Hemoglobinopathies

Hb Distributions of some common hemoglobinopathies

 
A1
A2
F
S
C
(a2b2)
(a2d2)
(a2g2)
(a2b2) 6glu-val
(a2b2) 6glu-lys
Normal
96 - 98%
1.4 - 4%
-
-
-
S trait
60 - 75%
-
-
25 - 45%
-
SS
-
-
sm
>95%
-
S - HPFH
-
-
20-30%
70 - 80%
-
S/b thal
0 - 15%
4 - 5%
> 20%
60 - 80%
-
SC
-
-
-
45 - 55%
45 - 55%
b thal minor
92 - 96%
4 - 8%
-
-
-
a/b thal with F
80 - 90%
> 3.5%
5 - 15%
-
-
b thal major
0 -20%
5 - 8%
70 - 90%
-
-
db thal trait
80 - 90%
1.4 - 4%
5 - 20%
-
-
HPFH het
65 - 85%
-
15 - 35%
-
-
C-b thal
0 -15%
-
2 -10%
-
60 - 90%

Cellulose Acetate (pH 8.6) Alkaline
-
C
S
F
A
+
applic
CA, CS
A2
D, G
H, I
N, B
J
E, O
Lepore
CA = carbonic anhydrase, CS = Constant Spring, B = Barts

Citrate Agar (pH 6.2) Acid
+
C
S
A
F
-
O
applic
A2
 
   
D, G, E, Lepore, J

EXAMPLES:

Cellulose Acetate (pH 8.6) Alkaline
-
C/A2
S
F
A
+
Normal*
I
I
I
Hb H disease
I
I
I
II
Hb C trait
I
I
I
Hb S/b thal or transfused S
I
I
I
I
b thal minor
I
I
I
I
Homozygous b+ thal or b0 thal transfused
I
I
I
I
Newborn
I
I
S trait neonate
I
I
I
S trait/A'2 trait
II
I
I
I
Heredit spherocytosis or myelofibrosis
I
I
I
I
S trait neonate, mishandled, with methemoglobins
II
II
II
HbE/iron def or HbE/a thal
I
I
I
HbS trait, HbD or G trait
I
I
I
I
HbG-Philadelphia trait
I
I
I
I
Hb S disease or HbS/HbD
I
I
I
Hb Lepore
I
I
I
I
HbS/HbG-Phil or HbSC transfused with normal blood
I
I
I
I
* also see in Hb Mamlo (erythrocytosis), b thal minor or Hb H with iron deficiency