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

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

doi:10.1111/j.1365-2141.2010.08216.x

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

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

Research output: Contribution to journal › Article › peer-review

146 Scopus citations

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)
Pages (from-to)	196-199
Number of pages	4
Journal	British Journal of Haematology
Volume	150
Issue number	2
DOIs	https://doi.org/10.1111/j.1365-2141.2010.08216.x
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

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Rosenberg, P. S., Zeidler, C., Bolyard, A. A., Alter, B. P., Bonilla, M. A., Boxer, L. A., Dror, Y., Kinsey, S., Link, D. C., Newburger, P. E., Shimamura, A., Welte, K., & Dale, D. C. (2010). Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy: Short report. British Journal of Haematology, 150(2), 196-199. https://doi.org/10.1111/j.1365-2141.2010.08216.x

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 2010, 'Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy: Short report', British Journal of Haematology, vol. 150, no. 2, pp. 196-199. https://doi.org/10.1111/j.1365-2141.2010.08216.x

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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.",

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author = "Rosenberg, {Philip S.} and Cornelia Zeidler and Bolyard, {Audrey A.} and Alter, {Blanche P.} and Bonilla, {Mary A.} and Boxer, {Laurence A.} and Yigal Dror and Sally Kinsey and Link, {Daniel C.} and Newburger, {Peter E.} and Akiko Shimamura and Karl Welte and Dale, {David C.}",

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AU - Bonilla, Mary A.

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AU - Welte, Karl

AU - Dale, David C.

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

Abstract

Keywords

ASJC Scopus subject areas

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