The serum ferritin levels and liver iron concentrations in patients with alpha-thalassemia: is there a good correlation?

Figures & data

Figure 1. Serum ferritin levels and liver iron concentrations among patients with alpha-thalassemia disease.

Figure 2. Grap box of serum ferritin to liver iron concentration ratios in patients with alpha-thalassemia disease.

Table 1. Baseline clinical characteristics of 65 patients with alpha-thalassemia.

Characteristics	Deletional alpha-thalassemia (group A, n=22)	Non-deletional alpha-thalassemia (group B, n=43)
Mean age ± SD, years (at enrolment)	35 ± 18	28 ± 13
Mean hemoglobin ± SD, g/dL	8 ± 0.7	8.6 ± 0.9
Mean red blood cell transfusion ± SD, ml/kg/year	34 ± 26	31 ± 27
Mean serum ferritin ± SD, ng/mL	1008 ± 653	862 ± 604
Liver iron concentration ± SD, mg Fe/g dw	8.2 ± 7.3	8.6 ± 5.8
Mean time interval between serum ferritin and MRI-T2* measurement, day	32 ± 28	37 ± 27
Transfusion-dependent-thalassemia, n (%)
No	22 (100)	38 (88.4)
Yes	0 (0)	5 (11.6)
Gender, n (%)
Female	10 (45)	23 (53)
Male	12(55)	20 (47)
Splenectomy, n (%)
No	21 (95)	31 (72)
Yes	1 (5)	12 (28)
Genotype group, n (%)
- Hb H disease	12 (54.5)	–
- Hb H disease with Hb CS	–	20 (46.5)
- Hb H disease with Hb Paksé	–	2 (4.5)
- EABart’s disease*	9 (41)	–
- EABart’s disease with Hb CS**	–	20 (46.5)
- EABart’s disease with Hb Paksé***	–	1 (2.5)
- EFBart’s disease ****	1 (4.5)	–

Abbreviation: Hb CS = Hemoglobin Constant spring, Hb Paksé= Hemoglobin Paksé *compound heterozygous Hb H and heterozygous Hb E **compound heterozygous Hb H with Hb CS and heterozygous Hb E ***compound heterozygous Hb H with Hb Pakséand heterozygous Hb E **** compound heterozygous Hb H and homozygous Hb E.

Figure 3. The ROC curve of liver iron concentration ≥ 7 mg Fe/g dw and serum ferritin levels in patients with alpha-thalassemia disease.

Table 2. Predictive values of serum ferritin of < 300 ng/ml for dose interruption and serum ferritin of > 2,000 ng/ml for dose escalation of iron chelation therapy in patients with alpha-thalassemia.

	Alpha-thalassemia (this study)	NTDT (Taher et al, 2015)
Positive predictive value (PPV) for dose interruption	89.8	86.1
Risk of chelating despite LIC < 3 mg Fe/g dw (%)	10.2	13.9
Negative predictive value (NPV) for dose interruption	84.3	40
Risk of not chelating despite LIC ≥ 3 mg Fe/g dw (%)	15.7	60
Positive predictive value (PPV) for dose escalation	28.3	100
Risk of dose escalation despite LIC < 7 mg Fe/g dw (%)	71.7	0
Negative predictive value (NPV) for dose escalation	100	40
Risk of not dose escalation despite LIC ≥ 7 mg Fe/g dw (%)	0	60

Taher AT, Porter JB, Viprakasit V, et al. Defining serum ferritin thresholds to predict clinically relevant liver iron concentrations for guiding deferasirox therapy when MRI is unavailable in patients with non-transfusion-dependent thalassaemia. Br J Haematol. 2015;168(2):284–290.

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