Retrospective epidemiological study of Latin American patients with transfusional hemosiderosis: the first Latin American epidemiological study in iron overload – the RELATH study

Figures & data

Figure 1. Patient distribution by country (n = 960).

Table 1. Demographics and patient characteristics

Characteristic	Value or n (%)
	All	SCD	Thalassemia	MDS	Others
	n = 960	n = 464	n = 230	n = 69	n = 197
Age, years – mean (SD)	28·5 (19·8)	25·0 (14·8)	21·4 (12·6)	62·7 (20·4)	33·2 (23·3)
Race – n (%)
White/Caucasian	294 (30·8)	47 (10·2)	145 (63·3)	39 (56·5)	63 (32·3)
Black/African	354 (37·0)	316 (68·3)	21 (9·2)	3 (4·3)	14 (7·2)
Hispanic	244 (25·5)	88 (19·0)	34 (14·8)	25 (36·2)	97 (49·7)
Other	111 (11·6)	24 (5·2)	54 (23·6)	4 (5·8)	29 (14·9)
Pediatric population (n)	355	187	94	5	30
Age, years – mean (SD)	10·9 (4·4)	11·6 (4·0)	10·3 (4·8)	62·7 (20·4)	33·2 (23·3)
2–6 years (n)	66	20	26	1	14
6–12 years (n)	150	92	32	3	8
12–18 years (n)	139	75	36	1	8
Prior RBC transfusions
⩾20 – n (%)	836 (87·1)	413 (89·0)	210 (91·3)	51 (73·9)	162 (82·1)
10–20 – n (%)	124 (12·9)	51 (11·0)	20 (8·7)	18 (26·1)	35 (17·8)
RBC transfusions/year – mean (SD)	11·8 (8·9)	8·5 (4·8)	14·4 (8·9)	16·9 (14·7)	15·1 (10·6)
Pediatric patients	11·8 (6·1)	9·6 (4·0)	13·3 (6·1)
Median serum ferritin level (mcg/l)	1960	1951	2000	1945·5	1902
(range)	(44–21 828)	(51–13 101)	(405–15 874)	(50–7532)	(44–21 829)
Pediatric patients (mcg/L)	1913	1801	1960	1000·0	2000
(range)	(290–21 829)	(290–9049)	(478–7030)	(445–3405)	(920–21 829)

Note: MDS, myelodysplastic syndrome; SCD, sickle-cell disease; SD, standard deviation.

Table 2. Iron overload complications in the overall population

n (%)	All	SCD	Thalassemia	MDS	Other
	n = 960	n = 464	n = 230	n = 69	n = 197
Endocrine
Growth curve <50th percentile	106 (11·0)	23 (5·0)	50 (21·7)	0 (0)	33 (16·8)
GH level deficiency	20 (2·1)	1 (0·2)	18 (7·8)	0 (0)	1 (0·5)
Hypogonadism	55 (5·7)	2 (0·4)	46 (20·0)	0 (0)	7 (3·6)
LH, FSH deficiency	26 (2·7)	0 (0)	21 (9·1)	0 (0)	5 (2·5)
Hyperglycemia	86 (9·0)	16 (3·4)	21 (9·1)	21 (30·4)	28 (14·2)
T4 deficiency, TSH elevated	41 (4·3)	8 (1·7)	22 (9·6)	6 (8·7)	5 (2·5)
Hypocalcemia	27 (2·8)	5 (1·1)	16 (7·0)	2 (2·9)	4 (2·0)
Cardiac
Decreased LVEF	25 (2·6)	12 (2·6)	9 (3·9)	1 (1·4)	3 (1·5)
Symptomatic congestive heart failure	47 (4·9)	31 (6·7)	11 (4·8)	3 (4·3)	2 (1·0)
Hepatic
Abnormal liver function enzymes	198 (20·6)	27 (5·8)	63 (27·4)	54 (78·3)	54 (27·4)
Abnormal bilirubin	210 (21·9)	19 (4·1)	69 (30·0)	61 (88·4)	61 (31·0)
Abnormal liver ultrasound	105 (10·9)	10 (2·2)	37 (16·1)	29 (42·0)	29 (14·7)
Other
Skin pigmentation	120 (12·5)	17 (3·7)	50 (21·7)	8 (11·6)	45 (22·8)
Arthropathy	37 (3·9)	23 (5·0)	5 (2·2)	0 (0)	9 (4·6)

Note: LH, luteinizing hormone; FSH, follicle stimulating hormone; TSH, thyroid stimulating hormone; LVEF, left ventricular ejection fraction.

Figure 2. Hemoglobin level at initiation of transfusions by age (A) and disease (B).

Table 3. Hepatic complications in pediatric patients with transfusional iron overload

Liver abnormality	SCD	Thalassemia	Total
	n = 187	n = 94	n = 355
Enzymes – n (%)	77 (41·2)	41 (43·6)	144 (40·6)
Bilirubin – n (%)	106 (56·7)	32 (34·0)	149 (42·0)
Ultrasound – n (%)	45 (24·1)	20 (21·3)	76 (21·4)
CT scan – n (%)	1 (0·5)	2 (2·1)	3 (0·8)
MRI – n (%)	3 (1·6)	3 (3·2)	6 (1·7)
Biopsy; fibrosis – n (%)	0 (0)	12 (12·8)	12 (3·4)
Biopsy; cirrhosis – n (%)	0 (0)	4 (4·3)	4 (1·1)
Biopsy; other – n (%)	3 (1·6)	10 (10·6)	15 (4·2)

Note: CT, computed tomography; MRI, magnetic resonance imaging.

*Includes five patients with MDS.