To obtain information on the cellular mechanism of induction of fetal hemoglobin (HbF) by sodium butyrate (NaB), we treated adult baboons with NaB and assessed its effects on HbF expression. Infusion of NaB increased F reticulocytes and F-positive CFUe and e-cluster colonies without induction of reticulocytosis or increase in progenitor cell numbers. Addition of NaB in bone marrow cultures incr...

Ketones can reactivate the production of fetal hemoglobin (HbF) in vitro and in vivo. A reactivation of HbF by ketones, which are generated during starvation, remains largely speculative. Therefore, we investigated HbF in 31 women with anorexia nervosa or bulimia, using both of these as models of intermittent starvation ketosis. For comparison, we also studied 42 female control subjects matched...

Sickle transgenic mice expressing exclusively human globins are desirable for studying pathophysiology and testing gene therapy strategies, but they must have significant pathology and show evidence of amelioration by antisickling hemoglobins. Mice were generated that expressed exclusively human sickle hemoglobin with 3 levels of HbF using their previously described sickle constructs (cointegra...

potent induction of fetal hemoglobin (hbf) production results in alleviating the complications of β-thalassemia and sickle cell disease (scd). hbf inducer agents can trigger several molecular signaling pathways critical for erythropoiesis. janus kinase/signal transducer and activator of transcription (jak/stat), mitogen activated protein kinas (mapk) and phosphoinositide 3-kinase (pi3k) are con...

To the Editor: remains const t and seems to be under genetic control.' Available data suggest that at least two types of genetic determinants might In normal adults. synthesis of fetal hemoglobin (HbF) persists at be involved in the level of HbF expression: one linked to the pa very low level ( < I % ) and is restricted to a subpopulation of globin gene cluste?.' and the other(s) unlinke...

Genetic studies identify common variants within the HBS1L-MYB intergenic region (HMIP), BCL11A, and Xmn1-HBG2 as associated with elevated fetal hemoglobin (HbF) levels other clinically important human hematological traits. Recent suggest HbF is a predictor of outcome in MDS/AML patients receiving decitabine. We assessed effects genetic on traits Myeloproliferative Neoplasm (MPN), Myelodysplasti...

Hydroxyurea (HU) can increase fetal hemoglobin (HbF) in kg. All four quartiles had substantial increases of F cells in sickle cell anemia (HbSS). To identify determinants of the the first year. This was maintained for 2 years only in the top HbF response, we studied 150 HU-treated patients grouped three quartiles. Leukocyte and reticulocyte counts decreased by quartiles of change in HbF from ba...

Hydroxyurea (HU) can increase fetal hemoglobin (HbF) in sickle cell anemia (HbSS). To identify determinants of the HbF response, we studied 150 HU-treated patients grouped by quartiles of change in HbF from baseline to 2 years. Half of the HU-assigned patients had long-term increments in HbF. In the top two quartiles, HbF increased to 18.1% and 8.8%. These patients had the highest baseline neut...

We have analyzed the synthesis of fetal hemoglobin (HbF) in the differentiated erythroid colonies produced by erythropoietin-responsive committed erythroid progenitors in plasma cultures of human marrow and peripheral blood. Two independent biochemical techniques. carboxymethyl-cellulose column chromatography of 3H-leucine-Iabeled globin. and polyacrylamide gel electrophoresis of 59Fe-labeled h...

The clinical efficacy of hydroxyurea (HU) in the treatment of sickle cell anemia has mainly been attributed to increased levels of fetal hemoglobin (HbF), which reduces the tendency for sickle hemoglobin to polymerize, thereby reducing the frequency of the vaso-occlusive phenomena associated with the disease. However, benefits from HU treatment in patients have been reported in advance of incre...