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Mature T cell and natural killer (NK) cell neoplasms

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Overview

Mature T cell and natural killer (NK) cell neoplasms are rare tumors that result from clonal proliferation of mature post-thymic lymphocytes, which make up about 10 to 12% of all lymphoid cancers. The diseases are usually diagnosed in adults with a age ranging from 17 to 90, and in the total population of this group, men, more than women are manifest to develop these neoplasms. The World Health Organization classifies this group of disorders into NK/T cell lymphoma, nasal type (NKTCL), aggressive NK-cell leukemia (ANKCL) and chronic lymphoproliferative disorders of NK-cells. As a group of biologically and clinically heterogeneous disorders, most NKTCL is exclusively extranodal with the nose and nasopharynx as its common initial sites, whereas ANKCL manifests to be a systemic disease with multiorgan involvement and rapidly evolutes to death.

Signs, symptoms and treatments

Nasal NKTCL patients present symptoms like mass, obstruction swelling, or bleeding, while the ANKCL population shows fever and other extreme systemic symptoms, such as hepatosplenomegaly, pancytopenia and abnormal liver function. Systemic chemotherapy is still the major treatment for unspreaded NK/T-cell lymphomas, but the hematopoietic stem cell transplantation hasn't been prospectively evaluated. Meanwhile, combined chemotherapy-radiotherapy and combination of chemotherapy are also the current treatment strategies depending on the grade and symptoms of the disease.

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