Tumor cells of patients with cutaneous T-cell lymphoma (CTCL) have the cell
surface phenotype of mature T-helper lymphocytes, and it may be impossible
to differentiate them from nonmalignant lymphocytes in skin and blood. Unt
il now, no specific cell membrane marker of CTCL has been reported. In the
current study, it is reported for the first time that CTCL cells express th
e major histocompatibility complex class I binding p140-killer cell immunog
lobulin-like receptor, which has been described on a minor subset of natura
l kilter lymphocytes and on a marginal circulating CD8(+) T lymphocyte subs
et. Interestingly, the molecular characterization of this KIR expressed by
CTCL allowed us to isolate a novel allelic form of p140-KIR3DL, resulting i
n 4 amino acid substitutions, 3 in the extracellular immunoglobulin-like do
main of the protein and one in the cytoplasmic region. This finding is like
ly to be important both for the pathophysiology and for the clinical treatm
ent of patients with CTCL. (Blood, 2001;97: 1388-1391) (C) 2001 by The Amer
ican Society of Hematology.