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NK / T-cell
This is a rare subtype of lymphoma which is very aggressive. Due to its
rarity there is not a lot of information to be found about it. At the bottom of
the page are some abstracts from Pubmed regarding treatment strategies that you
can read. The following is a quote from the
NCI's web site
"Extranodal NK-/T-cell lymphoma (nasal type) is an aggressive
lymphoma marked by extensive necrosis and angioinvasion, most often presenting
in extranodal sites, in particular the nasal or paranasal sinus region. Other
extranodal sites include the palate, trachea, skin, and gastrointestinal tract.
Hemophagocytic syndrome may occur; historically these tumors were considered
part of “lethal midline granuloma.” In most cases, EBV genomes are detectable in
the tumor cells and immunophenotyping shows CD56 positivity.
Cases with blood and marrow involvement are considered NK-cell leukemia. In
addition to doxorubicin-based combination chemotherapy, the increased risk of
CNS involvement and of local recurrence has led to recommendations for radiation
therapy locally, often prior to the start of chemotherapy, and for intrathecal
prophylaxis and/or prophylactic cranial irradiation.
The highly aggressive course, with poor response and short survival with
standard therapies, has led some investigators to recommend bone marrow or
peripheral stem cell transplantation consolidation. NK-/T-cell lymphoma
presenting only in the skin has a more favorable prognosis, especially in
patients with coexpression of CD30 with CD56."
Lymphoma of the nasal cavity and paranasal sinuses: improved outcome and altered
prognostic factors with combined modality therapy
Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features,
tumor immunophenotype, and treatment outcome in 113 patients
Non-Hodgkin's lymphoma of the paranasal sinuses: clinical and pathological
features, and response to combined-modality therapy
Diagnosis and management of primary nasal lymphoma of T-cell or NK-cell origin.
Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the
skin: an increasingly recognized entity with an aggressive course.
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