Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy.

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.

• Authors: Philip S Rosenberg, Cornelia Zeidler, Audrey A Bolyard, Blanche P Alter, Mary A Bonilla, Laurence A Boxer, Yigal Dror, Sally Kinsey, Daniel C Link, Peter E Newburger, Akiko Shimamura, Karl Welte, David C Dale
• Journal: British journal of haematology (Br J Haematol) Vol. 150 Issue 2 Pg. 196-9 (Jul 2010) ISSN: 1365-2141 [Electronic] England
• PMID: 20456363 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, N.I.H., Intramural)

Chemical References

• Granulocyte Colony-Stimulating Factor

Topics

• Epidemiologic Methods
• Granulocyte Colony-Stimulating Factor (therapeutic use)
• Humans
• Leukemia, Myeloid, Acute (etiology)
• Myelodysplastic Syndromes (etiology)
• Neutropenia (complications, congenital, drug therapy)