The most common haemoglobin abnormalities:
S S i i c c k k l l e e C C e e l l l l T T r r a a i i t t
This is a heterozygous state showing HbA and HbS and a normal amount of HbA
Results on citrate agar show haemoglobins in the HbA and HbS migratory positions (zones).
S S i i c c k k l l e e C C e e l l l l A A n n a a e e m m i i a a
This is a homozygous state showing almost exclusively HbS, although a small amount of HbF may also
be present.
S S i i c c k k l l e e - - C C D D i i s s e e a a s s e e
This is a heterozygous state demonstrating HbS and HbC.
S S i i c c k k l l e e C C e e I I l l - - T T h h a a l l a a s s s s a a e e m m i i a a D D i i s s e e a a s s e e
This condition shows HbA, HbF, HbS, and HbA
In Sickle Cell b o -Thalassaemia HbA is absent.
In Sickle Cell b + -Thalassaemia HbA is present in reduced quantities.
T T h h a a l l a a s s s s a a e e m m i i a a - - C C D D i i s s e e a a s s e e
This condition shows HbA, HbF and HbC.
C C D D i i s s e e a a s s e e
This is a homozygous state showing almost exclusively HbC
T T h h a a l l a a s s s s a a e e m m i i a a M M a a j j o o r r
This condition shows HbF, HbA and HbA
L L I I M M I I T T A A T T I I O O N N S S
Some abnormal hemoglobins have similar electrophorotic mohilities and must be differentiated by
other methodologies.
Further testing required:
1. Globin chain analysis (both acid and alkaline) and structural studies may be necessary in order to
positively identify some of the more rare haemoglobins.
2. Anion exchange column chromatography is the most accurate method for quantitating HbA
Helena BioSciences Sickle-Thal Quik Column Method (Cat. No. 5334) for quantitation of HbA
the presence of HbS, or the Helena BioSciences Beta-Thal HbA
(Cat. No. 5341) are recommended. HbA
tests in the diagnosis of B-thalassaemia trait.
3. Low levels of HbF (1-10%) may be accurately quantitated by radial immunodiffusion using the
Helena BioSciences HbF-QuiPlate Procedure (Cat. No. 9325).
R R E E F F E E R R E E N N C C E E V V A A L L U U E E S S
At birth, the majority of haemoglobin in the erythrocytes of the normal individual is foetal haemoglobin,
HbF. Some of the major adult haemoglobin, HbA, and a small amount of HbA
At the end of the first year of life and through adulthood, the major haemoglobin present is HbA with
up to 3.7% HbA
and less than 2% HbF.
2
.
2
.
2
quantitation is one of the most important diagnostic
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5
SAS-MX ACID HB-10
on cellulose acetate.
2
Quik Column Procedure
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, are also present.
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in
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English