Clonal Disease of Natural Killer-Large Granular Lymphocytes(Lgl) in Taiwan
Resource
BRITISH JOURNAL OF HAEMATOLOGY v.103 n.4 pp.1124-1128
Journal
BRITISH JOURNAL OF HAEMATOLOGY
Journal Volume
v.103
Journal Issue
n.4
Pages
1124-1128
Date Issued
1998
Date
1998
Author(s)
CHOU, WEN-CHIEN
CHIANG, I-PING
TANG, JIH-LUH
SU, IH-JEN
HUANG, SHANG-YI
CHEN, YAO-CHANG
LIU, MING-CHI
LEE, FEN-YU
WANG, CHIU-HWA
SHEN, MING-CHING
CHUANG, SOU-MING
TIEN, HWEI-FANG
Abstract
Lymphoproliferative diseases of large granular lymphocytes ( LDGL) may arise from either CD3(+) T cells or CD3(-) natural killer (NK) cells. LDGL with clonal proliferation of large granular lymphoeytes (LGL) is defined as LGL leukaemia. The number of patients with NK-LGL leukaemia reported is limited and the pathogenesis of the disease is not yet clear. From 1991 to 1998 six patients with cytogenetically proved clonal disease of NK-LGL were identified in our institute. All were seropositive far Epstein-Barr virus (EBV). EBV RNA or DNA could be detected in LGL from four patients by EBV in situ hybridization or Southern blot analysis. Most patients ran an aggressive clinical course and five died of the disease. Nonrandom clonal chromosomal abnormalities, including duplication of 1q, rearrangement at 3q and loss of chromosomes Y, 13 or 10, were noted in the six patients from this study and in eight from the literature. The implications of these recurrent cytogenetic aberrations in the development and progression of the disease deserve further studies.