Extranodal NK-T-cell lymphoma pathophysiology

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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

Extra nodal NK-T-cell lymphoma, nasal type, can develop in either T cells or natural killer cells, but most often in the NK cells. Natural killer cells are a type of lymphocyte that is closely related to T cells and attack foreign cells. On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma. On microscopic histopathological analysis, medium-sized tumor cells and polymorphic infiltrate of nonneoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.

Pathophysiology

Physiology

• NK cells are CD3 and myloperoxidase negative on their surface.
• NK cells have germline configuration of T-cell receptor and immunoglobulin genes.

Immunotype

• The immunophenotype of NK lymphoma cells is classically positive for CD2, CD56, and cytoplasmic CD3 epsilon.
• They are negative for surface CD3. Unlike normal NK cells, the tumor cells are usually negative for CD7 and CD16.
• They express cytotoxic granule associated proteins granzyme B, T-cell restricted intracellular antigen (TIA-1), and perforin.

Gross Pathology

• On gross pathology, angiocentric and angiodestructive pattern of growth with associated geographical necrosis and ulceration are characteristic findings of extranodal NK-T-cell lymphoma.
• Coagulative necrosis and apoptotic bodies are frequently encountered.

Microscopic Pathology

• On microscopic histopathological analysis, medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells are characteristic findings of extranodal NK-T-cell lymphoma.
• The tumor cells are small to medium in size with occasional large and anaplastic forms.
• The lymphoma cells may be admixed with a polymorphic infiltrate of nonneoplastic inflammatory cells including small lymphocytes, plasma cells, histiocytes, and eosinophils.

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