sickle cell Flashcards
(6 cards)
L-glutamine
Endari
L-glutamine is an twice daily oral medication that was shown to decrease frequency of pain.
Anti oxidant.
It increases NAD redox potential in sickle red blood cells through increasing the availability of reduced glutathion
Voxelotor
Oxbryta
as approved in 2019 for patients aged 12 years and older and then expanded to ages 4 years and older in 2021 because, in trials, patients taking voxelotor experienced a 1 g/dL rise in hemoglobin.
Inhibit hbS polymerization by binding to HbS and increasing oxygen affinity —> less sickling
Crizanlizumab
Adakveo
IV monthly. after a loading period that was approved in 2019, as well, for patients 16 years and older with sickle cell disease to prevent pain.
blocking the interaction between P-selectin and its ligand, P-selectin glycoprotein ligand-1 (PSGL-1).
P-Selectin and Vaso-Occlusive Crises
P-selectin is an adhesion molecule expressed on the surface of activated platelets and endothelial cells. It plays a crucial role in the inflammatory process and in the development of VOCs
TCDi vs non imaging TCD
It is important to know that TCDi provides lower-velocity measurements than non-imaging TCD (in the same vessel in the same person)
on average, one needs to subtract 15 cm/s to a TCDi velocity to convert it to a comparable non-imaging reference range.
The STOP criteria cutoffs (abnormal is 200 cm/s or greater) are based on non-imaging TCD.
The patient should also be at their baseline (not in a vaso-occlusive episode) or recently transfused.
Anamnestic response
In delayed hemolytic transfusion reaction.
The patient had formed an RBC alloantibody after at least one of the previous transfusions.
The titer of the alloantibody waned over the years and was undetectable by initial RBC screen (indirect antiglobulin test).
The reappearance of the antibody with antigen stimulation (exposure) is called an anamnestic response, which can occur 3 to 14 days after reexposure to the antigen
Hydroxyurea
