Abstract 4625

BACKGROUND

Blood transfusions are the standard of care in β thalassemia and transfusions are also indicated in Sickle Cell Disease (SCD) patients with hypersplenism, recurrent vaso-occlusive crises and for stroke prevention. Iron overload caused by blood transfusions in thalassemia and in SCD may affect morbidity and mortality. Recent studies of iron overload in SCD suggest that the biologic features of SCD and the chronic inflammatory state may protect SCD patients from iron damage.

DESIGNS AND METHODS

In view of the controversy regarding the effect of iron overload in patients with SCD we studied the iron status, including non transferrin bound iron (NTBI) and labile plasma iron (LPI) levels in a cohort of thirty six SCD patients and compare the results with 43 thalassemia patients.

RESULTS

Our results indicate that none of the SCD patients had clinical symptoms of iron overload. Only two SCD patients had NTBI values in the grey zone (0.4 units) and none had positive values. By contrast, 14 patients with Thalassemia Major and 3 with Thalassemia Intermedia had NTBI values above 0.6, level that are in the positive pathological range. Similarly, four thalassemia patients, but only one SCD patient had positive LPI levels.

CONCLUSIONS

The parameters of iron status in SCD patients, even after frequent transfusions are different when compared to patients with thalassemia. The low NTBI and LPI levels found in patients with SCD are in keeping with the absence of clinical signs of iron overload in this disease.

Table

Iron overload in Sickle Cell Disease and β thalassemia - Laboratory data.

Sickle Cell Disease
β Thalassemia
p *
SSSβThalTotal SCDβTh Mβ TITotal β Thalassemia
Hgb (gr/dl) 8.9±1.5 8.3±1 8.7±1.3 8.3±0.9 8.2±1 8.3±0.9 0.171 
Serum Iron (μg/dl) 97±37 110±51 102±43 209±50 150±57** [0.007] 195±57 <0.001 
Serum Transferrin (μg/dl) 209±36 194±28 203±33 148±31 141±24 [<0.001] 146±29 <0.001 
Transferrin Saturation(%) 33±16 38±17 35±17 96±17 73±28** [<0.001] 91±22 <0.001 
Ferritin (ng/dl) 1083±1528 1361±1494 1202±1498 3388±2344 2354±2105 [NS] 3149±2306 <0.001 
Patients studied  16 / 21 13 / 15 29 / 36 29 / 30 8 / 13 37 / 43  
NTBI (units) 0.0±0.1 (0) 0.0±0.1 (0) 0.0±0.1 (0) 0.56±0.52 (14) 0.5±0.65 (3) [<0.001] 0.55±0.54 (17) <0.001 # 
LPI (units) 0.1±0.2 (1) 0.0±0.1 (0) 0.1±0.1 (1) 0.22±0.33 (3) 0.2±0.35 (1) 0.21±0.33 (4) 0.017 
DCI (units) 0.0±0.0 (0) 0.0±0.0 (0) 0.0±0.0 (0) 0.14±0.36 (4) 0.0±0.0 (0) [0.07] 0.11±0.33 (4) 0.066 
Sickle Cell Disease
β Thalassemia
p *
SSSβThalTotal SCDβTh Mβ TITotal β Thalassemia
Hgb (gr/dl) 8.9±1.5 8.3±1 8.7±1.3 8.3±0.9 8.2±1 8.3±0.9 0.171 
Serum Iron (μg/dl) 97±37 110±51 102±43 209±50 150±57** [0.007] 195±57 <0.001 
Serum Transferrin (μg/dl) 209±36 194±28 203±33 148±31 141±24 [<0.001] 146±29 <0.001 
Transferrin Saturation(%) 33±16 38±17 35±17 96±17 73±28** [<0.001] 91±22 <0.001 
Ferritin (ng/dl) 1083±1528 1361±1494 1202±1498 3388±2344 2354±2105 [NS] 3149±2306 <0.001 
Patients studied  16 / 21 13 / 15 29 / 36 29 / 30 8 / 13 37 / 43  
NTBI (units) 0.0±0.1 (0) 0.0±0.1 (0) 0.0±0.1 (0) 0.56±0.52 (14) 0.5±0.65 (3) [<0.001] 0.55±0.54 (17) <0.001 # 
LPI (units) 0.1±0.2 (1) 0.0±0.1 (0) 0.1±0.1 (1) 0.22±0.33 (3) 0.2±0.35 (1) 0.21±0.33 (4) 0.017 
DCI (units) 0.0±0.0 (0) 0.0±0.0 (0) 0.0±0.0 (0) 0.14±0.36 (4) 0.0±0.0 (0) [0.07] 0.11±0.33 (4) 0.066 

p = p value between SCD and β Thalassemia.

**

p= 0.005 between αThM and β TI.

[ ] p between ThI and SCD.

¶= N0 of patients studied by NTBI, LPI and DCI analysis.

The results were expressed as mean ± SD. () = No of patients with positive values (>0.7).

#

= p <0.01 (x2 test) – No of patients with positive NTBI, thalassemia vs SCD.

SCD: Sickle Cell Disease, SS: Homozygous Sickle Cell, SβThal: Sickle Cell β Thalassemia. β ThM: β Thalassemia Major, β TI: β Thalassemia Intermedia.

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Disclosures:

Koren:Novartis Oncology: Research Funding. Levin:Novartis Oncology: Research Funding.

Topics:
iron, iron overload, plasma, sickle cell anemia, transferrin, thalassemia, blood transfusion, cooley's anemia, thalassemia intermedia, ferritin

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2009 by The American Society of Hematology
2009
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