Diamond-blackfan anemia: in vitro response of erythroid progenitors to the ligand for c-kit
| Autor: | J L, Abkowitz, K M, Sabo, B, Nakamoto, C A, Blau, F H, Martin, K M, Zsebo, T, Papayannopoulou |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
Adult
Erythroid Precursor Cells Male Stem Cell Factor Adolescent Immunology Granulocyte-Macrophage Colony-Stimulating Factor Cell Biology Hematology Hematopoietic Cell Growth Factors Biochemistry Culture Media Colony-Forming Units Assay Fanconi Anemia Bone Marrow Child Preschool hemic and lymphatic diseases Humans Female Interleukin-3 Lymphocytes Erythropoietin Cells Cultured |
| Zdroj: | Blood. 78:2198-2202 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood.v78.9.2198.bloodjournal7892198 |
| Popis: | To provide insights into the pathogenesis of Diamond-Blackfan anemia, we examined the in vitro response of erythroid progenitors to the recently isolated ligand for c-kit (stem cell factor, SCF). For these studies, marrow or blood mononuclear cells from 10 Diamond-Blackfan patients were cultured with erythropoietin (Ep), Ep and interleukin-3, Ep and granulocyte-macrophage colony-stimulating factor, or Ep and lymphocyte conditioned media (LCM). These combinations were tested in the presence or absence of SCF. The mean number of cells per erythroid burst increased 5 to 50-fold in cultures containing SCF. Furthermore, many additional erythroid bursts were seen (mean increment 3.2 x baseline values). Although burst-forming unit-erythroid (BFU-E) from all patients responded, there were differences among individuals in the sensitivity of their BFU-E to SCF. In six patients and all control studies, plateau frequencies of erythroid bursts were achieved with less than or equal to 10 ng/mL SCF, whereas in studies from the other four patients, over 50 ng/mL SCF was required. These data invite speculation that the c-kit receptor/ligand axis is involved in the pathogenesis of Diamond-Blackfan anemia. More importantly and regardless of whether the observed patterns of response reflect the primary defect or an epiphenomenon, our data strongly support a therapeutic trial of SCF in patients with Diamond-Blackfan anemia. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|