J Hematol

Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc

Journal website http://www.thejh.org

Original Article

Volume 5, Number 4, December 2016, pages 123-128

Beta-Globin Haplotypes and Alpha-Thalassemia 3.7 kb Deletion in Sickle Cell Disease Patients From the Occidental Brazilian Amazon

Figure 1. Hematologic parameters between haplotypes CAR/CAR, CAR/Ben and Ben/Ben linked to globin β^S gene of patients with sickle cell disease from Manaus, Amazon, Brazil.

**Table 1.** Hematologic and Biochemical Parameters of Patients With Hemoglobin SS and SC From Manaus, Amazon, Brazil
Hematological and biochemical parameters	HbSS DP	HbSC DP	P-value
HbF: fetal hemoglobin; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration; RDW: red blood cell distribution width; GGT: gamma-glutamyl transferase; LDH: lactate dehydrogenase activity.
HbF (%)	9.92 ± 6.39	3.42 ± 2.74	< 0.001
RBCs (× 10⁶/mm³)	2.90 ± 0.69	4.13 ± 0.62	< 0.001
Hemoglobin (g/dL)	8.52 ± 1.49	11.01 ± 1.93	< 0.001
Hematocrit (%)	25.62 ± 4.58	31.27 ± 4.83	< 0.001
MCV (fL)	89.59 ± 11.46	73.94 ± 11.43	< 0.001
MCH (pg)	29.97 ± 4.30	26.60 ± 4.06	0.002
MCHC (g/dL)	33.36 ± 1.94	35.15 ± 1.55	< 0.001
Reticulocytes (%)	3.26 ± 2.69	3.02 ± 1.90	0.892
RDW (%)	19.82 ± 2.55	17.20 ± 1.97	< 0.001
Leukocyte count (× 10⁹/L)	11,964.99 ± 4,318.96	8,997.77 ± 2,562.61	0.005
Platelet count (× 10⁹/L)	454.56 ± 179.14	322.00 ± 126.46	0.002
Urea (mg/dL)	20.55 ± 9.05	22.92 ± 7.28	0.366
Creatinine (mg/dL)	0.61 ± 0.17	0.80 ± 0.17	< 0.001
GGT (U/L)	31.75 ± 18.83	31.22 ± 18.43	0.937
Direct bilirubin (mg/dL)	0.90 ± 0.44	0.74 ± 0.26	0.238
Indirect bilirubin (mg/dL)	2.27 ± 1.57	1.17 ± 0.84	0.030
Glucose (mg/dL)	84.47 ± 10.02	83.92 ± 7.05	0.835
Triglycerides (mg/dL)	88.30 ± 42.25	68.87 ± 23.77	0.226
HDL cholesterol (mg/dL)	41.20 ± 13.00	52.85 ± 10.44	0.038
LDH (U/L)	521.17 ± 205.79	289.55 ± 129.18	0.001
Iron (μg/dL)	97.65 ± 49.70	69.55 ± 19.99	0.098
Ferritin (ng/mL)	925.13 ± 1415.76	241.95 ± 227.56	0.098

Table 1. Hematologic and Biochemical Parameters of Patients With Hemoglobin SS and SC From Manaus, Amazon, Brazil

Hematological and biochemical parameters

HbSS
DP

HbSC
DP

P-value

HbF: fetal hemoglobin; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration; RDW: red blood cell distribution width; GGT: gamma-glutamyl transferase; LDH: lactate dehydrogenase activity.

HbF (%)

9.92 ± 6.39

3.42 ± 2.74

< 0.001

RBCs (× 10⁶/mm³)

2.90 ± 0.69

4.13 ± 0.62

< 0.001

Hemoglobin (g/dL)

8.52 ± 1.49

11.01 ± 1.93

< 0.001

Hematocrit (%)

25.62 ± 4.58

31.27 ± 4.83

< 0.001

MCV (fL)

89.59 ± 11.46

73.94 ± 11.43

< 0.001

MCH (pg)

29.97 ± 4.30

26.60 ± 4.06

0.002

MCHC (g/dL)

33.36 ± 1.94

35.15 ± 1.55

< 0.001

Reticulocytes (%)

3.26 ± 2.69

3.02 ± 1.90

0.892

RDW (%)

19.82 ± 2.55

17.20 ± 1.97

< 0.001

Leukocyte count (× 10⁹/L)

