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: Ramyar Ghandriz MD[2] Sowminya Arikapudi, M.B,B.S. [3]

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.

Natural History

• Extranodal NK-T-cell lymphoma almost always shows an extranodal presentation.
• 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)

• Hemophagocytic syndrome results in

• Fever
• Hepatosplenomegaly
• Pancytopenia

Prognosis

• The prognosis for people with extranodal NK-T-cell lymphoma is often poor and the risk for relapse is high.
• 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.
• 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

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