1) The document provides interpretation of hematological data and hemoglobin typing results to determine different thalassemia and hemoglobinopathy conditions. 2) Key findings that can be determined include normal hemoglobin, beta-thalassemia trait, homozygous beta-thalassemia, Hb E trait, homozygous Hb E, beta-thalassemia/Hb E disease, and various alpha-thalassemia conditions. 3) Hemoglobin E levels can vary depending on whether it is a trait, homozygous Hb E, Hb E/beta-thalassemia, or coinheritance with alpha-thalassemia. Coinheritance of alpha- and

1. HbHb Typing InterpretationTyping Interpretation
Dr. Orapan Sripichai
Miss. Thongperm Munkongdee

2. Thalassemia Diagnosis
Hematological Data
CS/PS
A2/E
A
F
Bart’s
Hb Type
• Hb A2 (E)
• Hb F Quantitative
• Hb A
• Hb Bart’s
• Hb CS/PS
Qualitative
Hb Typing Data
☺ Hb ☺ MCV ☺ MCH ☺ MCHC ☺ RDW

3. CBC & Hb Typing DataCBC & Hb Typing Data
Patient Data: Name, Age, Sex, Blood transfusion, etc.
NORMAL
α-Thalassemiaβ-Thalassemia
• β-Thalassemia heterozygote
• Homozygous β-Thalassemia
• Hb E heterozygote
• Homozygous Hb E
• β-Thalassemia/Hb E disease
• Not rule out α-Thalassemia
• Hb H disease
• Hb H-CS/PS disease
• Hb Bart’s Hydrops Fetalis

4. Table 1. Hb Type, %A2(E), %F and MCV for thalassemia interpretation
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
α-thalassemia 2 trait A2A 2.9 ± 0.3 0.3 ± 0.1 80 ± 4
α-thalassemia 1 trait A2A 2.6 ± 0.3 0.6 ± 0.3 70 ± 5
β-thalassemia trait A2A 5.5 ± 1.3 1.5 ± 1.4 68 ± 7
Hb E Heterozygote EA 27.8 ± 7.5 1.2 ± 0.9 84 ± 5
Homozygous Hb E EE 90.2 ± 4.9 4.3 ± 2.7 70 ± 4
Hb H disease A2A Bart’s H 1.6 ± 1.2 0.6 ± 0.6 67 ± 7
β-thalassemia/Hb E disease EF 61.2 ± 13.6 31.1 ± 14.5 67 ± 6
EA Bart’s disease EA Bart’s 14.9 ± 1.6 2.6 ± 1.4 66 ± 6
EF Bart’s disease EF Bart’s 79.7 ± 8.7 10.2 ± 6.7 62 ± 5
Reference: Suthat Fucharoen et al. Thalassemia Workshop: From molecular biology to clinical medicine ; March 1998.

5. Normal (αα/αα, βN/βN)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
Hematological Data
Hb (g/dl) 14.5
MCV (fl) 85
MCH (pg) 31
MCHC (g/dl) 36.5
RDW (%) 12.5
Hemoglobin Typing
Hb type A2A
HbA2 (E) 3.2
HbF 0.0
HbA 86.2

6. ββ--ThalassemiaThalassemia

7. β-Thalassemia Trait (αα/αα, β0/βN)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
β-thalassemia trait A2A 5.5 ± 1.3 1.5 ± 1.4 68 ± 7

8. Homozygous β-Thalassemia (αα/αα, β0/β0)
31.3RDW (%)
26.3MCHC (g/dl)
24.2MCH (pg)
91.9MCV (fl)
4.9Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type A2F
HbA2 (E) 4.5
HbF 95.4
HbA 0.2
68 ± 795 ± 1.55.5 ± 1.5A2FHomozygous β-thalassemia
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

9. Hb E Trait (αα/αα, βE/βN)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
Hb E Heterozygote EA 27.8 ± 7.5 1.2 ± 0.9 84 ± 5
Hematological Data
Hb (g/dl) 11.2
MCV (fl) 81
MCH (pg) 24.4
MCHC (g/dl) 33.7
RDW (%) 14.9
Hemoglobin Typing
Hb type EA
HbA2 (E) 31.7
HbF 0.9
HbA 60.5

10. Homozygous Hb E (αα/αα, βE/βE)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
Homozygous Hb E EE 90.2 ± 4.9 4.3 ± 2.7 70 ± 4
Hematological Data
Hb (g/dl) 9.8
MCV (fl) 58.1
MCH (pg) 19.5
MCHC (g/dl) 33.6
RDW (%) 17.9
-HbA
2.1HbF
82.7HbA2 (E)
EEHb type
Hemoglobin Typing

