Myeloid progenitor cell growth characteristics and effect of G-CSF in a patient with congenital cyclic neutropenia.
A 17-year-old male with congenital cyclic neutropenia was treated with recombinant human granulocyte colony stimulating factor (G-CSF) administered subcutaneously at 1 to 2 micrograms/kg per day. The peak and nadir counts of neutrophils and the peak counts of monocytes were significantly elevated, and the period of cycling decreased from 3 to 2 weeks. Bone marrow culture studies revealed the following abnormalities in granulocytic progenitor cells (CFU-G): a decrease in the concentrations of G-cluster forming cells, stimulated by a maximal dose of G-CSF, and a tendency of abnormally low responsive growth of the CFU-G to lower concentrations of G-CSF and GM-CSF. Our findings suggest that administration of G-CSF at relatively low doses overcomes or compensates for these abnormalities, though not completely, as fluctuation in the neutrophil counts persisted.
Tsunogake
S
S
Third Department of Internal Medicine, Dokkyo University School of Medicine, Tochigi, Japan.
Nagashima
S
S
Maekawa
R
R
Takano
N
N
Kajitani
H
H
Saito
K
K
Enokihara
H
H
Furusawa
S
S
Shishido
H
H
D001853:Bone Marrow / Q000473:pathology; D002478:Cells, Cultured; D002648:Child; D016179:Granulocyte Colony-Stimulating Factor / Q000494:pharmacology / Q000627:therapeutic use*; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000494:pharmacology; D006410:Hematopoiesis / Q000187:drug effects*; D006412:Hematopoietic Stem Cells / Q000187:drug effects* / Q000473:pathology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007958:Leukocyte Count / Q000187:drug effects; D008297:Male; D009503:Neutropenia / Q000151:congenital / Q000473:pathology* / Q000628:therapy
D007155:Immunologic Factors; D016179:Granulocyte Colony-Stimulating Factor; D016178:Granulocyte-Macrophage Colony-Stimulating Factor