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Objective:To compare the efficacy of concurrent and asynchronous radiochemotheray for early extranodal nasal natural killer/T-cell lymphoma (NKTCL).Methods:From 2007 to 2020, 278 patients with early NKTCL treated with comprehensive treatment in the Affiliated Tumor Hospital of Guizhou Medical University were recruited. According to the adjusted Nomogram-revised risk index (NRI) prognostic model, there were 49 cases in the good prognostic group without adverse prognostic factors (age>60 years old, increased serum lactate dehydrogenase (LDH), ECOG score ≥2, primary tumor invasion (PTI), Ann Arbor stage Ⅱ, and 229 cases in the poor prognostic group with any adverse prognostic factors. 145 of these cases were treated with concurrent radiochemotherapy, and 133 of them were treated with asynchronous radiochemotherapy.Results:The 5-year overall survival (OS) rate of the whole group was 71.0%, and the progression-free survival (PFS) rate was 67.6%. The 5-year OS rate in the good prognostic group was 95.6%, and 65.4% in the poor prognostic group ( P<0.001). In the poor prognostic group, the 5-year OS rates of patients with NRI=1(low-and moderate-risk group), NRI=2(moderate-and high-risk group), NRI≥3(high-risk group) were 72.1%, 61.1% and 47.7%, respectively ( P=0.007). There was no significant difference in curative effect between the concurrent and asynchronous radiochemotherapy groups. The 5-year OS rates were 70.6% and 69.8%( P=0.783), and the 5-year PFS rates were 67.6% and 65.2%( P=0.631). Further stratified analysis showed that the 5-year OS rates of patients with NRI=1 receiving concurrent and asynchronous radiochemotherapy were 73.1% and 76.5%( P=0.576), 62.6% and 69.3%( P=0.427) for those with NRI=2, and 58.1% and 42.3% for those with NRI≥3( P=0.954). Conclusions:Comprehensive treatment can significantly improve the prognosis of early NKTCL in the poor prognostic group. In the sequence of radiotherapy and chemotherapy, there is no significant difference in 5-year OS and PFS rates between concurrent and asynchronous radiochemotherapy. Sequential treatment with better tolerance can be adopted for early NKTCL with poor prognosis.
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Extranodal natural killer(NK)/T cell lymphoma, nasal type (ENKTL) is a type of highly aggressive non-Hodgkin lymphoma, closely associated with Epstein-Barr virus (EBV) infection in terms of carcinogenesis, and is prevalent in East Asia and South America.Clinicopathologically, ENKTL is characterized by diffuse tumor cell infiltration, angiocentral destructive growth pattern, and prominent tissue necrosis.Typically, ENKTL presents with extranodal involvements, particularly the upper aerodigestive tract, such as nasal cavity, nasopharynx, paranasal sinuses.Great advances has been made in the last decade regarding the molecular pathogenesis, refined tumor staging and risk assignment, and treatment strategies including local radiotherapy, concurrent chemoradiotherapy and sandwich therapy.Accordingly, response rate and long-term prognosis increase remarkably for stage Ⅰ and stage Ⅱ tumors, though clinical outcomes remains relatively poor for advanced ENKTL.Novel immunotherapy has been proving to be an a promising treatment modality for relapsed or refractory ENKTL.These relevant advances are reviewed in the present paper.
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Extranodal natural killer(NK)/T cell lymphoma,nasal type (ENKTL) is a type of highly aggressive non-Hodgkin lymphoma,closely associated with Epstein-Barr virus (EBV) infection in terms of carcinogenesis,and is prevalent in East Asia and South America.Clinicopathologically,ENKTL is characterized by diffuse tumor cell infiltration,angiocentral destructive growth pattern,and prominent tissue necrosis.Typically,ENKTL presents with extranodal involvements,particularly the upper aerodigestive tract,such as nasal cavity,nasopharynx,paranasal sinuses.Great advances has been made in the last decade regarding the molecular pathogenesis,refined tumor staging and risk assignment,and treatment strategies including local radiotherapy,concurrent chemoradiotherapy and sandwich therapy.Accordingly,response rate and long-term prognosis increase remarkably for stage Ⅰ and stage Ⅱ tumors,though clinical outcomes remains relatively poor for advanced ENKTL.Novel immunotherapy has been proving to be an a promising treatment modality for relapsed or refractory ENKTL.These relevant advances are reviewed in the present paper.
