Extranodal NK-T-cell lymphoma natural history, complications and prognosis

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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.

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 :

References

  1. 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. 2.0 2.1 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. 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
  4. Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 04, 2016



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