Clonal disease of natural killer large granular lymphocytes in Taiwan.

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 lymphocytes (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 for 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.

journal_name

Br J Haematol

authors

Chou WC,Chiang IP,Tang JL,Su IJ,Huang SY,Chen YC,Liu MC,Lee FY,Wang CH,Shen MC,Chuang SM,Tien HF

doi

10.1046/j.1365-2141.1998.01109.x

subject

Has Abstract

pub_date

1998-12-01 00:00:00

pages

1124-8

issue

4

eissn

0007-1048

issn

1365-2141

journal_volume

103

pub_type

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