title: [Aggressive natural killer cell lymphoproliferative disease of large granular lymphocytes with leukemia-like clinical course in the terminalstage].
abstract: A 20-year-old man was admitted to our clinic with fever elevation up to 39 degrees C for two months, generalized lymphadenopathy and hepatosplenomegaly. Histological examination of right scalene lymph node with HE staining showed T cell lymphoma-like finding. The patient was given vindesine and prednisolone, and there was almost no clinical improvement. Abnormal large granular lymphocyte appeared in peripheral blood and increased up to 17,000/microliters in the terminal stage of clinical course. These lymphocytes had abundant pale cytoplasm with rich large azurophilic granules and a large nucleus with a few nucleoli. The phenotype of these cells were as follows: Fc gamma R+, CD2+, CD5-, CD7-, CD3-, CD4-, CD1-, CD8-, sIg-, CD20-, CD11-, CD13-, OKIa+, CD25-, CD16+, Leu7-. These cells did not have the activity of antibody dependent cellular cytotoxicity but had natural killer activity. The gene of T cell receptor (beta and gamma chain) did not rearranged in these cells. We concluded that the abnormal cells were derived from natural killer cells, which caused aggressive clinical course.
authors.affiliation: Second Department of Internal Medicine, Mie University School of Medicine.
authors.forename: T
authors.initials: T
authors.lastname: Tsukada
authors.forename: T
authors.initials: T
authors.lastname: Ohno
authors.forename: K
authors.initials: K
authors.lastname: Morita
authors.forename: H
authors.initials: H
authors.lastname: Otsuji
authors.forename: T
authors.initials: T
authors.lastname: Sekine
authors.forename: M
authors.initials: M
authors.lastname: Nishikawa
authors.forename: T
authors.initials: T
authors.lastname: Kobayashi
authors.forename: K
authors.initials: K
authors.lastname: Kita
authors.forename: S
authors.initials: S
authors.lastname: Shirakawa
authors.forename: K
authors.initials: K
authors.lastname: Namba
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D006801:Humans; D007694:Killer Cells, Natural / Q000473:pathology*; D008232:Lymphoproliferative Disorders / Q000276:immunology / Q000473:pathology*; D008297:Male; D013163:Splenomegaly; D013601:T-Lymphocytes / Q000473:pathology
chemical_list: D015703:Antigens, CD