Has angiocentric and angiodestructive growth pattern.
Strong association with Epstein-Barr virus.
Has angiocentric and angiodestructive growth pattern.
Characterized by expression of NK cell marker CD56 and lack of expression of surface CD3, but presence of cytoplasmic CD3.
Characterized by expression of NK cell marker CD56 and lack of expression of surface CD3, but presence of cytoplasmic CD3.
Radiotherapy is the treament of choice for loclized disease.
No significant benefit with addition of chemotherpay to radiation.
5 year disease free survival and overall survival ranges from 30-40%.
More common in adults and children at a median onset of 45 years.
NK/T-cell lymphoma occur most frequently in Central and South America and the Far East.
Increasing incidence in the US.
There is an increased incidence and Asian and Pacific Islanders than whites.
Increased incidence may be reflection of increased immigration patterns in diversity of the US population.
Makes up 3.6% of adult lymphomas.
Constitutes between 0.2% and 1.6% of the newly diagnosed cases of non-Hodgkin’s lymphoma in children and adolescents.
ExtranodalNK/T-cell lymphoma is a rare lymphoma associated with Epstein-Barr virus.
An aggressive malignancy with a poor prognosis.
The upper aerodigestive tract most commonly involves especially the nasal and paranasal areas.
Patients typically present with nasal obstruction, epistaxis and destruction of the nasal structures.
The skin can be a primary site.
Manifestations include generalized painful ulcerated nodules of the trunk and limbs or abscess-like swelling of the extremities.
Can be complicated by hemophagocytic lymphohistiocytosis.
Diagnosis requires Epstein-Barr virus be found in malignant cells.
Epstein-Barr virus DNA load appears to be a biomarker of tumor load.
Process spurred by excessive immune activation.
Clinical features are variable and nonspecific and include: fever, pancytopenia, hepatosplenic megaly, coagulopathy, elevated f2242itin levels, impaired liver functions, skin rash, hyper triglyceridemia, neurologic symptoms, hypofibrinogenemis, and lymphadenopathy.
Treatment for patients with advanced disease is L-aspariginase.
Patients with hematophagocytic lymphohistiocytosis syndrome have the highest mortality rate.