Natural killer cell neoplasms: A distinctive group of highly aggressive lymphomas/leukemias
Section snippets
Clinical features and behavior
Extranodal NK/T-cell lymphomas show a predilection for Asians, Mexicans, and South Americans.2, 10, 53, 77 It most commonly involves the nasal cavity, which is the prototype of this form of lymphoma. The median age of patients is 53 years, and the male to female ratio is about 3:1.3, 14
Clinical features and behavior
Aggressive NK cell leukemia represents the leukemic end in the range of NK cell neoplasms, analogous to the acute lymphoblastic leukemia-lymphoblastic lymphoma spectrum and chronic lymphocytic leukemia-small lymphocytic lymphoma spectrum.12, 33 While there are usually easily identified neoplastic cells in the peripheral blood and bone marrow, involvement can be subtle, rendering a distinction from disseminated extranodal NK/T-cell lymphoma difficult. Hypersensitivity to mosquito bites may
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EBV–Associated Lymphoproliferative Disorders: Update in Classification
2019, Surgical Pathology ClinicsCitation Excerpt :This hypothesis is mainly based on the similar ethnic background, morphologic picture, immunophenotype, strong EBV association, and genotype (lack of T-cell receptor gene rearrangements).135 At present, the most accepted differentiating features between the two entities are that aggressive NK-cell leukemia often presents at a younger age (almost a decade younger, with a median age of 40 years), shows B symptoms, has more frequent hepatosplenic and bone marrow involvement, has a lower frequency of cutaneous involvement and more frequent expression of CD16 (positive in 75% of cases), and usually disseminates early in presentation.136–138 Moreover, an array-based comparative genomic hybridization study has reported significance differences in genetic changes between the two disorders, in which 7p−, 17p−, and 1q+ are more frequent in aggressive NK-cell leukemia, whereas 6q− is more common in nasal NK-cell lymphoma.139
Malignant Neoplasms of the Nasal Cavity, Paranasal Sinuses, and Nasopharynx
2019, Head and Neck Pathology: A Volume in the Series: Foundations in Diagnostic PathologyEpstein-Barr virus (EBV)–associated lymphoid proliferations, a 2018 update
2018, Human PathologyCD21-independent Epstein-Barr virus entry into NK cells
2018, Cellular ImmunologyCitation Excerpt :Epstein-Barr virus (EBV), a member of the γ-herpesvirus family, is one of the most common viruses and persists in a majority of humans as a life-long, asymptomatic infection within B lymphocytes [1]. Extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKTCL) is a rare EBV-associated non-Hodgkin lymphoma, which affects the nasal cavity [3] and upper aerodigestive tract [4] and is a major subset of NK cell, or more rarely T-cell, neoplasms [2]. The transforming properties of EBV and its role in the pathogenesis of a range of B cell malignancies are well established.
Diagnostic Pathology: Blood and Bone Marrow
2018, Diagnostic Pathology: Blood and Bone MarrowFlow Cytometric Principles in Hematopathology
2018, Hematopathology: A Volume in the Series: Foundations in Diagnostic Pathology
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*Address correspondence to Dr Michael M.C. Cheung, Department of Clinical Oncology, Room 1116, 11/F, Block R, Queen Elizabeth Hospital, Gascoigne Road, Kowloon, Hong Kong