title: Large natural killer cell lymphoma arising from an indolent natural killer cell large granular lymphocyte proliferation.
abstract: Natural killer cell large granular lymphocyte proliferation is a relatively rare disorder that typically runs a chronic, indolent course. We present a patient with a 3 1/2-year history of natural killer cell large granular lymphocyte proliferation terminating in large cell lymphoma with natural killer cell features. The diagnosis of natural killer cell large granular lymphocyte proliferation was based on flow cytometric demonstration of an expanded population of CD3- CD16+/CD56+ lymphocytes in the peripheral blood. The patient experienced various rheumatologic symptoms, but was hematologically stable for 3 1/2 years. He then developed fevers, night sweats, weight loss, and a left lower lobe lung mass. Resection of the mass showed a large cell lymphoma with immunohistochemical positivity for CD2, CD7, CD56, and T-cell intracellular antigen-1, compatible with natural killer cell origin. In situ hybridization for Epstein-Barr virus and polymerase chain reaction analysis for T-cell receptor gene rearrangement were negative. To our knowledge, this is the second documented report of chronic natural killer cell large granular lymphocyte proliferation terminating in an aggressive large natural killer cell lymphoma.
authors.affiliation: Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, 6 Founders Pavilion, 3400 Spruce St, Philadelphia, PA 19104, USA. roulletm@uphs.upenn.edu
authors.forename: Michele R
authors.initials: MR
authors.lastname: Roullet
authors.forename: Dennis B
authors.initials: DB
authors.lastname: Cornfield
mesh_terms: D017252:CD3 Complex / Q000097:blood; D019002:CD56 Antigen / Q000097:blood; D002908:Chronic Disease; D005434:Flow Cytometry; D006098:Granulocytes / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007694:Killer Cells, Natural / Q000473:pathology*; D008214:Lymphocytes / Q000378:metabolism; D016403:Lymphoma, Large B-Cell, Diffuse / Q000209:etiology* / Q000473:pathology*; D008232:Lymphoproliferative Disorders / Q000097:blood / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D039102:Poly(A)-Binding Proteins / Q000097:blood; D017452:Receptors, IgG / Q000097:blood; D000074019:T-Cell Intracellular Antigen-1
chemical_list: D017252:CD3 Complex; D019002:CD56 Antigen; D039102:Poly(A)-Binding Proteins; D017452:Receptors, IgG; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human