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Title: Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy.
Authors: Rosenberg PS,  Zeidler C,  Bolyard AA,  Alter BP,  Bonilla MA,  Boxer LA,  Dror Y,  Kinsey S,  Link DC,  Newburger PE,  Shimamura A,  Welte K,  Dale DC
Journal: Br J Haematol
Date: 2010 Jul
Branches: BB, CGB
PubMed ID: 20456363
PMC ID: PMC2906693
Abstract: In severe congenital neutropenia (SCN), long-term therapy with granulocyte colony-stimulating factor (G-CSF) has reduced mortality from sepsis, revealing an underlying predisposition to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We have reported the early pattern of evolution to MDS/AML, but the long-term risk remains uncertain. We updated a prospective study of 374 SCN patients on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. Long-term, the annual risk of MDS/AML attained a plateau (2.3%/year after 10 years). This risk now appears similar to, rather than higher than, the risk of AML in Fanconi anaemia and dyskeratosis congenita.