LIVER IRON CONTENT (LIC) IN ADULTS WITH NON-TRANSFUSION DEPENDENT SICKLE CELL DISEASE (NT-SCD). CORRELATION WITH SERUM FERRITIN AND LIVER ENZYMES CONCENTRATIONS

Vincenzo De Sanctis

Abstract

Abstract. Introduction: Sickle cell disease (SCD) is an important cause of morbidity and mortality worldwide, causing damage and dysfunction in multiple organs. The complications of this disease are numerous, affect every organ and/or tissue in the body and vary considerably among patients over the time which challenge its management. Aim of our study: To determine the iron status of 17 patients with NT-SCD patients and 6 patients with TD- SCD using both serum ferritin level (SF ) and Ferriscan® evaluation of liver iron content (LIC) and correlate values of LIC on the one hand with SF levels and some hepatic functions (ALT, AST, ALP and albumin).  Results: 17 adults with NT-SCD (n = 17, age: 32±15 years) were studied.  Seven of NT-SCD had serum ferritin > 500 μg/L, 4 out of the seven had high liver iron measured by FerriScan®  (> 30 mg/kg/ tissue dry weight - DW).  Two patients had high liver iron content despite a concomitant serum ferritin concentration < 500 μg/L.  Two patients had high serum ferritin (1.117 μg/L and 675 μg/L) while their LIC was normal (< 30 mg/kg/DW).  5 patients had elevated ALT and/or AST concentrations. In TD-SCD (n = 6, age = 25 ±11 years), 2 patients had serum ferritin <500 μg/L, one of them had high LIC (127 mg/kg/DW). Liver enzymes were high in two patients.  Serum ferritin concentration was correlated significantly with LIC  (r = 0.85, p < 0.001). Neither serum ferritin level, nor LIC was correlated significantly with hepatic enzyme levels. Conclusions:  A significant number of our patients with ND-SCD had high LIC , high serum ferritin and elevated hepatic enzymes (ALT and AST). Despite some  limitations of our study (small NT-SCD cohort),  these findings have important clinical implications. We recommend  to measure serum ferritin and LIC in NT-SCD patients to apply therapeutic preventive measures with iron chelation therapy in patients with high LIC.  

Keywords

Hematology

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Submitted: 2016-12-21 13:16:34
Published: 2017-06-20 00:00:00
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