Granulocyte colony-stimulating factor corrects the neutropenia associated with glycogen storage disease type Ib.
A young woman with glycogen storage disease, type Ib, and chronic neutropenia had severe recurrent infections. In a life-threatening situation, treatment with granulocyte colony-stimulating factor (G-CSF) resulted in the prompt correction of neutropenia. Subsequently, daily G-CSF therapy has allowed the maintenance of a normal neutrophil count and marked clinical improvement over a period of 18 months. The spectrum of neutropenic conditions which are responsive to G-CSF should include this inherited metabolic disorder.
Wang
W C
WC
Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, TN 38101.
Crist
W M
WM
Ihle
J N
JN
Arnold
B A
BA
Keating
J P
JP
D000328:Adult; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use*; D006801:Humans; D009503:Neutropenia / Q000188:drug therapy* / Q000209:etiology; D011994:Recombinant Proteins / Q000627:therapeutic use
D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor