Hemoglobin F mitigation of sickle cell complications decreases with aging
نویسندگان
چکیده
منابع مشابه
Level of Hemoglobin F and Gg Gene Expression in Sickle Cell Disease and Their Association with Haplotype and XmnI Polymorphic Site in South of Iran
Background: Molecular genetic factors regulating hemoglobin F (Hb F) expression are important modifiers of the severity of sickle cell anemia (SS). Methods: The prevalence of XmnI polymorphic site, the Gg:Ag ratio and the Hb F level were determined using PCR-RFLP procedure, HPLC and alkaline denaturation method, respectively, in various haplotypes of 52 patients with SS, 18 patients with sickle...
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Patients with sickle cell anemia were treated with daily doses of hydroxyurea, to assess pharmacokinetics, toxicity, and increase in fetal hemoglobin (Hb) production in response to the drug. Plasma hydroxyurea clearances were not a useful guide to maximum tolerated doses of the drug. The mean daily single oral dose that could be maintained for at least 16 weeks was 21 mg/kg (range, 10 to 35 mg/...
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Red cell survival was measured in ten subjects with S-C disease and one with S-O Arab (alpha 2 beta 2-121 glu yields lys) disease using both DF32p and 51Cr as tags. Red cell volume was slightly reduced in most patients (87% plus or minus 20% of predicted normal). In nine SC patients, mean red cell life (DF32p) was 28.9 plus or minus 4.0 days. For one SC subject it was significantly longer (47.9...
متن کاملHydroxyurea induction of hemoglobin F production in sickle cell disease: relationship between cytotoxicity and F cell production.
Initial alterations in fetal hemoglobin (HbF) production among eight sickle cell anemia subjects treated with hydroxyurea (Hu) are summarized. Four of these subjects had been previously treated with 5-azacytidine (5-aza). All subjects treated with Hu (50 mg/kg/d for three to five days) had suppression of their total reticulocyte counts by seven days, whereas the four subjects previously treated...
متن کاملFetal Hemoglobin and F-Cell Responses to Long-Term Hydroxyurea Treatment in Young Sickle Cell Patients
We have studied the cellular and molecular responses to long-term hydroxyurea (HU) treatment in 29 severely affected young patients with sickle cell disease (mean age, 10.9 6 4.1 years). Patients received HU at 20 mg/kg/d on 4 consecutive days per week initially, with a monthly escalated dose avoiding marrow-toxicity (mean steady-state dose, 34.2 6 4.6 mg/kg/d) for 12 to 36 months (mean duratio...
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ژورنال
عنوان ژورنال: American Journal of Hematology
سال: 2020
ISSN: 0361-8609,1096-8652
DOI: 10.1002/ajh.25759