title: Enteropathy-type T-cell lymphoma expressing NK-cell intraepithelial lymphocyte (NK-IEL) phenotype.
abstract: Enteropathy-type T-cell lymphoma (ETL) is an intraepithelial T-lymphocyte (T-IEL) tumor. The tumor cells are usually CD3+, CD4-, CD8+, and contain cytotoxic granule associated proteins. We report on a CD3-negative CD56-positive enteropathy-associated lymphoma (ETL). This is the first case report of CD3-negative, CD56-positive, CD94-negative, and CD161-positive ETL. ETL cells originate from intraepithelial T-lymphocytes of the intestine. CD3-negative intraepithelial lymphocytes are known as natural killer (NK)-IELs. The phenotype of NK-IELs is also CD3-negative, CD56-positive, CD94-negative, and CD161-positive, while most normal NK cells express CD56 and CD94. CD3-negative lymphoma cells in this report also expressed CD56 and CD161, but not CD94. Because Southern blotting analysis showed a rearrangement of T-cell receptor (TCR) Cbeta in this case, the tumor is classified as an ETL. Based on the findings, NK-IELs may originate from T-cells, not NK-cells.
authors.affiliation: Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
authors.forename: Naoko
authors.initials: N
authors.lastname: Inagaki
authors.forename: Daisuke
authors.initials: D
authors.lastname: Asaoka
authors.forename: Kiyoshi L
authors.initials: KL
authors.lastname: Mori
authors.forename: Naomi
authors.initials: N
authors.lastname: Sohda
authors.forename: Ichiro
authors.initials: I
authors.lastname: Miura
authors.forename: Hiroto
authors.initials: H
authors.lastname: Miwa
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Sato
authors.forename: Kazuo
authors.initials: K
authors.lastname: Oshimi
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D017252:CD3 Complex / Q000032:analysis; D019002:CD56 Antigen / Q000032:analysis; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D016099:Endoscopy, Gastrointestinal; D017809:Fatal Outcome; D005770:Gastrointestinal Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D016130:Immunophenotyping; D007416:Intestinal Perforation / Q000209:etiology; D007694:Killer Cells, Natural / Q000737:chemistry / Q000473:pathology*; D017493:Leukocyte Common Antigens / Q000032:analysis; D016246:Lymphocytes, Tumor-Infiltrating; D016399:Lymphoma, T-Cell / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D008297:Male; D008875:Middle Aged; D014411:Neoplastic Stem Cells / Q000737:chemistry; D010538:Peritonitis / Q000209:etiology; D010641:Phenotype; D011241:Prednisone / Q000008:administration & dosage; D011504:Protein-Losing Enteropathies / Q000209:etiology*; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D017252:CD3 Complex; D019002:CD56 Antigen; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D017493:Leukocyte Common Antigens; D011241:Prednisone