Lack of response of bone marrow, in vitro, to growth factors in congenital neutropenia.
Severe congenital neutropenia has a poor outlook. In vitro clonogenic assays using recombinant growth factors may improve understanding of the underlying pathogenetic mechanisms and identify those in whom growth factors might be clinically useful. Marrow from a boy with congenital neutropenia was cultured with a variety of recombinant growth factors. The results show that the neutropenia did not result from a lack of myeloid progenitors but that these progenitors could not produce mature neutrophils. Bone marrow transplantation is being considered as the most likely approach to correct neutropenia.
Chang
J
J
Department of Haematology, Christie Hospital, Manchester, United Kingdom.
Craft
A W
AW
Reid
M M
MM
Coutinho
L H
LH
Dexter
M
M
D000380:Agranulocytosis / Q000503:physiopathology*; D001853:Bone Marrow / Q000187:drug effects* / Q000473:pathology / Q000503:physiopathology; D002478:Cells, Cultured; D002648:Child; D003114:Colony-Forming Units Assay; D003115:Colony-Stimulating Factors / Q000494:pharmacology; D016179:Granulocyte Colony-Stimulating Factor; D016178:Granulocyte-Macrophage Colony-Stimulating Factor; D006133:Growth Substances / Q000494:pharmacology*; D006801:Humans; D007377:Interleukin-3 / Q000494:pharmacology; D008297:Male; D009503:Neutropenia / Q000151:congenital / Q000503:physiopathology*; D011994:Recombinant Proteins
D003115:Colony-Stimulating Factors; D006133:Growth Substances; D007377:Interleukin-3; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D016178:Granulocyte-Macrophage Colony-Stimulating Factor