Sei sulla pagina 1di 14

Practical

Clinical Hematology
What is Sickle cell:
Sickle cell anemia is an inherited disorder that leads to
the production of an abnormal hemoglobin variant,
hemoglobin S (HbS)

Hemoglobin S production arises from an altered


(mutated) S gene. Differ on Hb A by one amino acid
substitution. .
Amino acid substitution in HbS
One S gene SS gene (homozygous SS)

Sickle cell trait, 20% to 40% of the Sickle cell anemia, 80% to 100% of the
hemoglobin is HbS hemoglobin is HbS

Does not have any health problems No symptoms under 'normal' conditions.

But in 'sickling crises' such as infection


or dehydration
There is some symptom like haemolytic
anaemia (jaundice)
RBCs have Hbs
In the RBC, variant Hbs can form polymers in low
oxygen conditions, changing the shape of the RBC
from a round disc to a characteristic crescent (sickle)
shape.
This altered shape limits the RBCs ability to flow
smoothly throughout the body, limits the
hemoglobins ability to transport oxygen, and
decreases the RBCs lifespan from 120 days to about 10-
20 days.
The affected person can become anemic because the
body cannot produce RBCs as fast as they are
destroyed.
Also, sickled blood cells can become trapped in blood
vessels reducing or blocking blood flow. This can
damage organs, muscles, and bones and may lead to
life-threatening conditions.
Sickle cell test:
A sickle cell test is a blood test done to screen for sickle
cell trait or sickle cell disease.
There are three method
1. A saline citrate with paraffin oil.
2. Sodium Metabisulfite Method.
3. Solubility Test.
General Principle
We will make the conditions at which oxygen tension
decline to induced the sickling process of Hbs in RBCs.
A saline citrate with paraffin oil
Principle:
a saline citrate suspension of blood is allowed to stand
in a test tube under a layer of paraffin oil until sickling
takes place.
In employing any of the common diagnostic tests for
sickling it is desirable to obtain blood which has a low.
fraction of oxyhemoglobin.
Sodium Metabisulfite Method
Principle
When a drop of blood is sealed between a cover
slip and a slide, the decline in oxygen tension
due to oxidative processes in the blood cells
leads to sickling.
In this method added with blood drop a
chemical reducing agents. Such as sodium
metabisulfite. This rapidly reduces
oxyhemoglobin to reduced hemoglobin, then
this will be accelerate sickling .
Solubility test
Principle
Erythrocytes are lysed by saponin and the released
hemoglobin is reduced by sodium hydrosulfite in a
concentrated phosphate buffer.
Under these conditions, reduced HbS is characterized
by its very low solubility and the formation of crystals.
Principle
The presences of HbS or HbC are indicated by the
turbid solutions. The normal HbA under these same
conditions results in a clear non-turbid solutions.

Potrebbero piacerti anche