title: IL-3 increases marrow and peripheral erythroid precursors in chronic pure red cell aplasia presenting in childhood.
abstract: In most patients with Diamond-Blackfan anaemia (DBA) the addition of IL-3 to in vitro cultures increases the number of erythroid progenitors. However, a small proportion of patients show an erythroid response to in vivo administration of IL-3. We treated two adult patients with corticosteroid-refractory DBA, who had shown a clear in vitro erythroid response to IL-3, with increasing doses of IL-3 (from 2.5 to 10 micrograms/kg/d) by subcutaneous injections for 7 and 9 weeks. Monitoring of marrow and circulating progenitors showed a sustained elevation of BFU-E and CFU-E as well as an increase of other progenitors and CD34+ cells during therapy. However, this effect did not translate into a clear clinical response, although transfusion requirements decreased transiently in one patient. This report shows that a sustained elevation of erythroid progenitors induced by in vivo IL-3 administration may not translate into an increase of mature red cells production in DBA patients.
authors.affiliation: Service d'Hématologie, Centre Hospitalier Lyon-Sud, Pierre-Bénite, France.
authors.forename: Y
authors.initials: Y
authors.lastname: Bastion
authors.forename: L
authors.initials: L
authors.lastname: Campos
authors.forename: N
authors.initials: N
authors.lastname: Roubi
authors.forename: J
authors.initials: J
authors.lastname: Bienvenu
authors.forename: P
authors.initials: P
authors.lastname: Felman
authors.forename: C
authors.initials: C
authors.lastname: Dumontet
authors.forename: B
authors.initials: B
authors.lastname: Coiffier
mesh_terms: D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D004306:Dose-Response Relationship, Immunologic; D015672:Erythroid Precursor Cells / Q000502:physiology; D004921:Erythropoietin / Q000627:therapeutic use; D005199:Fanconi Anemia / Q000097:blood / Q000628:therapy*; D005260:Female; D006412:Hematopoietic Stem Cells / Q000502:physiology; D006801:Humans; D007377:Interleukin-3 / Q000627:therapeutic use*; D008875:Middle Aged; D012010:Red-Cell Aplasia, Pure / Q000097:blood / Q000628:therapy*
chemical_list: D007377:Interleukin-3; D004921:Erythropoietin