ABSTRACT
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.
Subject(s)
Humans , Treatment Outcome , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Retrospective Studies , Etoposide , Neoplasm Recurrence, Local/drug therapy , Asparaginase , Deoxycytidine , Lymphoma, T-Cell, Peripheral/drug therapy , Lymphoma, Extranodal NK-T-Cell/therapy , Oxaliplatin/therapeutic useABSTRACT
Peripheral T-cell lymphomas (PTCLs) comprise a heterogeneous group of neoplasms that are derived from post-thymic lymphoid cells at different stages of differentiation and with different morphological patterns, phenotypes, and clinical presentations. PTCLs are highly diverse, reflecting the diverse cells from which they can originate and are currently sub-classified using World Health Organization (WHO) 2008 criteria. Peripheral T-Cell Lymphomas account for 5 percent-10 percent of all lymphoproliferative disorders in the Western hemisphere, with an overall incidence of 0.5-2 per 100,000 individuals per year, and have a striking epidemiological distribution, with higher incidence in Asia. The clinical features of PTCL are extremely heterogeneous. PTCLs express even more clinical diversity than B-cell non-Hodgkin's lymphomas, and there is a close, though not absolute, relationship between some unusual clinical features and certain histological subtypes.
Linfomas T periféricos (PTCLs) compreendem um grupo heterogêneo de neoplasias que derivam das células linfoides pós-tímicas nos diversos estágios de maturação, com diversos padrões histológicos, fenotípicos, e clínicos. PTCLs são muito diversos entre si e refletem diversas células das quais foram originadas e são atualmente subclassificadas, usando-se a classificação da Organização Mundial da Saúde (OMS) 2008, apresentada neste texto na tabela 1. PTCLs compreendem 5 por cento-10 por cento de todas as doenças linfoproliferativas no mundo ocidental, com uma incidência global de 0.5 a 2 a cada 100.000 pessoas por ano e têm uma distribuição epidemiológica diversa com maior incidência na Ásia. Os achados clínicos dos PTCLs são muito heterogêneos. PTCLs expressam muito maior variação de apresentações clínicas do que os linfomas B, e há uma íntima, mas não absoluta, relação entre algum achado clínico não usual e certos subtipos histológicos. O autor faz aqui uma revisão do assunto altamente contemporâneo
Subject(s)
Lymphoma, T-Cell, Peripheral , Reference Standards , T-Lymphocytes , Lymphoma, T-Cell, Peripheral/drug therapy , Lymphoma, T-Cell, Peripheral/epidemiology , Drug Therapy , Lymphoproliferative Disorders , NeoplasmsABSTRACT
Thirty-two patients with fever of unknown origin, weight loss, anemia, elevated serum levels of alkaline phosphatase and/or lactate dehydrogenase were evaluated. Histopathologic findings of the liver showed T-cell infiltration in the hepatic sinusoids and portal tracts. The cellular morphology varied from mature lymphocyte to malignant lymphoid cells. We divided the cases into four groups on the basis of cellular atypia. Group A and group B showed mature lymphoid cell infiltration, however, only group B had multiple large areas of hepatocellular necrosis. Group C showed atypical lymphoid cell infiltration. In group D, definite malignant lymphoid cell infiltrates were demonstrated. Groups B, C, and D patients had a very poor prognosis. All of them died despite chemotherapy. Group A patients had a better prognosis. Those who had chemotherapy achieved a complete remission. Progression to a higher group occurred in two of six patients with group B lesions and one of seven patients with group C lesions. The EBV-RNA genomes were found increasingly in the higher groups. This study supports the concept that these groups of disease represent a spectrum of peripheral T-cell proliferations.