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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 publication

DOI

10.1111/j.1365-2141.2009.07720.x

Type

Journal article

Journal

Br J Haematol

Publication Date

06/2009

Volume

146

Pages

86 - 90

Keywords

Antigens, CD34, Case-Control Studies, Disease Progression, Down-Regulation, Gene Expression Profiling, Humans, Immunohistochemistry, Lymphoid Enhancer-Binding Factor 1, Myelodysplastic Syndromes, Neutropenia, Oligonucleotide Array Sequence Analysis, RNA, Messenger, Reverse Transcriptase Polymerase Chain Reaction, Sequence Analysis, DNA