11,964.99 ± 4,318.96

8,997.77 ± 2,562.61

0.005

Platelet count (× 10⁹/L)

454.56 ± 179.14

322.00 ± 126.46

0.002

Urea (mg/dL)

20.55 ± 9.05

22.92 ± 7.28

0.366

Creatinine (mg/dL)

0.61 ± 0.17

0.80 ± 0.17

< 0.001

GGT (U/L)

31.75 ± 18.83

31.22 ± 18.43

0.937

Direct bilirubin (mg/dL)

0.90 ± 0.44

0.74 ± 0.26

0.238

Indirect bilirubin (mg/dL)

2.27 ± 1.57

1.17 ± 0.84

0.030

Glucose (mg/dL)

84.47 ± 10.02

83.92 ± 7.05

0.835

Triglycerides (mg/dL)

88.30 ± 42.25

68.87 ± 23.77

0.226

HDL cholesterol (mg/dL)

41.20 ± 13.00

52.85 ± 10.44

0.038

LDH (U/L)

521.17 ± 205.79

289.55 ± 129.18

0.001

Iron (μg/dL)

97.65 ± 49.70

69.55 ± 19.99

0.098

Ferritin (ng/mL)

925.13 ± 1415.76

241.95 ± 227.56

0.098

**Table 2.** Hematologic Parameters Between Haplotypes Linked to Globin β^S Gene of Patients With Sickle Cell Disease From Manaus, Amazon, Brazil
Hematological parameters	CAR/CAR (N = 73; 52.5%)	CAR/Benin (N = 33; 23.7%)	CAR/Senegal (N = 4; 2.9%)	CAR/Cameroon (N = 2; 1.4%)	Benin/Benin (N = 25; 18%)	Benin/Senegal (N = 2; 1.4%)	P-value
RBCs: red blood cell; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; RDW: red blood cell distribution width; HbF: fetal hemoglobin.
RBCs (× 10⁶/mm³)	2.76 ± 0.52	2.71 ± 0.63	3.47 ± 0.75	3.12 ± 0.13	3.04 ± 0.78	2.88 ± 0.28	0.027
Hemoglobin (g/dL)	8.18 ± 1.23	8.37 ± 1.38	9.52 ± 1.43	9.20 ± 1.31	8.60 ± 0.99	8.65 ± 2.19	0.127
Hematocrit (%)	24.56 ± 3.73	25.16 ± 4.47	28.84 ± 3.65	27.26 ± 3.81	26.10 ± 4.02	29.70 ± 3.56	0.046
MCV (fL)	90.0 ± 8.88	94.0 ± 13.45	85.4 ± 15.94	87.10 ± 9.90	87.80 ± 12.56	84.4 ± 20.36	0.196
MCH (pg)	30.07 ± 3.27	31.57 ± 5.10	28.06 ± 5.38	29.43 ± 4.82	29.20 ± 4.82	28.70 ± 9.19	0.166
Reticulocytes (%)	3.37 ± 4.41	3.76 ± 7.09	2.86 ± 3.62	1.3 ± 2.90	3.52 ± 4.09	-	0.845
RDW (%)	20.12 ± 2.21	20.03 ± 2.64	20.70 ± 2.78	16.43 ± 2.10	19.83 ± 2.38	19.25 ± 1.76	0.171
Leukocytes (× 10⁹/L)	12.01 ± 3.35	13.69 ± 5.08	7.37 ± 5.97	12.65 ± 6.01	10.35 ± 3.56	10.79 ± 3.68	0.002
HbF (%)	5.92 ± 3.05	11.14 ± 3.92	19.44 ± 9.63	17.23 ± 2.87	20.97 ± 2.75	17.80 ± 1.83	< 0.001
Platelet (× 10⁹/L)	496 ± 176	455 ± 148	315 ± 256	420 ± 112	397 ± 194	470 ± 260	0.048

Table 2. Hematologic Parameters Between Haplotypes Linked to Globin β^S Gene of Patients With Sickle Cell Disease From Manaus, Amazon, Brazil

Hematological parameters

CAR/CAR (N = 73; 52.5%)

CAR/Benin (N = 33; 23.7%)

CAR/Senegal
(N = 4; 2.9%)

CAR/Cameroon (N = 2; 1.4%)

Benin/Benin (N = 25; 18%)

Benin/Senegal (N = 2; 1.4%)

P-value

RBCs: red blood cell; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; RDW: red blood cell distribution width; HbF: fetal hemoglobin.

