Abstract
We recently reported on the risk for death from sepsis and myelodysplastic syndrome/acute myeloid leukemia (MDS/AML) in patients with severe congenital neutropenia (SCN) on long-term granulocyte-colony stimulating factor (G-CSF) enrolled in the Severe Chronic Neutropenia International Registry (SCNIR). (
Rosenberg et al,
, and Blood
2006
; 107
:4628
-35Brit J Haematol
2008
; 140
:210
-3Donini, et al.
), the predominant problem is a poor ANC response; the biological basis for poor responses of some SCN patients to G-CSF is still unknown. Based on these data, we recommend careful consideration of a match related or unrelated donor hematopoietic stem cell transplant for all patients not achieving a consistent ANC >1.0 × 109 with G-CSF therapy.Blood
109
:4716
, 2007
Disclosures: Boxer:Amgen: Equity Ownership. Dale:Amgen: Consultancy; NIH: Research Funding; Merck: Research Funding; Genzyme: Research Funding; Cellerant: Consultancy; Telik: Consultancy; Schering-Plough: Consultancy; Caremark: Consultancy.
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Corresponding author
2008, The American Society of Hematology
2008
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