Nasal/nasal T/NK cell lymphoma
Introduction
Introduction of nasal/nasal T/NK cell lymphoma Nasal/nasal T/NK cell lymphoma (nasal/nasaltype T/NKcelllymphoma) is often found in extranodal tissues and is characterized by a high incidence of nasal involvement. In some cases, skin damage occurs before extradermal damage, suggesting that this type of lymphoma can also be a primary cutaneous lymphoma. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious Complications: swelling
Cause
Nasal/nasal T/NK cell lymphoma etiology
(1) Causes of the disease
The cause is not yet clear.
(two) pathogenesis
The pathogenesis is still unclear.
Prevention
Nasal/nasal T/NK cell lymphoma prevention
Nasal NK/ T-cell lymphoma is rare in clinical practice, its clinical manifestations are atypical, pathological morphology is diverse, and it is easy to be misdiagnosed, and there is still a lack of in-depth understanding of the nature of its disease. In terms of molecular genetics and pathogenesis, the comparability of results is relatively poor due to the different research methods of various scholars. In order to understand the disease at a molecular level, it is necessary to use a combination of gene chip, fluorescence in situ hybridization and other new technologies for comprehensive research. At present, domestic and foreign scholars have made various attempts to treat the disease and achieved certain curative effects. However, due to the rapid clinical progress and high malignancy of nasal type NK/T-cell lymphoma, various current treatment options have made Its clinical complete response rate is still relatively low, resulting in a poor prognosis. Therefore, it is necessary to further explore its new treatment plan, new drug selection, comprehensive consideration of its prognostic factors, thereby further improving the patient's treatment effect and survival prognosis.
Complication
Nasal/nasal T/NK cell lymphoma complications Complications swelling
Some cases can be complicated by bloodthirsty syndrome. Nasal NK/T-cell lymphoma that occurs outside the nasal cavity has various manifestations depending on the site of involvement. The skin is easily affected and manifests as nodules with ulcers. Occurring in the intestines, perforation often occurs. Most patients have reached a high clinical stage at the time of the visit, showing multiple external involvement. There are systemic symptoms such as fever, discomfort, and weight loss. Lymph node involvement is part of the spread of the disease. Bone marrow and blood involvement can also occur, which overlaps with invasive NK cell leukemia.
Symptom
Nasal/nasal T/NK cell lymphoma symptoms Common symptoms Cervical lymphadenopathy Generalized red plaque Skin diffuse redness
In the tumor that occurs in the nose, the patient exhibits nasal obstruction and nasal discharge, which is caused by the mass and the damage of the middle and facial structures caused by the mass. Tumors can invade surrounding tissues such as the nasopharynx, paranasal sinuses, eyelids, mouth, ankles, and oropharynx. Tumors are often confined to the upper respiratory tract, rarely involving the bone marrow, but quickly spread to different parts: the skin, the gastrointestinal tract, the testes, and the cervical lymph nodes.
Occurs in Asians, especially women, with an average age of onset of about 40 years. The lesion is a generalized red plaque or a localized tumor.
Examine
Nasal/nasal T/NK cell lymphoma examination
Histopathology: The dermis was found to be diffusely infiltrated with moderate-sized atypical lymphocytes, showing pro-epidermal properties, often in a vascular center pattern.
Immunohistochemistry: Lymphoma cells are CD2 and CD3 and natural killer markers CD56 phenotype, common Epstein-Barr virus, visible T cell receptor gene cloning rearrangement and EBV genome clonal integration.
Diagnosis
Diagnosis of nasal/nasal T/NK cell lymphoma
diagnosis
According to the clinical manifestations, the characteristics of skin lesions, histopathological features, and immunohistochemical examination can be diagnosed.
Differential diagnosis
The morphology of extranodal NK/T-cell lymphoma in various sites is basically similar. Occurred in the mucosal area, ulceration often occurs. Tumor cells are diffusely infiltrated and are common in vessel infiltration and vascular destruction. Coagulative necrosis and apoptotic bodies are common because of vascular obstruction caused by tumor cells, but recent studies have suggested that certain chemical factors and cytokines are also involved.
Extranodal NK/T-cell lymphoma involves a wide range of cell morphology. Cells may be small, medium, large or variable cells. Most cases are medium cells or mixed cells. The nucleus can be irregular or lengthened. The chromatin is granular, but the large nucleus is vesicular. Usually the nucleoli are not obvious or have small nucleoli. The cytoplasm is medium, lightly stained to translucent, and mitotic figures are easy to see, even in small cell-based cases. In the Kimssa staining of the print, the azure blue particles are easy to see. Electron microscopy shows electron density particles.
NK/T-cell lymphoma, especially lymphomas dominated by small cells and mixed cells, can be seen in a large number of reactive inflammatory cells, such as small lymphocytes, plasma cells, tissue cells, eosinophils (hence, formerly known as " Polymorphic reticulocyte hyperplasia"). These cases are very similar to inflammatory diseases. This lymphoma is sometimes associated with pseudoepithelial neoplasia, similar to squamous cell carcinoma.
Immunophenotype
The most typical immunophenotypes are: CD2+, CD56+, surface CD3-, cytosolic CD3+. Cytotoxic particle-associated proteins (eg, granzyme B, TIA-1, and perforin) are also expressed in most cases. Other T and NK cell-associated antigens were negative, including CD4, CD5, CD8, TCR, TCR, CD16 and CD57. CD43, CD45RO, HLA-DR, IL-2 receptor, Fas (CD95) and Fasligand are often positive. Occasionally there are cases of CD7 or CD30 positive.
Many people have classified CD3, CD56-, cytotoxic molecule+, and EBV+ cases as nasal NK/T-cell lymphomas because these cases have clinical processes similar to those of CD56+ cases. However, when cytotoxic molecules and EBV are negative, they should not be diagnosed as nasal NK/T-cell lymphoma, but should be diagnosed as "peripheral T-cell lymphoma without other characteristics." It should be emphasized that although CD56 (N-CAM) is a very useful marker for NK/T-cell lymphoma, it is not specific and can be found in peripheral T-cell lymphoma, especially those expressing -T cell receptors. Lymphoma.
