Extranodal NK-T-cell lymphoma classification

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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

In contrast with B-cell lymphoma, classification of such a rare neoplasm has been controversial. Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extranodal NK-T-cell lymphoma, nasal type. Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into extranodal NK-T-cell lymphoma, nasal type, and extranodal NK-T-cell lymphoma, extra nasal type which contains two major subclass: Enteropathy type and subcutaneous panniculitis-like.

Classification

• In contrast with B-cell lymphoma, classification of such a rare neoplasm has been controversial, since the cytologic features have not been very useful. Further, by many entities, T-cell and natural killer cell (NK) neoplasms do not share any similar immuno-phenotype. Because of such matter, clinical features became handier for classification and somehow even more important than the precise cell of origin.

• Since the majority of cytotoxic T-cell and NK cell lymphomas are located out of lymph nodes, the gene expressing cytotoxic molecules may predispose to apoptosis by tumor cells and by standard cells.

• Three major categories of extranodal T/NK cell tumors include:

• • Extranodal NK/T-cell lymphoma, nasal type lymphoma
  • Extranodal NK/T-cell lymphoma, enteropathy type lymphoma
  • Extranodal NK/T-cell lymphoma, subcutaneous panniculitis-like

Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes:

• NK cell-derived neoplasms, namely, aggressive NK cell leukemia
• Extranodal NK/T-cell lymphoma, nasal type

Based on the organ involvement, extranodal NK-T-cell lymphoma may be classified into:

• Extranodal NK-T-cell lymphoma, nasal type
• Extranodal NK-T-cell lymphoma, extra nasal type

Extranodal NK-T-cell lymphoma classification

Name	Description
Extranodal NK-T-cell lymphoma, nasal type	Most commonly affects the nose or nasal passages and paranasal sinuses
Extranodal NK-T-cell lymphoma, extra nasal type	Can affect the: Gastrointestinal tract (most common extranasal sites) Skin Testicles Soft tissues Kidneys Brain or spinal cord (central nervous system) Respiratory tract

Natural Killer cell neoplasms are classified by the world health organization (WHO) into:

• NK/T-cell lymphoma, nasal type (NKTCL)
• Aggressive NK-cell leukemia (ANKCL)
• Chronic lymphoproliferative disorders of NK-cell
  

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