive non-Hodgkin's lymphoma (NHL). The disease has rapid clinical progress, high degree of malignancy and poor prognosis. Traditional chemoradiotherapy regimens have not shown good efficacy. In recent years, the immunotherapy of tumors has developed rapidly. At present, it has shown strong therapeutic activity in the treatment of various solid tumors such as non-small cell lung cancer, prostate cancer, melanoma and kidney cancer. Multiple tumor immunotherapy drugs have been approved by the US Food and Drug Administration (FDA) for clinical use. This article reviews recent novel immunotherapeutic regimens of ENKTCL, hoping to change the treatment modality of this malignant disease.

[14-3-3ζ protein mediates gemcitabine resistance in NK/T-cell lymphoma].

Objective: To explore the molecular mechanisms of 14-3-3ζ in gemcitabine resistance in extranodal NK/T-cell lymphoma, nasal type (ENKTL) . Methods: The effects of cell proliferation and invasion were detected by cell counting kit-8 (CCK-8) assay and transwell assay. YTS cells were exposed to gradually increased concentrations of gemcitabine to establish gemcitabine-resistant YTS cells (YTS-gem) in vitro. 14-3-3ζ specific siRNA lentiviral vector was transfected into YTS and YTS-gem cells to downregulate 14-3-3ζ expression, and stable transfected cell clones were screened. The protein expression was determined by Western blot. Results: ①14-3-3ζ expression was significantly up-regulated in gemcitabine resistant YTS-gem cells, comparing with that of YTS cells (P<0.05) . ②The results of CCK-8 and transwell assay showed that downregulation of 14-3-3ζ significantly reduced the cell proliferation and invasion abilities (P<0.05) . ③Downregulation of 14-3-3ζ could restore gemcitabine sensitivity in gemcitabine resistant YTS-gem cells (P<0.05) . ④Western blotting results showed that knockdown of 14-3-3ζ significantly upregulated pro-apoptotic Bax, and downregulated anti-apoptotic Bcl-2, Caspase-3, cleaved caspase-3, Cyclin D1 in gemcitabine-resistant YTS-gem cells (P<0.05) . There was no significant difference in p53 ang P-gp expression levels. Conclusions: 14-3-3ζ was upregulated in gemcitabine resistant YTS cells. Overexpression of 14-3-3ζ promoted cell proliferation and enhanced cell migration. 14-3-3ζ contributed to gemcitabine resistance to ENKTL through anti-apoptosis.

[Long-term effect of risk-adaptive therapy for early stage extranodal NK/T cell lymphoma].

Objective: This study aimed to evaluate the role of extended involved-field intensity modulated radiation therapy (IMRT) for patients with early stage extranodal nasal-type NK/T-cell lymphoma (NKTCL) in a single center, and to explore the long-term effect of risk-adaptive therapy. Methods: Among 238 patients with early stage NKTCL, there were 191 in high-risk group [any risk factor of age > 60, elevated serum lactate dehydrogenase (LDH), Eastern Cooperative Oncology Group (ECOG) score≥2, primary tumor invasion, or Ann Arbor stage Ⅱ] and 47 in low-risk group (no risk factor). A total of 204 patients received radiotherapy combined with chemotherapy, 15 received radiotherapy alone and 19 received chemotherapy alone. One-hundred and eighty-six patients had radiotherapy at a dose ≥50 Gy and 159 patients received chemotherapy with asparaginase-based regimen. Results: The 5-year overall survival (OS) rate and progression- free survival (PFS)rate of all patients were 66.2% and 57.5%. Five-year OS and PFS rates in low-risk group were 91.8% and 88.0%, while 59.3% and 49.3% in high-risk group. The survival of patients in low-risk group were better than those in high-risk group (both P<0.001). The five-year OS rate in combined therapy group, radiotherapy group and chemotherapy group were 71.7%, 52.3% and 20.7%, respectively (P<0.001). The five-year PFS rate were 63.5%, 23.3% and 24.1%, respectively (P<0.001). Among 219 patients receiving radiotherapy, the 5-year OS and PFS rate of patients with primary site radiotherapy dose ≥ 50 Gy were 72.6% and 66.1%, higher than those of patients with a dose <50 Gy (57.3% and 30.9%, respectively; P=0.031, P<0.001). In the high-risk group, the 5-year OS and PFS rate of the patients who received radiotherapy combined with more than 4 cycles of chemotherapy were 66.5% and 62.6%, higher than those of patients received less than 4 cycles of chemotherapy (56.7% and 46.4%, P=0.045 and 0.020, respectively). Cox multivariate analysis showed that ECOG score (HR=2.208, P=0.001), primary site (HR=4.887, P<0.001), primary tumor invasion (HR=3.265, P=0.001) and radiation dose (HR=1.895, P=0.011) were independent factors of OS in early NKTCL patients. Conclusion: Extended-involved field IMRT with radiation dose more than 50 Gy was the main treatment for patients with early stage NKTCL. Radiotherapy combined with adequate cycle chemotherapy significantly improved prognosis of high-risk patients.

[Ocular natural killer/T cell lymphoma: a clinicopathologic analysis].

