Decision to start hydroxyurea in SCD

Baseline testing is required before starting hydroxyurea, and monitoring (eg, complete blood count, reticulocyte count, percent HbF, creatinine) continues throughout administration, with more frequent monitoring during dose escalation. Patients with SCD should receive comprehensive care from a clinician with experience managing hemoglobinopathies. The primary clinician may be aware of other aspects of the patient's clinical condition or the patient's or parents' values and preferences that may impact decision-making. Refer to UpToDate topics on SCD management and the use of hydroxyurea in SCD for further information.

SCD: sickle cell disease, which includes all homozygous (HbSS) or compound heterozygous genotypes; HU: hydroxyurea.
* Symptomatic disease generally refers to more than one painful episode or episode of dactylitis requiring hospitalization in a one-year period or more than one episode of acute chest syndrome in a two-year period. Different criteria may be needed for some individuals, such as a single clinically significant episode of dactylitis, hepatic sequestration, or splenic sequestration in an infant; chronic pain; severe, symptomatic anemia due to SCD in an adult; or repeated vaso-occlusive events at lower frequency than that used in trials for any age group.
¶ Genotype is usually inferred from hemoglobin analysis rather than DNA testing. Common genotypes are shown. Individuals with HbSS or sickle β⁰ thalassemia are most likely to develop symptomatic disease if untreated. Those with HbSC or HbS/β⁺ thalassemia are least likely to develop severe disease. Decisions for other genotypes are individualized.

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Decision to start hydroxyurea in SCD