Publications Search - Abstract View
| 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. |