Effects of 5-aza-2'-deoxycytidine on fetal hemoglobin levels, red cell adhesion, and hematopoietic differentiation in patients with sickle cell disease.

Fetal hemoglobin (HbF) decreases polymerization of sickle hemoglobin (HbS) and improves outcomes in sickle cell disease (SSD). Therefore, a therapeutic goal in SSD is pharmacologic reactivation of HbF. Silencing of the gamma-globin (HbF) gene is associated with DNA methylation.

Maintenance of elevated fetal hemoglobin levels by decitabine during dose interval treatment of sickle cell anemia.

We have previously demonstrated that 5-aza-2'-deoxycytidine (decitabine) augments fetal hemoglobin (HbF) levels in patients with sickle cell anemia (SS) who did not respond to hydroxyurea (HU). The present study was designed to determine the effect of repeated decitabine dosing on HbF levels and hematologic toxicity over a 9-month treatment period. Seven patients (5 HU nonresponders) were entered.

A natural history study of adolescents and young adults with sickle cell disease as they transfer to adult care: a need for case management services.

Life expectancy for adolescents with SCD now extends well into adulthood. As a result, adolescents transfer to adult care. Little empirical evidence exists to show how transfer occurs and how well the current practices now work.

Evaluation and Management of Sickle Cell Disease in the Emergency Department (An 18-year Experience): 1974--1992.

Painful episodes are the most frequent complaints of patients with sickle cell disease. The Emergency Department (ED) has provided management for acute events using the usual triage format for emergencies. A prospective study evaluated the role of the ED in the care of adults with sickle cell disease (SCD).