Title
Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy
UMMS Affiliation
Department of Pediatrics
Publication Date
2010-7
Document Type
Article
Subjects
Epidemiologic Methods; Granulocyte Colony-Stimulating Factor; Humans; Leukemia, Myeloid, Acute; Myelodysplastic Syndromes; Neutropenia
Disciplines
Hematology | Oncology | Pediatrics
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.
DOI of Published Version
10.1111/j.1365-2141.2010.08216.x
Source
Br J Haematol. 2010 Jul;150(2):196-9. Epub 2010 Apr 29. Link to article on publisher's website
Journal/Book/Conference Title
British journal of haematology
Related Resources
PubMed ID
20456363
Repository Citation
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. Hematology/Oncology. https://doi.org/10.1111/j.1365-2141.2010.08216.x. Retrieved from https://escholarship.umassmed.edu/peds_hematology/131