Abstract:
:Severe congenital neutropenia (CN) is a rare bone marrow failure syndrome with a high incidence of acute leukemia. In previous studies, we could show that point mutations in the gene for the granulocyte colony-stimulating factor (G-CSF) receptor CSF3R are a highly predictive marker for leukemic development in CN patients. To find out at which stage of hematopoietic development these mutations emerge and how they are propagated during hematopoietic differentiation, we analyzed single cells of different hematopoietic subpopulations from CN patients with CSF3R mutations. We found that CSF3R mutations are not restricted to the myeloid compartment but are also detectable in lymphoid cells, although at a much lower percentage. From our observations, we conclude that CSF3R mutations are acquired in multipotent hematopoietic progenitor cells in CN patients and that they are clonally expanded in myeloid cells expressing the G-CSF receptor due to the growth advantage mediated by the CSF3R mutation.
journal_name
Bloodjournal_title
Bloodauthors
Germeshausen M,Welte K,Ballmaier Mdoi
10.1182/blood-2008-09-178087subject
Has Abstractpub_date
2009-01-15 00:00:00pages
668-70issue
3eissn
0006-4971issn
1528-0020pii
blood-2008-09-178087journal_volume
113pub_type
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