Objective: To evaluate the clinicopathological features of ocular natural killer(NK)/T cell lymphoma. Methods: Data of 21 patients (22 eyes) with ocular NK/T cell lymphoma treated at Eye & ENT Hospital of Fudan University from January 2006 to March 2018 were retrospectively analyzed for clinical data, morphology, immunophenotype and outcomes. Results: There were 10 males and 11 females with ages from 3 to 77 years (mean, 43 years). There were 20 unilateral cases (10 left eyes and 10 right eyes) and 1 bilateral case. Except for 1 case of corneal perforation resulting from the involvement of the conjunctiva and cornea, the other cases all involved the orbit (including eyelids and conjunctiva) as demonstrated by radiologic studies, with the lacrimal sac involved in 3 cases, and the nasal cavity or maxillary sinus involved in 2 cases. Three patients had been previously diagnosed sinonasal NK/T cell lymphoma with radiotherapy and chemotherapy. Two patients had a history of ovarian NK/T cell lymphoma with chemotherapy. One patient had multiple ulcers of skin and mucosa at presentation. There were 13 primary ocular NK/T cell lymphomas without evidence of nasal or systemic involvement. All patients presented with eyelid swelling and decreased visual acuity. There were proptosis in 18 cases, motility restriction in 13 cases, eyelid ulceration in 3 cases, and fever in 4 cases. They had all been previously diagnosed as orbital pseudotumor or cellulitis and there was no response to steroids and antibiotics. Pathological examination showed atypical lymphoid infiltration with an angioinvasive growth pattern causing coagulative necrosis. Cytologically, the medium-sized neoplastic cells showed irregular folded nuclei. The neoplastic cells were positive for cytoplasmic CD3ε, CD56, and cytotoxic molecules and Epstein-Barr virus-encoded RNA (EBER) in situ hybridization. Seven patients were lost to follow-up. Ten patients died 2.0 to 17.0 months after diagnosis (mean, 6.3 months) despite treatment with chemotherapy and radiotherapy. Conclusions: Ocular NK/T cell lymphoma is a rare form of ocular lymphoma. There are primary NK/T cell lymphoma and secondary ocular NK/T cell lymphoma with nasal or systemic involvement. The rarity of this tumor and inflammatory signs make it challenging to identify these tumors early. The neoplastic cells are positive for cytoplasmic CD3ε, CD56, cytotoxic molecules and EBER in situ hybridization. Despite aggressive therapy, it demonstrates high lethality with poor prognosis. (Chin J Ophthalmol, 2019, 55: 374-380).

Classification for primary cutaneous lymphoproliferative disorders: an update / 中华皮肤科杂志

Primary cutaneous lymphoproliferative disorders are a group of heterogeneous diseases that mainly involve the skin and are completely different from lymph node lymphomas.Although rare,they are still an important part of lymphoid and hematopoietic tumors.In the 2017 World Health Organization (WHO) classification for tumors of hematopoietic and lymphoid tissues and the 2018 updated version of WHO-European Organization for the Research and Treatment of Cancer (EORTC) classification for cutaneous lymphomas,classifications of most diseases do not change,but the understanding of some diseases such as Epstein-Barr virus-associated lymphoproliferative diseases and primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder has been changed,and new diseases have been added.This article introduces updates on the classification of primary cutaneous lymphoproliferative disorders based on the 2017 WHO and 2018 WHO-EORTC classification systems.

Pulmonary extranodal NK/T-cell lymphoma, nasal type: a clinicopathological analysis of eleven cases / 中华病理学杂志

Objective@#To investigate the clinicopathological features, diagnosis and differential diagnosis of pulmonary extranodal NK/T-cell lymphoma, nasal type (ENKTL-NT).@*Methods@#Eleven cases of newly diagnosed (10 puncture biopsies and 1 transbronchial biopsy), previously untreated pulmonary ENKTL-NT were collected at the First Affiliated Hospital of Zhengzhou University, from August 2013 to November 2018. The clinicopathological features including histomorphology, immunohistochemistry and in situ hybridization were collected and analyzed.@*Results@#Among the 11 cases, 8 were males and 3 were females, with a male to female ratio of 8∶3.The age range was from 30 to 74 years, with an average of 48 years and a median of 43 years. Tumors involved bilateral lung lobes in 8 cases, the upper left lobe in 1 case, lower left lobe in 1 case, and upper right lobe in 1 case. Main clinical symptoms included fever, often accompanied by cough, and bloody sputum in most cases. All cases were stage Ⅳ E. Histological features included scattered or focal aggregates of marked pleomorphic tumor lymphocytes, accompanied by necrosis and heavy admixture of inflammatory cells. In a few cases, diffuse neoplastic lymphocytes or vascular central and destructive infiltrations were seen. Tumor cells in most cases expressed CD3ε, CD3, CD43, CD56, TIA-1, granzyme B, but did not express CD20, CD79a, and CD5. Ki-67 index ranged from 40%to 90%.All cases were positive for EBER by in situ hybridization. Four of five patients died during follow-up with a survival period of only 1 week to 13 months.@*Conclusions@#Pulmonary ENKTL-NT is rare, high grade malignancy with a poor prognosis. Misdiagnosis is common due to lesional necrosis and heterogeneous cell components. Immunohistochemistry and EBER in situ hybridization are essential for accurate diagnosis.