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

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213 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
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

Keywords

acute myeloid leukaemia
granulocyte colony-stimulating factor
myelodysplastic syndromes
severe congenital neutropenia

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10.1111/j.1365-2141.2010.08216.x

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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

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

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.",

keywords = "acute myeloid leukaemia, granulocyte colony-stimulating factor, myelodysplastic syndromes, severe congenital neutropenia",

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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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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AU - Rosenberg, Philip S.

AU - Zeidler, Cornelia

AU - Bolyard, Audrey A.

AU - Alter, Blanche P.

AU - Bonilla, Mary A.

AU - Boxer, Laurence A.

AU - Dror, Yigal

AU - Kinsey, Sally

AU - Link, Daniel C.

AU - Newburger, Peter E.

AU - Shimamura, Akiko

AU - Welte, Karl

AU - Dale, David C.

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AB - 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.

KW - acute myeloid leukaemia

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

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