Marked downregulation of the granulopoiesis regulator LEF1 is associated with disease progression in the myelodysplastic syndromes

Andrea Pellagatti, Teresa Marafioti, Jennifer C. Paterson, Luca Malcovati, Matteo G. Della Porta, Martin Jädersten, Beena Pushkaran, Tracy I. George, Daniel A. Arber, Sally Killick, Aristoteles Giagounidis, Eva Hellström-Lindberg, Mario Cazzola, James S. Wainscoat, Jacqueline Boultwood

Research output: Contribution to journalArticle

Abstract

Lymphoid enhancer-binding factor 1 (LEF1) is a neutrophilic granulopoiesis regulator whose absence is critical in congenital neutropenia. We have shown LEF1 downregulation in the CD34 + cells of the majority of myelodysplastic syndromes (MDS) patients. LEF1 was the most significant differentially expressed gene between early and advanced MDS. Marked LEF1 downregulation was found in 27/32 patients with advanced MDS and in 6/35 patients with early MDS, and was associated with neutropenia. Downregulation of LEF1 mRNA was reflected at the protein level. Immunostaining for CD34/LEF1 may represent a marker of advanced MDS. LEF1 may play a role in the defective maturation of myeloid progenitors in MDS.

Original languageEnglish
Pages (from-to)86-90
Number of pages5
JournalBritish Journal of Haematology
Volume146
Issue number1
DOIs
Publication statusPublished - Jul 2009

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Keywords

  • CD34 cells
  • LEF1
  • MDS
  • Neutropenia

ASJC Scopus subject areas

  • Hematology

Cite this

Pellagatti, A., Marafioti, T., Paterson, J. C., Malcovati, L., Della Porta, M. G., Jädersten, M., Pushkaran, B., George, T. I., Arber, D. A., Killick, S., Giagounidis, A., Hellström-Lindberg, E., Cazzola, M., Wainscoat, J. S., & Boultwood, J. (2009). Marked downregulation of the granulopoiesis regulator LEF1 is associated with disease progression in the myelodysplastic syndromes. British Journal of Haematology, 146(1), 86-90. https://doi.org/10.1111/j.1365-2141.2009.07720.x