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Objective To investigate the clinicopathologic features of inert natural killer/T (NK/T)-cell lymphoma with CD30-positive large cell transformation. Methods The pathological data of one patient diagnosed as inert NK/T-cell lymphoma with CD30-positive large cell transformation in the Third Hospital Affiliated to Soochow University from April 2006 to March 2017 were collected. The histopathological features and immunohistochemical phenotype of the patient were observed and followed up. Results The patient received biopsy twice in April 2006 and March 2017. The first tumorectomy in sacral canal showed that diffuse and small lymphoid cell hyperplasia, irregular nucleus, granular chromatin, unobvious nucleolus, focal necrosis. Immunohistochemistry showed CD20(-), CD3(-), ALK(-), CD30(-), CD43(+), CD2(+), CD56(+), TIA-1(+), GrB(+), CD4(-), CD8(-). Finally, the patient was diagnosed as extranodal NK/T-cell lymphoma, nasal type. The microscopic examinations after the second left cervical lymph node biopsy showed large cells infiltrated into the background of small lymphocytes, plentiful cytoplasm, large nuclei, irregular nucleus, part of nuclear folding, obvious nucleolus, mitotic figures visible, visible intravascular tumor suppository, consistent small lymphocytes. Blood vessel invasion could be seen, as wells as consistent small lymphocytes, regular form, less cytoplasm, rare nuclear fission. Immunohistochemistry showed AE1/AE3(-), CD20(-), CD3(+), ALK (-), CD30(+), CD43(+), CD2(+), EMA(-), Ki-67(70%+), CD56(+), TIA-1(+), GrB(+), CD4(-), CD8(-). Finally, the patient was diagnosed as NK/T-cell lymphoma, nasal, recurrence. In situ hybridization showed EB virus encoded RNA (EBER) was positive for two biopsies. Conclusions Inert extranodal NK/T-cell lymphoma is very rare. The pathogenesis is closely related to EB virus infection. Its diagnosis must rely on histopathology and immunohistochemistry.
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INTRODUCCIÓN: El linfoma extranodal natural killer/célula T (NK/T) de tipo nasal, es una neoplasia poco frecuente, con una alta letalidad, caracterizada por destrucción ósea alrededor de los senos paranasales, el septum nasal u obstrucción de la vía aérea. Puede presentar compromiso primario de la piel, vía aérea y otros órganos. OBJETIVO: Presentar un caso ilustrativo de una afección poco frecuente y de curso agresivo en población pediátrica, para facilitar la sospecha diagnóstica y el rápido reconocimiento por parte de los especialistas. CASO CLÍNICO: Adolescente de 14 años, que consultó por lesiones solevantadas en brazos y piernas, no dolorosas, sugerentes de paniculitis subcutánea, las cuales evolucionaron a máculas violáceas ulceradas. La biopsia de las lesiones fue compatible con linfoma NK/T de tipo nasal. Fue derivada a oncología pediátrica, donde recibió tratamiento quimioterápico. Pese a los esfuerzos médicos, la paciente falleció a los 8 meses producto de una infección pulmonar grave secundaria a inmunosupresión. CONCLUSIONES: El linfoma extranodal NK/T, tipo nasal es una neoplasia poco frecuente, que se comporta de forma agresiva, con una alta mortalidad sin tratamiento. Por lo que su reconocimiento es de gran relevancia para el diagnóstico precoz y rápida derivación a Hemato-Oncología.
