Extranodal NK-T-cell lymphoma natural history, complications and prognosis: Difference between revisions
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If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop [[proptosis]] and [[hard palate]] perforation. Common complications of extranodal NK-T-cell lymphoma include [[hepatosplenomegaly]], and [[pancytopenia]]. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.<ref name=cancer.gov> Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016</ref><ref name=Hindawi>Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016 </ref> | If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop [[proptosis]] and [[hard palate]] perforation. Common complications of extranodal NK-T-cell lymphoma include [[hepatosplenomegaly]], and [[pancytopenia]]. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.<ref name=cancer.gov> Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016</ref><ref name=Hindawi>Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016 </ref> | ||
==Natural History== | ==Natural History== | ||
* Extranodal NK-T cell lymphoma almost always shows an extranodal presentation.<ref name=cancer.gov> Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016</ref><ref name=Hindawi>Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016 </ref> | * Extranodal NK-T-cell lymphoma almost always shows an extranodal presentation.<ref name=cancer.gov> Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016</ref><ref name=Hindawi>Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016 </ref> | ||
* Some cases may be accompanied by secondary [[lymph node]] involvement although rare instances of primary lymph node disease in the absence of extranodal involvement has been reported. | * Some cases may be accompanied by secondary [[lymph node]] involvement although rare instances of primary lymph node disease in the absence of extranodal involvement has been reported. | ||
* Extension to adjacent tissues such as the [[nasopharynx]], [[paranasal sinuses]], [[orbit]], [[oral cavity]], [[palate]], and [[oropharynx]] are possible. | * Extension to adjacent tissues such as the [[nasopharynx]], [[paranasal sinuses]], [[orbit]], [[oral cavity]], [[palate]], and [[oropharynx]] are possible. |
Revision as of 16:01, 19 February 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
If left untreated, patients with extranodal NK-T-cell lymphoma, nasal type may progress to develop proptosis and hard palate perforation. Common complications of extranodal NK-T-cell lymphoma include hepatosplenomegaly, and pancytopenia. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. Prognosis is generally regarded as poor.[1][2]
Natural History
- Extranodal NK-T-cell lymphoma almost always shows an extranodal presentation.[1][2]
- Some cases may be accompanied by secondary lymph node involvement although rare instances of primary lymph node disease in the absence of extranodal involvement has been reported.
- Extension to adjacent tissues such as the nasopharynx, paranasal sinuses, orbit, oral cavity, palate, and oropharynx are possible.
- Retro-orbital involvement causes proptosis and impairment of extraocular movement.
- Occasionally local extension from the nasal cavity causes destruction of the hard palate with the characteristic midline perforation, previously referred to as “lethal midline granuloma”.
- At presentation, the disease is often localized to the upper aerodigestive tract.
- Disseminated disease may have involvement of the lymph nodes, bone marrow and peripheral blood.
-
NK/T-cell lymphoma of the nasal type presenting with perforation of hard palate.[2]
Complications
- Extranodal NK-T-cell lymphoma, nasal type, often develop hemophagocytic syndrome. (uncontrolled activation of certain parts of the immune system)[3]
- Hemophagocytic syndrome results in
Prognosis
- The prognosis for people with extranodal NK-T cell lymphoma is often poor and the risk for relapse is high.[3]
- People with extranodal NK-T-cell lymphoma confined to the nose or nasal passages have a better prognosis than those people with more widespread disease.
- The International Prognostic Index (IPI) predicts outcome in nasal NK-T-cell lymphoma.[4]
- Patients with International Prognostic Index of 1 or less were shown to have a better overall survival.
- In a recent retrospective analysis of 172 patients with extranodal NK-T-cell lymphoma, nasal type 4 prognostic factors were identified :
- Nonnasal type
- Stage
- Performance status
- Number of extranodal sites
- In a Korean prognostic model, four prognostic groups have been identified depending on :
- B-symptoms
- Stage
- LDH level
- Regional lymphadenopathy
References
- ↑ 1.0 1.1 Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 04, 2016
- ↑ 2.0 2.1 2.2 Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016
- ↑ 3.0 3.1 Extranodal NK/T-cell lymphoma, nasal type. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/extranodal-nk-t-cell-lymphoma-nasal-type/?region=on. Accessed on February 04, 2016
- ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 04, 2016