RBCs (× 10⁶/mm³)

2.76 ± 0.52

2.71 ± 0.63

3.47 ± 0.75

3.12 ± 0.13

3.04 ± 0.78

2.88 ± 0.28

0.027

Hemoglobin (g/dL)

8.18 ± 1.23

8.37 ± 1.38

9.52 ± 1.43

9.20 ± 1.31

8.60 ± 0.99

8.65 ± 2.19

0.127

Hematocrit (%)

24.56 ± 3.73

25.16 ± 4.47

28.84 ± 3.65

27.26 ± 3.81

26.10 ± 4.02

29.70 ± 3.56

0.046

MCV (fL)

90.0 ± 8.88

94.0 ± 13.45

85.4 ± 15.94

87.10 ± 9.90

87.80 ± 12.56

84.4 ± 20.36

0.196

MCH (pg)

30.07 ± 3.27

31.57 ± 5.10

28.06 ± 5.38

29.43 ± 4.82

29.20 ± 4.82

28.70 ± 9.19

0.166

Reticulocytes (%)

3.37 ± 4.41

3.76 ± 7.09

2.86 ± 3.62

1.3 ± 2.90

3.52 ± 4.09

0.845

RDW (%)

20.12 ± 2.21

20.03 ± 2.64

20.70 ± 2.78

16.43 ± 2.10

19.83 ± 2.38

19.25 ± 1.76

0.171

Leukocytes (× 10⁹/L)

12.01 ± 3.35

13.69 ± 5.08

7.37 ± 5.97

12.65 ± 6.01

10.35 ± 3.56

10.79 ± 3.68

0.002

HbF (%)

5.92 ± 3.05

11.14 ± 3.92

19.44 ± 9.63

17.23 ± 2.87

20.97 ± 2.75

17.80 ± 1.83

< 0.001

Platelet (× 10⁹/L)

496 ± 176

455 ± 148

315 ± 256

420 ± 112

397 ± 194

470 ± 260

0.048

**Table 3.** Analysis of Hematologic Data in Patients of Profile SCD Carrier and Wide Type α-Thalassemia 3.7 kb Deletion From Manaus, Amazon, Brazil
Hematological parameters	Median ± DP
RBCs: red blood cell; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration.
RBC (× 10⁶/mm³)	2.77 ± 0.67	3.31 ± 0.61	0.001
Hemoglobin (g/dL)	8.37 ± 1.50	9.16 ± 1.51	0.026
Hematocrit (%)	25.18 ± 4.52	27.90 ± 4.92	0.012
MCV (fL)	90.56 ± 10.42	84.83 ± 13.99	0.012
MCH (pg)	30.61 ± 4.18	28.07 ± 4.38	0.011
MCHC (g/dL)	33.34 ± 2.15	32.99 ± 1.91	0.481
Reticulocytes (%)	3.40 ± 5.44	4.95 ± 4.70	0.387
Leukocytes (× 10⁹/L)	12,098.59 ± 4,575.25	10,967.72 ± 3,435.04	0.274
Platelet (× 10⁹/L)	474.66 ± 185.41	454.31 ± 201.29	0.643

Table 3. Analysis of Hematologic Data in Patients of Profile SCD Carrier and Wide Type α-Thalassemia 3.7 kb Deletion From Manaus, Amazon, Brazil

Hematological parameters

Median ± DP

Wide type (N = 116)

Carrier (N = 23)

P-value

RBCs: red blood cell; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration.

RBC (× 10⁶/mm³)

2.77 ± 0.67

3.31 ± 0.61

0.001

Hemoglobin (g/dL)

8.37 ± 1.50

9.16 ± 1.51

0.026

Hematocrit (%)

25.18 ± 4.52

27.90 ± 4.92

0.012

MCV (fL)

90.56 ± 10.42

84.83 ± 13.99

0.012

MCH (pg)

30.61 ± 4.18

28.07 ± 4.38

0.011

MCHC (g/dL)

33.34 ± 2.15

32.99 ± 1.91

0.481

Reticulocytes (%)

3.40 ± 5.44

4.95 ± 4.70

0.387

Leukocytes (× 10⁹/L)

12,098.59 ± 4,575.25

10,967.72 ± 3,435.04

0.274

Platelet (× 10⁹/L)

474.66 ± 185.41

454.31 ± 201.29

0.643