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]

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.
• 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)

This condition results in

• Fever
• Hepatosplenomegaly
• Pancytopenia

References

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