title: Chronic T-cell lymphoproliferative disease expressing natural killer cell receptors: clinicopathological and molecular features.
abstract: The frequency and clinicopathological significance of the expression of natural killer cell receptors (NKRs) in T-cell malignancies remain undefined. A 71-year-old man presented with leukocytosis, generalized lymphoadenopathy, and hepatosplenomegaly. Bone marrow and lymph node biopsies showed a T-cell lymphoproliferative disease expressing NKRs (CD2(+), CD3(+), CD4(+), CD5(+), CD7(+), CD8(-), CD56(-), CD94(+), CD158a(+), CD158b(+), CD161(-), p70(-), TCRalphabeta(1), TCRgammadelta(2), TIA-1(-)). An abnormal clone, 46,Y,add(X)(p14),der(1)t(1;6)(p33;p21),t(7;12)(p10;q10), was found on conventional karyotyping. Comparative genomic hybridization confirmed these findings, and showed a deletion of 12p that was not apparent on karyotyping. Clinically, the disease remained indolent and responded transiently to purine analogs but not to intensive chemotherapy. Peripheral T-cell lymphoproliferative disease of CD4(+)alphabeta(1)NKR(+) phenotype is hitherto undescribed. The issues of whether this case was derived from transformation of a rare T-cell subtype or represented aberrant T-cell expression of NK-cell antigens, and the clinicopathologic significance of these T-cell neoplasms warrant further studies.
authors.affiliation: Department of Medicine, Professorial Block, Queen Mary Hospital, Pokfulam Road, Hong Kong, China. ylkwong@hkucc.hku.hk
authors.forename: Y L
authors.initials: YL
authors.lastname: Kwong
authors.forename: C C
authors.initials: CC
authors.lastname: Lam
authors.forename: C
authors.initials: C
authors.lastname: Choy
authors.forename: C
authors.initials: C
authors.lastname: Man
authors.forename: W Y
authors.initials: WY
authors.lastname: Au
authors.forename: L L
authors.initials: LL
authors.lastname: Siu
mesh_terms: D000740:Anemia / Q000209:etiology; D015703:Antigens, CD / Q000032:analysis / Q000096:biosynthesis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001854:Bone Marrow Cells / Q000378:metabolism / Q000473:pathology; D002872:Chromosome Deletion; D002908:Chronic Disease; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D007621:Karyotyping; D007694:Killer Cells, Natural / Q000378:metabolism*; D008206:Lymphatic Diseases / Q000209:etiology; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000235:genetics* / Q000378:metabolism / Q000628:therapy; D008297:Male; D009693:Nucleic Acid Hybridization; D016066:Pleural Effusion, Malignant / Q000209:etiology; D011948:Receptors, Antigen, T-Cell / Q000032:analysis / Q000096:biosynthesis* / Q000145:classification; D013601:T-Lymphocytes / Q000378:metabolism* / Q000473:pathology; D014178:Translocation, Genetic / Q000235:genetics
chemical_list: D015703:Antigens, CD; D011948:Receptors, Antigen, T-Cell