Lack of expression of inhibitory KIR3DL1 receptor in patients with natural killer cell-type lymphoproliferative disease of granular lymphocytes

Cristina Gattazzo, Antonella Teramo, Marta Miorin, Elisa Scquizzato, Anna Cabrelle, Mirna Balsamo, Carlo Agostini, Elena Vendrame, Monica Facco, Maria Paola Albergoni, Livio Trentin, Massimo Vitale, Gianpietro Semenzato, Renato Zambello

Research output: Contribution to journalArticle

Abstract

Background Natural killer cell-type lymphoproliferative disease of granular lymphocytes is a disorder characterized by chronic proliferation of CD3-CD16+ granular lymphocytes. By flow cytometry analysis, we previously demonstrated a dysregulation in killer immunoglobulin-like receptor (KIR) expression in natural killer cells from patients with this lymphoproliferative disease, the activating KIR receptors being mostly expressed. We also found that patients with natural killer cell-type lymphoproliferative disease of granular lymphocytes usually had KIR genotypes characterized by multiple activating KIR genes. Design and Methods We investigated the mRNA levels of the KIR3DL1 inhibitory and the related KIR3DS1 activating receptors in 15 patients with natural killer cell-type lymphoproliferative disease of granular lymphocytes and in ten controls. These genes are usually expressed when present in the genome of the Caucasian population. Results We demonstrated the complete lack of KIR3DL1 expression in most of the patients analyzed, with the receptor being expressed in 13% of patients compared to in 90% of controls (P

Original languageEnglish
Pages (from-to)1722-1729
Number of pages8
JournalHaematologica
Volume95
Issue number10
DOIs
Publication statusPublished - Oct 2010

Keywords

  • KIR3DL1 down-regulation
  • Lymphoproliferative disease of granular lymphocytes
  • Natural killer cells

ASJC Scopus subject areas

  • Hematology

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    Gattazzo, C., Teramo, A., Miorin, M., Scquizzato, E., Cabrelle, A., Balsamo, M., Agostini, C., Vendrame, E., Facco, M., Albergoni, M. P., Trentin, L., Vitale, M., Semenzato, G., & Zambello, R. (2010). Lack of expression of inhibitory KIR3DL1 receptor in patients with natural killer cell-type lymphoproliferative disease of granular lymphocytes. Haematologica, 95(10), 1722-1729. https://doi.org/10.3324/haematol.2010.023358