INTRODUCTION: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum or obstruction of the airway. Also, may be primary skin involvement, airway and other organs. OBJECTIVE: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists. CASE REPORT: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae. Skin biopsy showed lesion compatible with NK/T lymphoma, nasal type. She was referred to pediatric oncology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression. CONCLUSIONS: Extranodal NK/T-cell lymphoma, nasal type, is a rare neoplasm that behaves aggressively, with high mortality without treatment, therefore, its recognition has a high importance for early diagnosis and prompt referral to Hematology-Oncology.
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Humans , Female , Adolescent , Skin Neoplasms/diagnosis , Lymphoma, Extranodal NK-T-Cell/diagnosis , Fatal OutcomeABSTRACT
Objective To evaluate the prognostic value of anemia in patients diagnosed with extranodal nasal-type natural killer (NK)/T cell lymphoma (NKTCL).Methods Clinical data of 1 225 NKTCL patients receiving the first course of treatment from 10 medical institutions in China were retrospectively analyzed.According to the diagnostic criteria in China,anemia was defined as the hemoglobin (Hb) level＜ 120 g/L for the male and＜ 110 g/L for the female from the sea-level area.The severity of anemia was classified into the extremely severe anemia (Hb ≤ 30 g/L),severe anemia (31-60 g/L),moderate anemia (61-90 g/L) and mild anemia (＞90 g/L).Results Among 1 225 patients,199(16.2％) were complicated with anemia,who had more adverse prognostic factors compared with their counterparts without anemia.Among NKTCL patients with anemia,the proportion of patients with stage Ⅱ-ⅣV,a median age＞ anemia,Eastern Cooperative Oncology Group (ECOG) score of 2-4 and NK/T-cell lymphoma prognostic index (NKTCLPI) ≥ 2 was relatively high.Patients with anemia obtained worse clinical prognosis than those without anemia.The 5-year overall survival (OS) and progression-free survival (PFS) in NKTCL patients with anemia were calculated as 49.4％ and 35.4％,significantly lower compared with 63.3％ and 56.0％ in their counterparts without anemia (both P＜0.01).Single factor analysis demonstrated that anemia,age,ECOG score,group B symptom,lactate dehydrogenase,primary tumor site,primary tumor invasion and staging were the prognostic factors of OS and PFS.Multivariate analysis revealed that anemia was still the independent prognostic factor.Conclusions Anemia is not common in patients with NKTCL and these patients obtain poor clinical prognosis.Anemia is an independent prognostic factor for patients with NKTCL.
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Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.
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Objective To investigate the feasibility of the scheme of target volume delineation with extended involved?field intensity?modulated radiotherapy (IMRT) for patients with early?stage nasal NK/ T?cell lymphoma (NC?NKTL). Methods Twenty?one patients with stage IE?IIE NC?NKTL were treated with short?course chemotherapy combined with radiotherapy from 2011 to 2013. The majority of patients received the GELOX regimen. All patients received extended involved?field IMRT with a dose of 54?? 6 Gy in 26 fractions for gross tumor volume, 50?? 7 Gy in 26 fractions for high?risk clinical target volume (CTV), and 45?? 5 Gy in 26 fractions for low?risk CTV. The dose distribution, short?term treatment outcomes, and adverse reactions were analyzed. Results The 2?year sample size was 12. The 2?year follow?up rate was 100%. The 2?year local control rate ( LRC) was 100%. The 2?year overall and progression?free survival rates were 90?? 5% and 90?? 5%, respectively. The median coverage rates of planning target volumes with 90% of the prescribed doses of 54?? 6 Gy, 50?? 7 Gy, and 45?? 5 Gy were 99?? 8%, 99?? 6%, and 99?? 7%, respectively. No grade 3 or 4 adverse reactions were observed in patients. Conclusions The scheme of target volume delineation and dose configuration in our study not only achieves excellent target volume coverage, but also reduces adverse reactions in patients, which achieves a 2?year LRC ideal for patients with early?stage NC?NKTL.
