Extranodal NK-T-cell lymphoma overview

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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

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 are closely related to T cells and attack foreign cells. Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extra nodal 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. 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.There are no established causes for extranodal NK-T-cell lymphoma. Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, and peripheral T cell lymphoma.Patients of all age groups may develop extranodal NK-T-cell lymphoma. Males are more commonly affected with extranodal NK-T-cell lymphoma than females. The majority of extranodal NK-T-cell lymphoma cases are reported in Asian, Central-American, and South-American countries.There are no established risk factors for extranodal NK-T-cell lymphoma. According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.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.The most common symptoms of extranodal NK-T-cell lymphoma include fever, weight loss, skin rash, night sweats, protrusion of eye, swelling of the face, discharge from the nose, nose bleeds, blockage of the nasal passages, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.Common physical examination findings of extranodal NK-T-cell lymphoma include fever, rash, ulcer, proptosis, midfacial destructive lesions, epistaxis, nasal obstruction due to mass, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.Laboratory tests for extranodal NK-T-cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.

Classification

Extranodal NK-T-cell lymphoma may be classified according to WHO into 2 subtypes: NK cell-derived neoplasms, namely, aggressive NK cell leukemia and extra nodal 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.

Pathophysiology

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 are 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.

Causes

There are no established causes for extranodal NK-T-cell lymphoma.

Differential Diagnosis

Extranodal NK-T-cell lymphoma must be differentiated from other diseases such as anaplastic large cell lymphoma, non specific inflammatory process, enteropathy associated T cell lymphoma, and peripheral T cell lymphoma.

Epidemiology and Demographics

Patients of all age groups may develop extranodal NK-T-cell lymphoma. Males are more commonly affected with extranodal NK-T-cell lymphoma than females. The majority of extranodal NK-T-cell lymphoma cases are reported in Asian, Central-American, and South-American countries.

Risk Factors

There are no established risk factors for extranodal NK-T-cell lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for extranodal NK-T-cell lymphoma.

Natural History, Prognosis, and Complications

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.

Diagnosis

Symptoms

The most common symptoms of extranodal NK-T-cell lymphoma include fever, weight loss, skin rash, night sweats, protrusion of eye, swelling of the face, discharge from the nose, nose bleeds, blockage of the nasal passages, chest pain, abdominal pain, bone pain, and painless swelling in the neck, axilla, groin, thorax, and abdomen.

Physical Examination

Common physical examination findings of extranodal NK-T-cell lymphoma include fever, rash, ulcer, proptosis, midfacial destructive lesions, epistaxis, nasal obstruction due to mass, chest tenderness, abdomen tenderness, bone tenderness, peripheral lymphadenopathy, and central lymphadenopathy.

Laboratory tests

Laboratory tests for extranodal NK-T-cell lymphoma include complete blood count (CBC), blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.