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- Title
Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy.
- Authors
Rosenberg, Philip S; Zeidler, Cornelia; Bolyard, Audrey A; Alter, Blanche P; Bonilla, Mary A; Boxer, Laurence A; Dror, Yigal; Kinsey, Sally; Link, Daniel C; Newburger, Peter E; Shimamura, Akiko; Welte, Karl; Dale, David C
- 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.
- Publication
British journal of haematology, 2010, Vol 150, Issue 2, p196
- ISSN
1365-2141
- Publication type
Journal Article
- DOI
10.1111/j.1365-2141.2010.08216.x