Yassin Mohamed, Soliman Ashraf, De Sanctis Vincenzo, Nashwan Abdelqadir, Abusamaan Sandra, Moustafa Abbas, Kohla Samah, Soliman Dina
Department of Hematology, Hamad Medical Center, Doha.
Department of Pediatric, University of Alexandria, Egypt.
Mediterr J Hematol Infect Dis. 2017 Jun 20;9(1):e2017037. doi: 10.4084/MJHID.2017.037. eCollection 2017.
Sickle cell disease (SCD) is one of the leading causes 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 challenging its management.
To determine the iron status of 17 patients with non-transfusion-dependent sickle cell disease ( NT-SCD) patients and six patients with transfusion dependent sickle cell disease (TD- SCD) using both serum ferritin level (SF) and Ferriscan® evaluation of liver iron content (LIC). We correlated the values of LIC with SF levels and some hepatic enzymes (alanine transaminase-ALT, aspartate aminotransferase -AST, alkaline phosphatase -ALP and albumin).
17 adults with NT-SCD (n = 17, age: 32±15 years) were studied. Seven of NT-SCD had SF > 500 μg/L, 4 out of the seven had high liver iron measured by FerriScan® (> 30 mg/g/ tissue dry weight - dw). Two patients had high LIC despite a concomitant SF concentration < 500 μg/L. Two patients had high SF (1.117 μg/L and 675 μg/L) while their LIC was normal (< 30 mg/g/dw). Five patients had elevated ALT and/or AST) concentrations. In TD-SCD (n = 6, age = 25 ± 11 years), 2 patients had SF <500 μg/L, one of them had high LIC (127 mg/g/DW). Liver enzymes were high in two patients. SF concentration correlated significantly with LIC (r = 0.85, p < 0.001). Neither SF level nor LIC was correlated significantly with hepatic enzyme levels.
A significant number of our patients with NT-SCD had high LIC, high SF and elevated liver enzymes (ALT and AST). Despite some limitations of our study, due to the limited number of NT-SCD patients, these findings have important clinical implications. Therefore, we recommend measuring SF and LIC in NT-SCD patients to apply preventive measures with iron chelation therapy in patients with high LIC.
镰状细胞病（SCD）是全球发病和死亡的主要原因之一，会导致多个器官的损伤和功能障碍。这种疾病的并发症众多，会影响身体的每个器官和/或组织，并且随着时间的推移，患者之间的差异很大，这给疾病的管理带来了挑战。
使用血清铁蛋白水平（SF）和Ferriscan®评估肝脏铁含量（LIC）来确定17例非输血依赖型镰状细胞病（NT-SCD）患者和6例输血依赖型镰状细胞病（TD-SCD）患者的铁状态。我们将LIC值与SF水平以及一些肝酶（丙氨酸转氨酶-ALT、天冬氨酸转氨酶-AST、碱性磷酸酶-ALP和白蛋白）进行了关联。
对17例NT-SCD成人患者（n = 17，年龄：32±15岁）进行了研究。NT-SCD患者中有7例SF>500μg/L，其中4例通过FerriScan®测量的肝脏铁含量较高（>30mg/g/组织干重-dw）。尽管同时SF浓度<500μg/L，但仍有2例患者的LIC较高。2例患者的SF较高（分别为1117μg/L和675μg/L），而其LIC正常（<30mg/g/dw）。5例患者的ALT和/或AST浓度升高。在TD-SCD患者（n = 6，年龄 = 25±11岁）中，2例患者的SF<500μg/L，其中1例患者的LIC较高（127mg/g/DW）。2例患者的肝酶较高。SF浓度与LIC显著相关（r = 0.85，p < 0.001）。SF水平和LIC均与肝酶水平无显著相关性。
我们的大量NT-SCD患者存在高LIC、高SF以及肝酶（ALT和AST）升高的情况。尽管我们的研究存在一些局限性，由于NT-SCD患者数量有限，但这些发现具有重要的临床意义。因此，我们建议对NT-SCD患者测量SF和LIC，以便对LIC高的患者应用铁螯合疗法的预防措施。
