Title

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

UMMS Affiliation

Department of Pediatrics

Date

7-2010

Document Type

Article

Medical Subject Headings

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.

Rights and Permissions

Citation: Br J Haematol. 2010 Jul;150(2):196-9. Epub 2010 Apr 29. Link to article on publisher's website

Related Resources

Link to article in PubMed

PubMed ID

20456363