期刊
SAUDI JOURNAL OF MEDICINE & MEDICAL SCIENCES
卷 6, 期 3, 页码 133-136出版社
WOLTERS KLUWER MEDKNOW PUBLICATIONS
DOI: 10.4103/sjmms.sjmms_107_18
关键词
Fetal hemoglobin; sickle cell disease; sickle hemoglobin
Sickle hemoglobin (alpha(2)ss(S)(2)) polymerization drives disease pathophysiology in sickle cell anemia. Fetal hemoglobin (alpha(2)gamma(2)) restricts disease severity by inhibiting the polymerization of sickle hemoglobin in a concentration-dependent manner. Clinical decision-making relies on diagnostic technologies evaluating fetal hemoglobin as mean percent or mean quantity in blood. Limitation of this approach is exemplified by patients with significant high fetal hemoglobin levels and severe disease, suggesting that fetal hemoglobin is unevenly distributed across F-cells. Therefore, determination of fetal hemoglobin/F-cell would provide a new paradigm for ascertaining prognosis and response to fetal hemoglobin-inducing agents. Measurement of fetal hemoglobin/F-cell, ultimately adapted to widespread standardized analytical use, is a promising fetal hemoglobin-related prognostic approach to monitor the severity of sickle cell disease and the best phenotype to follow when developing new candidate fetal hemoglobin inducers or titrating hydroxyurea in treated sickle cell patients.
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