期刊
BRITISH JOURNAL OF HAEMATOLOGY
卷 150, 期 2, 页码 196-199出版社
WILEY-BLACKWELL PUBLISHING, INC
DOI: 10.1111/j.1365-2141.2010.08216.x
关键词
severe congenital neutropenia; acute myeloid leukaemia; myelodysplastic syndromes; granulocyte colony-stimulating factor
类别
资金
- NIH, National Cancer Institute, Division of Cancer Epidemiology and Genetics
- NIH [2R24AI049393]
- German Ministry for Education and Research, BMBF [01 GM0618]
P>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 center dot 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.
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