Clinical Anslysis of Primary Adrenal NK/T-Cell Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical characteristics, diagnosis, and treatment of one patient with primary adrenal natural killer/T-cell lymphoma (PANKTCL), and to strengthen the understanding of this rare type of lymphoma.@*METHODS@#The clinical manifestations, diagnosis and treatment process, and prognosis of the patient admitted in our hospital were retrospectively analyzed.@*RESULTS@#Combined with pathology, imaging, bone marrow examination， etc, the patient was diagnosed with PANKTCL (CA stage, stage II; PINK-E score 3, high-risk group). Six cycles of "P-GemOx+VP-16" regimen（gemcitabine 1 g/m2 d1 + oxaliplatin 100 mg/m2 d 1 + etoposide 60 mg/m2 d 2-4 + polyethylene glycol conjugated asparaginase 3 750 IU d 5） was performed, and complete response was assessed in 4 cycles. Maintenance therapy with sintilimab was administered after the completion of chemotherapy. Eight months after the complete response, the patient experienced disease recurrence and underwent a total of four courses of chemotherapy, during which hemophagocytic syndrome occurred. The patient died of disease progression 1 month later.@*CONCLUSION@#PANKTCL is rare, relapses easily, and has a worse prognosis. The choice of the "P-GemOx+VP-16" regimen combined with sintilimab help to improve the survival prognosis of patient with non-upper aerodigestive tract natural killer /T-cell lymphoma.

Analysis on Clinical Characteristics and Prognosis of 47 Patients with Extranodal NK/T Cell Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical features and prognostic factors of patients with extranodal NK/T cell lymphoma (ENKTL).@*METHODS@#The clinical data of patients with ENKTL from November 2009 to November 2019 was collected and retrospectively analyzed to clarify the clinical features of ENKTL, and evaluate the factors that affected survival and prognosis.@*RESULTS@#Forty-seven patients with ENKTL were collected, median age was 40 (12-82) years old, and more common in males than females, at the ratio of 1.47∶ 1. The median follow-up was 28 (1-112) months, and 5-year overall survival (OS) rate was 49.3%. The 5-year OS rates of the subjects with ECOG performance stage 0-1 and ≥2 were 51.6% and 0 (P=0.001), respectively. The 5-year OS rates of International Prognostic Index (IPI) score 0-1 and ≥2 were 60.0% and 40.6% (P=0.027), respectively. The 5-year OS rates of Ann Arbor staging Ⅰ/Ⅱ and stage Ⅲ/Ⅳ were 61.3% and 31.7% (P=0.005), respectively. The 5-year OS rates of the patients with presentation of B symptoms and without presentation of B symptoms were 79.0% and 30.1% (P=0.013), respectively. The 5-year OS rates of plasma EBV-DNA level < 5×10@*CONCLUSION@#ECOG score, B symptoms, the copy number of EBV-DNA, and treatment regimens are independent prognostic factors for OS of patients with ENKTL.

SMILE en linfoma de células NK/T extranodal en recaída / SMILE in relapsed extranodal NK / T cell lymphoma

Introducción: El linfoma extranodal de células NK/T, tipo nasal es una variedad agresiva que se presenta con mayor frecuencia en Asia Oriental y América Latina. El régimen de tratamiento SMILE, basado en dexametasona, metotrexato, ifosfamida, L-asparaginasa y etopósido, mostró tasas de respuestas objetivas adecuadas en estudios de fase 1/2. Objetivo: presentar el primer reporte de la aplicación del protocolo SMILE en Cuba. Presentación del caso: paciente de 47 años de edad, blanca que presentó recaída temprana de linfoma extranodal de células NK/T tipo nasal en partes blandas de la pared anterior izquierda del tórax. Recibió quimioterapia SMILE por 4 ciclos y en la evaluación al final del tratamiento se comprobó que hubo una respuesta completa de la enfermedad(AU)

Introduction: Extranodal lymphoma of NK / T cells, nasal type is an aggressive variety that occurs most frequently in East Asia and Latin America. The SMILE treatment regimen, based on dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide, showed adequate objective response rates in phase 1/2 studies. Objective: to present the first report of the application of the SMILE protocol in Cuba. Case presentation : 47-year-old white patient who presented early relapse of extranodal lymphoma of nasal NK / T cells in soft parts of the left anterior wall of the thorax. He received SMILE chemotherapy for 4 cycles and the evaluation at the end of the treatment showed that there was a complete response of the disease(AU)

Linfoma nasal de células T/NK (granuloma letal de la línea media), una neoplasia agresiva: reporte de un caso / Nasal T/NK-cell lymphoma (lethal midline granuloma), an aggressive neoplasia: report of a case

