Extranodal NK/T-cell lymphoma, nasal type (Q39132): Difference between revisions
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Linfoma não Hodgkin de células T maduras, agressivo, predominantemente extranodal. É caracterizada por um infiltrado celular frequentemente angiocêntrico e angiodestrutivo composto por células NK/T positivas para o vírus Epstein-Barr (EBV). A cavidade nasal é a localização de envolvimento mais comum. Os pacientes frequentemente apresentam lesões destrutivas na face média (granuloma letal da linha média). A doença pode se disseminar rapidamente para várias localizações anatômicas, incluindo trato gastrointestinal, pele, testículos e linfonodos cervicais. É também conhecido como linfoma de células T angiocêntrico. O termo reticulose polimórfica tem sido amplamente utilizado para descrever as alterações morfológicas observadas nesse tipo de linfoma. No entanto, este termo também pode ser aplicado à granulomatose linfomatoide, que é um transtorno linfoproliferativo de células B positivo para EBV angiocêntrico e angiodestrutivo. | |||
| description / en | description / en | ||
An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised by an often angiocentric and angiodestructive cellular infiltrate composed of Epstein-Barr Virus (EBV) positive NK/T cells. The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic changes seen in this type of lymphoma. However, the latter term may also apply to lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive B-cell lymphoproliferative disorder. | |||
Revision as of 04:27, 13 August 2026
An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised by an often angiocentric and angiodestructive cellular infiltrate composed of Epstein-Barr Virus (EBV) positive NK/T cells. The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic changes seen in this type of lymphoma. However, the latter term may also apply to lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive B-cell lymphoproliferative disorder.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 2A90.6 |
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| English | Extranodal NK/T-cell lymphoma, nasal type |
An aggressive, predominantly extranodal, mature T-cell non-Hodgkin lymphoma. It is characterised by an often angiocentric and angiodestructive cellular infiltrate composed of Epstein-Barr Virus (EBV) positive NK/T cells. The nasal cavity is the most common site of involvement. Patients often present with midfacial destructive lesions (lethal midline granuloma). The disease may disseminate rapidly to various anatomic sites including the gastrointestinal tract, skin, testis, and cervical lymph nodes. It is also known as angiocentric T-cell lymphoma. The term polymorphic reticulosis has been widely used to describe the morphologic changes seen in this type of lymphoma. However, the latter term may also apply to lymphomatoid granulomatosis, which is an angiocentric and angiodestructive EBV positive B-cell lymphoproliferative disorder. |
