Epidemiologic study of major complications in adolescent and adult patients with thalassemia in Northeastern Thailand: the E-SAAN study phase I

Figures & data

Figure 1. Prevalence and numbers of disease-related complications in 380 patients with thalassemia by age group.

Table 1. Baseline clinical characteristics of 380 patients with thalassemia.

Characteristics	TDT (n = 91)	NTDT (n = 289)
Mean age ± SD, years (at enrollment)	19.5 ± 10	28 ± 13.5
Transfusion index ± SD (ml/kg/months)	62 ± 15	10 ± 14.1
Mean hemoglobin ± SD, g/dL	7.5 ± 0.9	8.2 ± 3
Mean platelet count ± SD, × 10^9/L	483 ± 265.4	349.8 ± 238.4
Mean nucleated RBC/100 WBC ± SD, × 10^6/L	186.8 ± 256	81 ± 218
Mean serum ferritin ± SD, ng/mL	2091 ± 1725	1490 ± 1765
Liver size ± SD, cm	3.3 ± 2.8	2 ± 5.2
Time after splenectomy	2 ± 11.5	1 ± 9
Gender, n (%)
Female	56 (61.5)	183 (63.3)
Male	35 (38.5)	106 (36.7)
Splenectomy, n (%)
No	44 (48.4)	71 (24.6)
Yes	47 (51.6)	218 (75.4)
Previous complications, n (%)
No	36 (39.5)	165 (57)
Yes	55 (60.5)	124 (43)
Previous blood transfusion, n (%)
No	0 (0)	16 (5.5)
Yes	91 (100)	273 (94.5)
Current iron chelation, n (%)
No	7 (7.7)	130 (45)
Deferoxamine	17 (18.6)	24 (8.3)
Deferiprone	32 (35.2)	110 (38.1)
Deferasirox	3 (3.3)	5 (1.7)
Combined deferoxamine and deferiprone	32 (35.2)	20 (6.9)
Genotype group, n (%)
β-Thalassemia/Hb E	91 (100)	139 (48.1)
Hb H disease	0 (0)	32 (11.1)
Hb H disease with Hb CS	0 (0)	42 (14.6)
Hb H disease with Hb Paksé	0 (0)	7 (2.4)
EABart’s disease^a	0 (0)	18 (6.2)
EABart’s disease with Hb CS^b	0 (0)	44 (15.2)
EFBart’s disease with Hb CS^c	0 (0)	2 (0.7)
EABart’s disease with Hb Paksé^d	0 (0)	2 (0.7)
EFBart’s disease^e	0 (0)	3 (1)

Notes: Hb CS = Hemoglobin Constant spring, Hb Paksé = Hemoglobin Paksé

^aCompound heterozygous Hb H and heterozygous Hb E.

^bCompound heterozygous Hb H with Hb CS and heterozygous Hb E.

^cCompound heterozygous Hb H with Hb CS and homozygous Hb E.

^dCompound heterozygous Hb H with Hb Pakséand heterozygous Hb E.

^eCompound heterozygous Hb H and homozygous Hb E.

Table 2. Prevalence of disease-related complications between patients with TDT and NTDT.

Complications	TDT, n (%) n = 91	NTDT, n (%) n = 289	Total, n (%) n = 380
No	36 (39.5)	165 (57.1)	201 (52.9)
Pulmonary hypertension	0 (0)	22 (7.6)	22 (5.8)
Heart failure	4 (4.4)	6 (2.1)	10 (2.6)
Extramedullary hematopoiesis	2 (2.2)	8 (2.8)	10 (2.6)
Osteoporosis	0 (0)	4 (1.4)	4 (1.1)
Cholelithiasis	25 (27.5)	51 (17.6)	76 (20)
Diabetes mellitus	3 (3.3)	0 (0)	3 (0.8)
Hypothyroidism	4 (4.4)	0 (0)	4 (1.1)
Thrombosis	0 (0)	4 (1.4)	4 (1.1)
Infection	17 (18.7)	27 (9.3)	44 (11.5)
Leg ulcer	0 (0)	2 (0.7)	2 (0.5)

Notes: TDT = transfusion-dependent thalassemia, NTDT = non-transfusion-dependent thalassemia.

Table 3. Univariate analysis of risk factors for disease-related complications in 91 patients with TDT.

Variables	OR	95% CI of OR	p-Value
Age group
10–20 year old	1.0	–	–
21–30 year old	1.0	0.3–3.2	0.9
31–40 year old	4.1	0.4–36.1	0.2
41–50 year old	1.3	0.1–15.8	0.8
More than 50 year old	ND	ND	ND
Female gender	0.5	0.2–1.3	0.2
Splenectomy	6.1	2.4–15.8	0.0001
Hemoglobin <8 g/dl	0.7	0.2–1.7	0.4
Serum ferritin >1000 ng/ml	1.1	0.3–3.1	0.9

Notes: OR = odds ratio, 95% CI = 95% confidence interval, ND = not determined.

Table 4. Univariate analysis of risk factors for disease-related complications in 289 patients with NTDT.

Variables	OR	95% CI of OR	p-Value
Age group
10–20 year old	1.0	–	–
21–30 year old	1.0	0.6–1.7	0.9
31–40 year old	2.1	0.9–4.9	0.07
41–50 year old	0.7	0.3–1.8	0.4
More than 50 year old	2.6	0.9–7.6	0.07
Genotype group
α-thalassemia	1	–	–
Combined α and β-thalassemia	1.2	0.6–2.6	0.5
β-Thalassemia/Hb E	1.7	0.8–3.2	0.1
Female gender	0.5	0.3–0.9	0.02
Splenectomy	2.5	1.5–4.1	0.0003
Hemoglobin <8 g/dl	1.5	0.9–2.4	0.09
Serum ferritin >800 ng/ml	1.1	0.3–3.1	0.9

Notes: OR = odds ratio, 95% CI = 95% confidence interval.

Table 5. Multivariate analyses of risk factors for major complications in 91 patients with TDT.

Variables	AOR	95% CI of AOR	p-Value
Age group
10–20 year old	1	–	–
21–30 year old	0.6	0.1–2.6	0.5
31–40 year old	4.9	0.4–54.9	0.1
41–50 year old	3	0.1–46.1	0.4
More than 50 year old	ND	ND	ND
Female gender	0.5	0.2–1.6	0.3
Splenectomy	7.4	2.4–22.1	0.0001
Hemoglobin <8 g/dl	0.4	0.1–1.3	0.1
Serum ferritin >1,000 ng/ml	1.2	0.3–4.7	0.7

Notes: AOR = adjusted odds ratio, 95% CI = 95% confidence interval, ND = not determined.

Table 6. Multivariate analyses of risk factors for major complications in 289 patients with non-transfusion-dependent thalassemia.

Variables	AOR	95% CI of AOR	p-Value
Age group
10–20 year old	1.0	–	–
21–30 year old	0.7	0.4–1.4	0.3
31–40 year old	1.8	0.7–4.4	0.1
41–50 year old	0.7	0.3–1.9	0.5
More than 50 year old	2.9	1.1–8.7	0.04
Genotype group
α-Thalassemia	1.0	–	–
combined α and -β-thalassemia	1.0	0.4–2.2	0.9
β-Thalassemia/Hb E	1.1	0.5–2.2	0.9
Female gender	0.5	0.3–0.9	0.03
Splenectomy	2.6	1.4–4.4	0.001
Hemoglobin <8 g/dl	1.4	0.8–2.3	0.2
Serum ferritin >800 ng/ml	1.1	0.6–1.7	0.9

Notes: AOR = adjusted odds ratio, 95% CI = 95% confidence interval.