Open AccessA case of secondary acute myeloid leukemia on a background of glycogen storage disease with chronic neutropenia treated with granulocyte colony stimulating factor
Author(s) -
Khalaf Dina,
Bell Heather,
Dale David,
Gupta Vikas,
Faghfoury Hanna,
Morel Chantal F.,
Tierens Anne,
Weinstein David A.,
Yan Jiong,
Thyagu Santhosh,
Maze Dawn
Publication year - 2019
Publication title -
jimd reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.412
H-Index - 25
ISSN - 2192-8312
DOI - 10.1002/jmd2.12069
Subject(s) - congenital neutropenia , medicine , neutropenia , granulocyte colony stimulating factor , myeloid leukemia , glycogen storage disease , glycogen storage disease type i , granulocyte , leukopenia , immunology , myeloid , myelodysplastic syndromes , disease , chemotherapy , bone marrow
Congenital neutropenias due to mutations in ELANE, SBDS or HAX1 or in the setting of glycogen storage disease (GSD) which is caused by SLC37A4 mutation, often require prolonged granulocyte colony stimulating factor (G‐CSF) therapy to prevent recurrent infections and hospital admission. There has been emerging evidence that prolonged exposure to G‐CSF in cases with congenital neutropenia other than GSD is associated with transformation to myelodysplastic syndrome/acute myeloid leukemia.