11. β0-Thalassemia/Hb E Disease (αα/αα, β0/βE)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
β0-thalassemia/Hb E disease EF 61.2 ± 13.6 31.1 ± 14.5 67 ± 6

12. αα--ThalassemiaThalassemia

13. α-Thalassemia 2 Trait (-α/αα, βN/βN)
14.0RDW (%)
34.9MCHC (g/dl)
27.0MCH (pg)
77.4MCV (fl)
13.2Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type A2A
HbA2 (E) 3.5
HbF 0.0
HbA 86.8
Normal hemoglobin type;
Not-rule out α-Thalassemia 2 trait
80 ± 40.3 ± 0.12.9 ± 0.3A2Aα-thalassemia 2 trait
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

14. α-Thalassemia 1 Trait (--/αα, βN/βN)
Normal hemoglobin type;
Not-rule out α-Thalassemia 1 trait
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
α-thalassemia 1 trait A2A 2.6 ± 0.3 0.6 ± 0.3 70 ± 5

15. Hb H Disease (--/-α, βN/βN)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
Hb H disease A2A Bart’s H 1.6 ± 1.2 0.6 ± 0.6 67 ± 7

16. Hb Bart’s Hydrops Fetalis Disease (--/--, βN/βN)
Hemoglobin Typing
Hb type Hb Bart’s
HbA2 (E) -
HbF -
HbA -
Hb Bart’s 100
22.2RDW (%)
24.5MCHC (g/dl)
26.3MCH (pg)
107.4MCV (fl)
7.6Hb (g/dl)
Hematological Data
Bart’s
Hb Bart’s
--Hb Bart’sHb Bart’s hydrop fetalis
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

17. Hb CS Trait (αCSα/αα,βN/βN)
13.7RDW (%)
35.2MCHC (g/dl)
27.1MCH (pg)
77.0MCV (fl)
14.7Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type A2A
HbA2
(E) 2.3
HbF 0.4
HbA 88.1
Normal hemoglobin type;Normal hemoglobin type;
NotNot--rule outrule out αα--Thalassemia 2 traitThalassemia 2 trait
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

18. Homozygous Hb CS (αCS α/αCSα, βN/βN)
17.1RDW (%)
32.9MCHC (g/dl)
25.4MCH (pg)
77.2MCV (fl)
11.9Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type CSA2A
HbA2
(E) 2.1
HbF 0.4
HbA 85.2
Not rule-out Hb CS homozygote
Genotyping
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

19. Hb H-CS Disease (--/αCSα, βN/βN)
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85
Hb H-CS disease CSA2A Bart’s H 1.6 ± 1.2 0.6 ± 0.6 77 ± 5

20. Not rule-out β-thalassemia trait coinheritance with α-thalassemia
15.6RDW (%)
32.9MCHC (g/dl)
25MCH (pg)
76MCV (fl)
10.5Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type A2A
HbA2 (E) 4.8
HbF 1.2
HbA 83.7
68 ± 71.5 ± 1.45.5 ± 1.3A2Aβ-thalassemia trait
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

21. Homozygous β-Thalassemia with Blood Transfusion
20.8RDW (%)
31.7MCHC (g/dl)
22.3MCH (pg)
70.3MCV (fl)
8.4Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type A2FA
HbA2 (E) 3.4
HbF 7.8
HbA 75.7
Not rule-out β+/ β+-thaassemia or β+/ β0-thaassemia
68 ± 795 ± 1.55.5 ± 1.5A2FHomozygous β-thalassemia
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

22. β+-Thalassemia/Hb E Disease (αα/αα, β+/βE)
23.9RDW (%)
29.9MCHC (g/dl)
17.0MCH (pg)
56.9MCV (fl)
6.3Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type EFA
HbA2
(E) 62.2
HbF 14.8
HbA 19.9
Not rule-outβ+ -thalassemia/Hb E or
β0-thalassemia/Hb E with blood transfusion
67 ± 631.1 ± 14.561.2 ± 13.6EFβ0-thalassemia/Hb E disease
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

