Article
Truncated CSF3 receptors induce pro-inflammatory responses in severe congenital neutropenia.
British journal of haematology - 1 Jan 2023
Olofsen Patricia A, Bosch Dennis A, de Looper Hans W J, van Strien Paulina M H, Hoogenboezem Remco M, Roovers Onno, van der Velden Vincent H J, Bindels Eric M J, De Pater Emma M, Touw Ivo P
Abstract excerpt
Severe congenital neutropenia (SCN) patients are prone to develop myelodysplastic syndrome (MDS) or acute myeloid leukaemia (AML). Leukaemic progression of SCN is associated with the early acquisition of CSF3R mutations in haematopoietic progenitor cells (HPCs), which truncate the colony-stimulating factor 3 receptor (CSF3R). These mutant clones may arise years before MDS/AML becomes overt. Introduction and...
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