RESUMEN El linfoma nasal de células T/NK es una neoplasia agresiva, infrecuente, con predilección por el sexo masculino. Representa el 1,5% del total de linfomas no Hodgkin, el grupo etáreo más afectado es entre 40 a 80 años. Su etiología es desconocida, pero se ha asociado con el virus de Epstein Barr. Se presenta el caso de una paciente mujer de 40 años, con dolor e induración de fosa nasal derecha asociado a secreción fétida, seropurulenta de 3 meses de evolución. Estudio histopatológico, reporta linfoma nasal de células T/NK. La paciente recibe quimioterapia, con mejoría clínica sustancial. El linfoma T/NK es una neoplasia con sintomatologia inespecífica, predominio que afecta nasofaringe, amígdalas y base de la lengua. Entre las manifestaciones encontramos dolor de garganta, obstrucción nasal, rinorrea, epistaxis y cefalea. El diagnóstico se sospecha con imágenes, pero es necesaria la confirmación histológica con marcadores de inmunohistoquímica CD45Ro, CD43, CD3, CD2, CD45Ro entre otros. Muchos casos suelen detectarse en fase tardía, cuando ya son evidentes los signos radiológicos de destrucción ósea. El diagnóstico diferencial incluye lesiones infecciosas o inflamatorias, la bola fúngica fue el diagnóstico inicial realizado en esta paciente. El tratamiento suele realizarse con radioterapia y quimioterapia.

ABSTRACT Nasal T-cell / NK lymphoma is an uncommon aggressive neoplasm with male predilection. It represents 1.5% of the total number of non-Hodgkin's lymphomas, the most affected age group is 40-80 years. Its etiology is unknown but has been associated with the Epstein Barr virus. We present the case of a female patient of 40 years, with pain and induration of the right nostril associated with fetid secretion, seropurulent of 3 months of evolution. Histopathological study, reports T-cell / NK nasal lymphoma. The patient receives chemotherapy, with substantial clinical improvement. T / NK lymphoma is a neoplasm with predominant non-specific symptomatology, affecting the nasopharynx, tonsils and base of the tongue. Among the manifestations we find sore throat, nasal obstruction, rhinorrhea, epistaxis and headache. Diagnosis is suspected with imaging, but histological confirmation with immunohistochemical markers CD45Ro, CD43, CD3, CD2, CD45Ro, among others is required. Many cases are usually detected in the late phase, when radiological signs of bone destruction are already visible. The differential diagnosis includes infectious or inflammatory lesions, the fungal ball was the initial diagnosis made in this patient. Treatment is usually done with radiotherapy and chemotherapy.

Clinical and otorhinolaryngological aspects of extranodal NK/T cell lymphoma, nasal type / Aspectos clínicos e otorrinolaringológicos do linfoma extranodal de células NK/T tipo nasal,

INTRODUCTION: Extranodal NK/T-Cell lymphoma, nasal type (NKTLN) is a disease that mainly affects the nasal cavity and the paranasal sinuses. Early nasal symptoms are nonspecific, simulating sinus infection. With disease progression, necrosis of the nasal mucosa increases, hindering histological diagnosis. Thus, multiple biopsies may be necessary until definitive diagnosis. Most studies on NKTLN address the hematological and immunological aspects of the disease. OBJECTIVES: To present data from a Brazilian quaternary hospital, with emphasis on the clinical aspects of the disease, and to correlate the findings with the most recent literature data. METHODS: Case study of seven patient files. RESULTS: Patients were evaluated on their medical history, number of biopsies necessary, association with Epstein-Barr virus, treatment, and outcome. All patients had nonspecific nasal complaints and underwent at least three cycles of antibiotic therapy. The earlier a biopsy was performed, the fewer biopsies were required to diagnose the disease and start treatment. However, this fact did not translate into better prognosis. CONCLUSION: The otolaryngologist plays a fundamental role in the prognosis of NKTLN and can shorten time between symptom onset and treatment of the patient. .

INTRODUÇÃO: O linfoma extranodal de células NK/T tipo nasal (LNKTN) é uma doença que acomete preferencialmente a cavidade nasal e os seios paranasais. Os sintomas nasais iniciais são inespecíficos, mimetizando um quadro de infecção nasossinusal. Com a progressão da doença, aumenta a necrose da mucosa nasossinusal, dificultando o diagnóstico histológico e podendo ser necessárias múltiplas biópsias até o diagnóstico definitivo. A maioria dos estudos sobre o LNKTN aborda aspectos imunológicos e hematológicos da doença. OBJETIVO: Apresentar a casuística de um hospital quaternário brasileiro, destacando os aspectos clínicos dos pacientes e correlacionando aos achados mais recentes da literatura. MÉTODO: Estudo de casos de sete pacientes. RESULTADOS: Pacientes foram avaliados quanto a aspectos de sua história clínica, número de biópsias necessárias, associação ao EBV, tratamento e evolução. Todos iniciaram o quadro com queixas inespecíficas nasais e foram submetidos a pelo menos três ciclos de antibióticos. Quanto mais precocemente a biópsia foi realizada, menos biópsias foram necessárias para se obter o diagnóstico e iniciar o tratamento. Entretanto, esta situação não refletiu num melhor prognóstico dos pacientes. CONCLUSÃO: O otorrinolaringologista tem papel fundamental no prognóstico do LNKTN, podendo encurtar o tempo entre o início dos sintomas e o tratamento do paciente. .