Higher fetal hemoglobin concentration in patients with sickle cell disease in eastern India reduces frequency of painful crisis

The XmnI polymorphic site 5’ to the gene Gγ in a Brazilian patient with sickle cell anaemia – fetal haemoglobin concentration, haematology and clinical features

“…Powars and Hiti [9] and Nagel and Steinberg [10] showed that high levels of HbF were associated with milder SCA symptoms. They also found that a higher expression of HbF in adulthood improves the rates of morbidity and mortality in SCA patients [11]. These results support our observations, in which the patient has a high level of HbF and a milder phenotype, therefore requiring fewer blood transfusions.…”

Fetal hemoglobin and alpha thalassemia modulate the phenotypic expression of HbSD‐Punjab

“…In our study, there was a negative association between HbF concentration and risk of VOC in the HbSD group. We had earlier reported the protective effect of HbF in reducing the frequency of VOC in HbSS cases [17]. However, the association between HbF and VOC is stronger (P = 0.005) in HbSD cases.…”

Low and fixed dose of hydroxyurea is effective and safe in patients with HbSβ+ thalassemia with IVS1‐5(G→C) mutation

“…However, there were several reasons for using HU at a dose of 10 mg/kg/day. (1) The b Sgene in SCD in this region is linked to Asian-Indian haplotype with high mean baseline %HbF (22.3 AE 6.9%) [43]. (2) Recently we observed that HU at a dose of 10 mg/kg/day was effective and safe in patients with SCA and HbSD-Punjab [13,14].…”