23. β0-Thalassemia/Hb E Disease with Blood Transfusion
27.2RDW (%)
33.1MCHC (g/dl)
21.1MCH (pg)
63.7MCV (fl)
5.7Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type EFA
HbA2
(E) 35.1
HbF 8.8
HbA 46.7
Not rule-out β0-thalassemia/Hb E
with blood transfusion
67 ± 631.1 ± 14.561.2 ± 13.6EFβ0-thalassemia/Hb E disease
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

24. Coinheritance ofCoinheritance of
αα-- andand ββ--ThalassemiaThalassemia

25. Hemoglobin E LevelsHemoglobin E Levels
• Hb E trait 25-30%
• Hb EE 85-95%
• Hb E/β-thalassemia 30-70%
• Hb E + α-thalassemia < 30%
• Hb E + α-thalassemia 2 trait 25-30%
• Hb E + α-thalassemia 1 trait 19-23%
• Hb E + Hb H (EA Bart’s disease) 13-18%

26. Not rule-out Double heterozygous Hb E
and α-thalassemia 2 Genotyping
Hemoglobin Typing
Hb type EA
HbA2 (E) 33.2
HbF 0.3
HbA 59.3
15.4RDW (%)
37.7MCHC (g/dl)
27.1MCH (pg)
71.9MCV (fl)
13.2Hb (g/dl)
Hematological Data
Hb E Trait with α-Thalassemia 2 Trait (-α/αα, βE/βN)
• Hb E trait 25-30%
• Hb E + α-thalassemia 2 trait 25-30%
• Hb E + α-thalassemia 1 trait 19-23%
• Hb E + Hb H (EA Bart’s disease) 13-18%

27. Not rule-out Double heterozygous Hb E
and α-thalassemia 1 Genotyping
Hemoglobin Typing
Hb type EA
HbA2 (E) 23
HbF 0.6
HbA 69.1
16.1RDW (%)
33.2MCHC (g/dl)
22.8MCH (pg)
68.6MCV (fl)
12.7Hb (g/dl)
Hematological Data
Hb E Trait with α-Thalassemia 1 Trait (--/αα, βE/βN)
• Hb E trait 25-30%
• Hb E + α-thalassemia 2 trait 25-30%
• Hb E + α-thalassemia 1 trait 19-23%
• Hb E + Hb H (EA Bart’s disease) 13-18%

28. EA Bart’s Disease (--/-α, βE/βN)
Hemoglobin Typing
Hb type EA Bart’s??
HbA2 (E) 16.6
HbF 1.1
HbA 74.1
17.2RDW (%)
31.7MCHC (g/dl)
14.3MCH (pg)
45.2MCV (fl)
9.0Hb (g/dl)
Hematological Data
Not rule-out EA Bart’s disease
;clinical study or genotyping
66 ± 62.6 ± 1.414.9 ± 1.6EA Bart’sEABart’s disease
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

29. CSEA Bart’s Disease (--/αCSα, βE/βN)
23.7RDW (%)
28.0MCHC (g/dl)
16.9MCH (pg)
60.3MCV (fl)
6.4Hb (g/dl)
Hematological Data
Hemoglobin Typing
Hb type CSEA Bart’s
HbA2 (E) 18.1
HbF 2.4
HbA 73.2
66 ± 62.6 ± 1.414.9 ± 1.6CSEA Bart’sCSEA Bart’s disease
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

30. EF Bart’s Disease (--/-α, βE/βE)
Not rule-out EF Bart’s disease
;clinical study or genotyping
Hemoglobin Typing
Hb type EF Bart’s
HbA2 (E) 95.5
HbF 5.1
HbA 4.1
22.4RDW (%)
26.5MCHC (g/dl)
15.1MCH (pg)
56.9MCV (fl)
6.9Hb (g/dl)
Hematological Data
62 ± 510.2 ± 6.779.7 ± 8.7EF Bart’sEF Bart’s disease
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation

31. Special CasesSpecial Cases

32. Normal Hb type with anemia;
not rule out α-thalassemia trait
Normal with Anemia
Interpretation Hb type %A2(E) %F MCV
Normal A2A 2.6 ± 0.4 0.5 ± 0.7 ≥ 85

33. HPFH Heterozygote
Hematological Data
Hb (g/dl) 10.2
MCV (fl) 76.2
MCH (pg) 35.7
MCHC (g/dl) 35.3
RDW (%) 17.4
Hemoglobin Typing
Hb type A2FA
HbA2 (E) 2.5
HbF 24.4
HbA 66.6
≥ 850.5 ± 0.72.6 ± 0.4A2ANormal
MCV%F%A2(E)Hb typeInterpretation