Non Hodgkin's Lymphoma
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 T / NK  cell Lymphoma  

 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."

  1. Lymphoma of the nasal cavity and paranasal sinuses: improved outcome and altered prognostic factors with combined modality therapy

  2. Primary non-Hodgkin's lymphoma of the nose and nasopharynx: clinical features, tumor immunophenotype, and treatment outcome in 113 patients

  3. Non-Hodgkin's lymphoma of the paranasal sinuses: clinical and pathological features, and response to combined-modality therapy

  4. Diagnosis and management of primary nasal lymphoma of T-cell or NK-cell origin.

  5. 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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