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 journalArticlepeer-review

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 languageEnglish (US)
Pages (from-to)196-199
Number of pages4
JournalBritish Journal of Haematology
Volume150
Issue number2
DOIs
StatePublished - Jul 2010
Externally publishedYes

Keywords

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

ASJC Scopus subject areas

  • Hematology

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