Blood, 1 February 2003, Vol. 101, No. 3, pp. 1203-1203
CORRESPONDENCE
To the editor:
Polymorphic expression of CD158k/p140/KIR3DL2 in Sézary
patients
Sézary syndrome (SS) is a leukemic form of epidermotropic
cutaneous T-cell lymphoma (CTCL) characterized by the rapid onset of a
pruriginous erythroderma with diffuse adenopathies. Sézary cells
are lymphocytes with a typical cerebriform nucleus and a CD4+CD45RO+ phenotype. No specific cell
membrane receptor has been described until now. Recently, we reported
that circulating and cutaneous Sézary cells express
CD158k/p140-KIR3DL2.1,2 This transmembrane receptor is a
member of the killer cell Ig-like receptors that inhibit
natural killer (NK)-mediated lysis after interaction with HLA-A. In healthy individuals, KIR3DL2 is only detected on
minor NKs and CD3+CD8+ subsets.3
Until now, 9 KIR3DL2 alleles have been
defined.4 We reported the restricted expression of the
KIR3DL2 008 allele from 2/2 CTCL lines derived from
Sézary patients, suggesting that expression of this allelic form
could be associated to CTCL malignancies.1 The role of
KIR3DL2 on CTCL cells is still unknown. It could be possible that the
KIR3DL2 polymorphism might influence the recognition of
HLA-I alleles. In order to determine whether Sézary cells express
a specific KIR3DL2 allelic form, we have determined the
repertoire of KIR3DL2 expression in 14 patients. Diagnosis
of Sézary syndrome was based on clinical criteria (erythroderma, pruritus, palmoplantar keratoderma, diffuse adenopathies) and biologic criteria, including typical circulating Sézary cells (> 1000 cells/µL), histologic data (cutaneous
epidermotropic T-cell lymphoma), and detection of an identical T-cell
clone in the blood and in the skin by qualitative polychain
reaction (PCR)-denaturing gradient gel electrophoresis (DGGE) 
.
Peripheral blood lymphocytes (PBLs) were isolated from heparinized
venous blood samples by centrifugation over Ficoll-Hypaque (Eurobio,
Paris, France). DNA was extracted using Dneasy tissue kit (Quiagen,
Valencia, CA). Three micrograms DNA was PCR-amplified through
40 cycles, with 2 different conditions depending on the KIR3DL2-specific primers used as described.3
Taq polymerase from Promega (Madison, WI), dNTP 10 mM each (Boehringer
Mannheim, Roche Diagnostics, Meylan, France) and 25 mM
Mg2+. The amplification products were analyzed on a 1%
agarose gel.
The results indicate that in all patients KIR3DL2 cDNA PCR
amplification gave good bands. Ten of 14 patients expressed 2 different alleles, including 009/007 (1 patient), 002/005 (1 patient), 007/006 (1 patient), 002/007 (3 patients), 003/007 (3 patients), and 005/007 (1 patient), whereas 4 of 14 patients were homozygous including 009 (2 patients), 002 (1 patient), and 001 (1 patient) alleles. None of
the patients was homozygous for KIR3DL2 008 found expressed in CTCL line.1 These results demonstrate that there is no
preferential allelic expression of KIR3DL2 associated with Sézary syndrome.
Philippe Musette, Laurence Michel, Francett Jean-Louis, Martine Bagot, and Armand Bensussan
Correspondence: Armand Bensussan, INSERM 448, Faculte de Medecine de Créteil, Créteil,
France
References
1.
Bagot M, Moretta M, Sivori S, et al.
CD4+ cutaneous T-cell lymphoma cells express the P140-killer cell immunoglobulin-like receptor.
Blood.
2001;97:1388-1391[Abstract/Free Full Text].
2.
Nikolova M, Bagot M, Boumsell L, Bensussan A.
Identification of cell surface molecules characterizing human cutaneous T-cell lymphomas.
Leuk Lymph.
2002;43:741-746[CrossRef][Medline]
[Order article via Infotrieve].
3.
Mingari MC, Moretta A, Moretta L.
Regulation of KIR expression in human T lymphocytes: a safety mechanism which may impair protective T cell responses.
Immunol Today.
1998;19:153-157[CrossRef][Medline]
[Order article via Infotrieve].
4.
Gardiner CM, Guethlein LA, Shilling HG, et al.
Different NK cell surface phenotypes defined by DX9 antibody are due to KIR3DL1 gene polymorphism.
J Immunol.
2001;166:2992-3001[Abstract/Free Full Text].
