Treatments for sickle cell disease
This type of therapy remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion.
Blood transfusions
This type of test measures red blood cell count, white blood cell count, hemoglobin, hematocrit, mean corpuscular volume, mean corpuscular hemoglobin, mean corpuscular hemoglobin concentration, and platelet count.
Complete blood count(CBC)
This type of genetic mutation leads to SCD
Point mutation
This medication is an amino acid that reduces sickling in SCD.
L-Glutamine
This is a diagnostic test in which peripheral blood is examined under the microscope. Can be used to diagnose conditions that cause abnormal counts or morphology of red blood cells, white blood cells, and/or platelets.
Peripheral blood smear
This gene and chromosome number are mutated in individuals with SCD
β-globin gene (chromosome 11)
This is the first monoclonal antibody approved for sickle cell disease to reduce the frequency of vaso-occlusive crises.
Crizanlizumab
refers to a radiographic appearance of the diploic space of the skull vault which results from a thickening of trabeculae as the diploic space expands. These trabeculae are perpendicular in orientation, interspersed by radiolucent marrow hyperplasia along with skull vault.
hair-on-end (“crew cut”) sign
HbS polymerizes when deoxygenated, causes
what to happen to erythrocytes
deformation of erythrocytes (“sickling”)
This phase of the cell cycle is where hydroxyurea exerts its action in treating sickle cell disease
What is the S phase
This syndrome in sickle cell disease is a leading thoracic complication - as well as leading cause of mortality - in those affected by sickle cell disease.
Acute chest syndrome
Most common acute complication of sickle cell disease
Characterized by recurrent episodes of severe throbbing or sharp pain
Typically affects the limbs, chest, and back and lasts for ∼ 7 days
Vasoocclusive crises (sickle cell pain crisis)
What class of drug does Hydroxyurea belong to
Antimetabolites
This is a hemoglobin protein variant present in sickle cell disease and individuals with sickle cell trait. Composed of two alpha subunits and two mutated beta subunits. The mutations result in proteins that have an increased tendency to polymerize into chains, which then deform red blood cells.
Hemoglobin S
This is a painful swelling into the hands and/or feet. It is caused by sickle cells blocking the tiny blood vessels in the hand and feet.
Dactylitis