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Background and purpose:Extranodal NK/T-cell lymphoma, nasal type (ENKTL) belongs to a rare type of non-Hodgkin's lymphoma (NHL). Its incidence rate in Asian country is higher than that in Western country. This disease is highly invasive, the pathogenesis of it is still unclear. Resent research shows that epstein-barr virus (EBV) is closely related to the occurrence of it. There is still no standard treatment guidelines of ENKTL, and the prognosis is very bad. Therefore, it is imperative to explore the pathogenesis of ENKTL. This study aimed to investigate the expressions of interleukin-2 (IL-2), interleukin-6 (IL-6) and tumor necrosis factor-α (TNF-α) in the serum of ENKTL patients and their clinical signiifcances. Methods: Luminex liquid chip technology was used to detect the expression levels of IL-2, IL-6 and TNF-αin the serum of 67 ENKTL patients and 26 normal persons. Results:The expression levels of IL-2, IL-6 and TNF-αin the serum of 67 ENKTL patients were (564.1±387.6), (293.3±191.6) and (181.3±91.8)pg/mL, while in the normal persons were (1 097.0±365.7), (417.5±289.6) and (291.3±89.4)pg/mL, respectively. Compared with normal persons, the expression levels of IL-2, IL-6 and TNF-αin ENKTL patients were signiifcantly lower (P<0.05). Further study showed that the expression level of TNF-αin 5 complete remission ENKTL patients [(162.7±10.3)pg/mL] was significantly higher than that in initial treatment patients [(125.2±7.3)pg/mL, P<0.05]. Conclusion:The expressions of IL-2, IL-6 and TNF-αare reduced in the serum of ENKTL patients, and the serum expression level of TNF-αis closely related to the effect of chemotherapy.
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Extranodal NK/T-cell lymphoma,nasal type (ENKTL) is a aggressive lymphoma which arises from NK cells or T cells and has a poor prognosis.This article mainly reviewed and focused on hematological stem cell transplantation in treating ENKTL.
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Primary nasal type natural killer (NK)/T cell (NKTC) lymphoma, a specific form of malignant lymphoma, has a higher geographic incidence in Oriental, Mexican, and South American populations than the Western population. In Koreans, it comprises 9-12% of all cases of non-Hodgkin's lymphoma. This type of lymphoma has also been named as angiocentic lymphoma and lethal midline granuloma because the most common site is the upper airway area and its clinical aggressiveness presents with a necrotic and destructive pattern. NKTC lymphoma can also be detected in different organs (testis, spleen, parotid gland, skin, gastroinstinal tract, central nervous system, lungs, bone marrow, etc.) other than the upper airway including the oral cavity. The lymphoma detected in the oral cavity shows various destructive and inflammatory changes, similar to the signs of inflammation and infection from periodontitis and pulpal disease, making a diagnosis difficult with just the clinical signs. For early detection, clinical, radiological, and pathological examinations are required. This report describes the clinical, radiological and histological characteristics with a case report for the early detection of NKTC lymphoma in the oral cavity.
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Bone Marrow , Central Nervous System , Early Diagnosis , Granuloma, Lethal Midline , Incidence , Inflammation , Lung , Lymphoma , Lymphoma, Non-Hodgkin , Mouth , Parotid Gland , Periodontitis , Skin , SpleenABSTRACT
El linfoma T/NK cutáneo símil hidroa vacciniforme (HV) es un linfoma poco común que afecta principalmente a niños asiáticos e indoamericanos. Se inicia con edema, vesículas, costras y cicatrices de tipo vacciniforme. De evolución prolongada, tiende a agravarse con los años, desfigurando y comprometiendo física y psíquicamente al paciente. Debido a la detección tardía del linfoma, en general el pronóstico de vida de estos pacientes es malo. Se presenta una paciente de 20 años con linfoma NK de tipo nasal, que comenzó a los 13 años con un cuadro de HV de mala evolución. Los autores proponen que en pacientes con HV atípica un tratamiento en etapas tempranas de la enfermedad podría mejorar su pronóstico.
