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.
Original language | English (US) |
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Pages (from-to) | 196-199 |
Number of pages | 4 |
Journal | British Journal of Haematology |
Volume | 150 |
Issue number | 2 |
DOIs | |
State | Published - Jul 2010 |
Externally published | Yes |
Keywords
- acute myeloid leukaemia
- granulocyte colony-stimulating factor
- myelodysplastic syndromes
- severe congenital neutropenia
ASJC Scopus subject areas
- Hematology