Table 1.

Hematologic data in various hemoglobin E syndromes10 (modified).

Hb, g/dLMCV, fLMCH, pgMCHC, g/dLRDW, %Alkali denaturation test (Hb F),%Hb typing
Abbreviations: CS, Hb Constant Spring; D, decreased; Hb, hemoglobin; MCH, mean corpuscular hemoglobin; MCHC, mean corpuscular hemoglobin concentration; MCV, mean corpuscular volume; N, normal; ND, not determined; RDW, red cell distribution width 
Normal M15.9 ± 0.9
 F12.5 ± 2.0 87 ± 6 31 ± 1.1 33 ± 0.9 13.1 ± 0.8 0.5 ± 0.2 A2 (2.5 ± 0.2) + A 
Hb E trait 12.8 ± 1.5 84 ± 5 30 ± 2.4 33 ± 1.8 14.1 ± 0.6 0.9 ± 0.7 E (29.4 ± 2.3%) + A 
Hb E α-thalassemia 2 13.1 ± 1.4 88 ± 4 ND ND ND  E (28.5 ± 1.5%) + A 
Hb E α-thalassemia 1 12.5 ± 1.4 77 ± 5 23 ± 1.1 32 ± 1.6 ND 0.9 ± 0.4 E (20.7 ± 1.2%) + A 
Homozygous HbE 11.4 ± 1.8 70 ± 4 22 ± 1.9 33 ± 1.7 15.6 1.8 ± 1.4 E (87.7 ± 5.9%) 
Hb E/β0-thalassemia 7.8 ± 2.6 67 ± 6 19 ± 3.6 28 ± 4.8 26.5 ± 5.6 42 ± 1.5 E (58 ± 1.5%) + F 
AE Bart’s disease       E (13.0 ± 2.1%) + A
 + Bart’s (2.2 ± 1.8%) 
    α-thalassemia 1/α-thalassemia 2-Hb E 9.1 ± 1.1 60 ± 3 17 ± 2 31 ± 4 ND 2.0 ± 0.7  
    α-thalassemia 1/Hb CS-Hb E 8.0 ± 0.9 67 ± 4 19 ± 2 29 ± 2 ND 2.3 ± 1.4 CS (1.1 ± 0.4%) + E (13.9 ± 1.8%) + A +
 Bart’s (3.9 ± 1.5%) 
Hb EE + Hb H 8.0 ± 1.3 63 ± 6 18 ± 2 29 ± 2 ND 5.8 ± 3.7 E (80%) + F + Bart’s(5%) or CS (1.9 ± 0.9%)
 + E (86.4 ± 8%) + F
 + Bart’s (3.7 ± 1.9%) 
Hb SE12 11.2 ± 1.8 75 ± 10 ND ND ND ND Hb S (62.8% ± 7.4%), Hb E (33.3% ± 3.6%), Hb F (range 1 – 5.2) 
Hb, g/dLMCV, fLMCH, pgMCHC, g/dLRDW, %Alkali denaturation test (Hb F),%Hb typing
Abbreviations: CS, Hb Constant Spring; D, decreased; Hb, hemoglobin; MCH, mean corpuscular hemoglobin; MCHC, mean corpuscular hemoglobin concentration; MCV, mean corpuscular volume; N, normal; ND, not determined; RDW, red cell distribution width 
Normal M15.9 ± 0.9
 F12.5 ± 2.0 87 ± 6 31 ± 1.1 33 ± 0.9 13.1 ± 0.8 0.5 ± 0.2 A2 (2.5 ± 0.2) + A 
Hb E trait 12.8 ± 1.5 84 ± 5 30 ± 2.4 33 ± 1.8 14.1 ± 0.6 0.9 ± 0.7 E (29.4 ± 2.3%) + A 
Hb E α-thalassemia 2 13.1 ± 1.4 88 ± 4 ND ND ND  E (28.5 ± 1.5%) + A 
Hb E α-thalassemia 1 12.5 ± 1.4 77 ± 5 23 ± 1.1 32 ± 1.6 ND 0.9 ± 0.4 E (20.7 ± 1.2%) + A 
Homozygous HbE 11.4 ± 1.8 70 ± 4 22 ± 1.9 33 ± 1.7 15.6 1.8 ± 1.4 E (87.7 ± 5.9%) 
Hb E/β0-thalassemia 7.8 ± 2.6 67 ± 6 19 ± 3.6 28 ± 4.8 26.5 ± 5.6 42 ± 1.5 E (58 ± 1.5%) + F 
AE Bart’s disease       E (13.0 ± 2.1%) + A
 + Bart’s (2.2 ± 1.8%) 
    α-thalassemia 1/α-thalassemia 2-Hb E 9.1 ± 1.1 60 ± 3 17 ± 2 31 ± 4 ND 2.0 ± 0.7  
    α-thalassemia 1/Hb CS-Hb E 8.0 ± 0.9 67 ± 4 19 ± 2 29 ± 2 ND 2.3 ± 1.4 CS (1.1 ± 0.4%) + E (13.9 ± 1.8%) + A +
 Bart’s (3.9 ± 1.5%) 
Hb EE + Hb H 8.0 ± 1.3 63 ± 6 18 ± 2 29 ± 2 ND 5.8 ± 3.7 E (80%) + F + Bart’s(5%) or CS (1.9 ± 0.9%)
 + E (86.4 ± 8%) + F
 + Bart’s (3.7 ± 1.9%) 
Hb SE12 11.2 ± 1.8 75 ± 10 ND ND ND ND Hb S (62.8% ± 7.4%), Hb E (33.3% ± 3.6%), Hb F (range 1 – 5.2) 

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