The hydroa-like cutaneous T-cell lymphoma is not a very common lymphoma,children from Asia and indoamericans are mainly aff ected. Theybegin with edema, vesicles, crusts and vacciniform scars with a prolongedevolution increasing through to the years. This illness aff ects the patientsphysically and psychologically because of its disfi guring eff ects.Due to the late detection of the lymphoma, generally the prognosis ofthese patients is poor. A patient of 20 years old, who suff ered of NK-celllymphoma nasal type, is presented. She began with hydroa vacciniformeat the age 13 years old, she developed a T/NK lymphoma and died. Theauthors propose that on patients with atypical hydroa vacciniformes, atreatment in earlier stages could off er a better prognosis (Dermatol Argent2009;15(5):350-353).
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Humans , Lymphoma, T-Cell, Cutaneous/pathology , Nose/pathology , Skin Neoplasms/pathology , Nose Neoplasms/pathology , Nose Diseases/pathology , Hydroa Vacciniforme/pathology , Epstein-Barr Virus Infections/pathology , Skin/pathologyABSTRACT
NK/T-cell lymphoma is rare type of lymphoproliferative disorder, but it is clinically aggressive with a poor prognosis. The World Health Organization (WHO) classified mature NK cell neoplasms into extranodal NK/T-cell lymphoma nasal type and aggressive NK cell leukemia. NK/T-cell lymphoma is characterized by a strong association with Epstein-Barr virus (EBV), and when it involved the skin, the lesions clinically appear as deep nodules, infiltrative plaques and ulcerative plaques. We herein report on a case of the nasal type of extranodal NK/T-cell lymphoma that showed the immunophenotype of CD20-, CD56+ and CD45RO+, and it was positive on in situ hybridization for EBV. Our case was characterized by the clinical resemblance with pyoderma gangrenosum and this malady generally displays chronic painful ulcer and necrotic lesions.
Subject(s)
Chronic Pain , Herpesvirus 4, Human , In Situ Hybridization , Killer Cells, Natural , Leukemia , Lymphoma , Lymphoproliferative Disorders , Prognosis , Pyoderma , Pyoderma Gangrenosum , Skin , Ulcer , World Health OrganizationABSTRACT
We report a case of a 38-year-old male with extranodal NK/T-cell lymphoma, nasal type, showing unusual clinical and pathological features. The patient was admitted for soft tissue swelling and tenderness in both legs. The patient had been treated intermittently 8 months prior for repeated muco-cutaneous ulcers. A muscle biopsy showed medium-sized atypical lymphoid cells with bizarre nuclei and plump cytoplasm, infiltrating to the skeletal muscle fibers with angiocentricity. The immunoresults were Ki-1+, CD56+, cytoplasmic CD3+, with EBV-in situ hybridization +. The patient rapidly deteriorated and died of sepsis and respiratory failure shortly after initiation of low-dose chemotherapy. A careful review of previous biopsies revealed scarce atypical lymphoid cells around vessels with similar immunoprofiles without the presence of Ki-1 positive cells. This case emphasizes that an extranodal NK/T-cell lymphoma may have a dermatomyositis-like diffuse presentation. Ki-1 co-expression can be an unexpected event in a process of the disease course; however, this should be validated with future studies.
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Adult , Humans , Male , Biopsy , Cytoplasm , Dermatomyositis , Drug Therapy , Leg , Lymphocytes , Lymphoma , Muscle Fibers, Skeletal , Respiratory Insufficiency , Sepsis , UlcerABSTRACT
Extranodal NK/T cell lymphoma(nasal type) is a common,high malignant degree and poor prognosis entity of the nasal lymphoma.It is the most important to understand its genesis,development,clinical manifestation,pathologic diagnosis,therapy and prognosis.