(M) Week 9: Special test for assessing certain hemoglobin disorders Flashcards
screening test to evaluate the presence of Hgb S in the patient’s blood
Dithionite solubility test
an unstable hemoglobin where point mutation of a single amino acid occurs in the beta globin chain
Hgb S
Hgb S is a genetic defect that causes the substitution of glutamic acid with _________ on the ___ position of amino acid in either one or two beta globin chains of the hemoglobin
valine on the 6th position
Hgb S causes the hemoglobin to ___________ within the red cells that changes the appearance of RBCs from disc to __________
precipitate
sickle shaped
Shape change to sickle may be triggered by the following factors:
hypoxia
acidosis
dehydration
What are the three forms of hemoglobin defect
sickle cell anemia = Hgb SS
sickle cell trait = Hgb AS
sickle cell disease = Hgb SC, SG, SD, SE, S-Beta thalassemia
which test principle is this:
The red cells are placed in a solution containing saponin and sodium dithionite reagent.
dithionite solutbility test
What is the action of the saponin reagent in the dithionite solubility test?
lyses the red cell, liberating the hemoglobin
What is the action of the sodium dithionite reagent if Hgb S is presnet
Hgb S is oxidized and forms tactoids / precipitates
Which diseases cause a positive result in the dithionite solubility test
Hgb SS / Hgb AS
What is the positive test result for dithionite solubility test
precipitates that causes turbidity
Which diseases causes a negative result on the dithionite solubility test?
sickle cell diseases
Used as a screening test for Hgb S
sickling test / sodium metabisulfite
+ dithionite solubility test
What shape does RBCs turb into when there is a presence of Hgb SS or As after being incubated with sodium metabisulfite
holly leaf appearance
a technique used to identify the specific type of hemoglobin present on an individual’s blood
Hgb electrophoresis
What blood disorders do Hgb electrophoresis confirm?
thalassemia and other hemmoglobinopathy
What are the two types of Hgb electrophoresis
cellulose acetate Hgb electrphoresis
citrate Hgb electrophoresis
what is the pH environment of cellulose acetate Hgb electrophoresis
8.0
what is the pH environment of citrate Hgb electrophoresis
6.0-6.2
What are the Hgb standards used for Hgb electrophoresis
Hgb A1
Hgb F
Hgb S
Hgb C
what is the order of migration in cellulose acetate Hgb electrophoresis (fastest to slowest)
Hgb A1
Hgb F
Hgb S
Hgb C
to which direction do the Hgb standards in cellulose acetate Hgb electrophoresis migrate to?
toward the anode (positive charge)
what is the order of migration in citrate Hgb electrophoresis (fastest to slowest)
Hgb C
Hgb S
Hgb F
Hgb A1