Abstract：
:Natural killer (NK)-cell malignancies are uncommon diseases. Previously known as polymorphic reticulosis or angiocentric T-cell lymphomas, they are classified by the World Health Organization as NK/T-cell lymphoma, nasal type and aggressive NK-cell leukemia. They are prevalent in Asia and South America, but exceptionally rare in western countries. Pathologically, NK-cell lymphomas show a polymorphic neoplastic infiltrate with an angioinvasive and angiodestructive pattern. Lymphoma cells are characteristically CD2+, CD56+ and cytoplasmic CD3epsilon+. T-cell receptor gene is germline, and clonal Epstein-Barr virus (EBV) infection is almost invariably. Clinically, they can be divided into nasal, non-nasal, and aggressive lymphoma/leukemia subtypes. Most nasal NK-cell lymphomas present with stage I/II disease, and frontline radiotherapy is the most important key to successful treatment. Many stage I/II patients treated with radiotherapy fail systemically, implying that concomitant chemotherapy may be needed. Chemotherapy is indicated for advanced nasal NK-cell lymphoma, and the non-nasal and aggressive subtypes. However, treatment results are unsatisfactory. High-dose chemotherapy with hematopoietic stem cell transplantation may be beneficial to selected patients. The International Prognostic Index and presentation EBV DNA load is of prognostic significance and may be useful in the stratification of patients for various treatment modalities.
journal_name
Leukemiajournal_title
Leukemiaauthors
Kwong YLdoi
10.1038/sj.leu.2403955subject
Has Abstractpub_date
2005-12-01 00:00:00pages
2186-94issue
12eissn
0887-6924issn
1476-5551pii
2403955journal_volume
19pub_type
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