|Year : 2014 | Volume
| Issue : 4 | Page : 148-150
Serum ferritin level in adult sickle cell anemia in Saudi population
Ahmed M Alsuliman, Mahmoud Albagshi, Kefah B Algadeeb, Abdulmottaleb Aldanden, Abbas I Alabdultif
Department of Medicine, Hematology Section, King Fahad Hospital, Hofuf, Saudi Arabia
|Date of Web Publication||13-Dec-2014|
Ahmed M Alsuliman
Department of Medicine, King Fahad Hospital
Source of Support: None, Conflict of Interest: None
Background: Vaso-occlusive and hemolysis are the clinical hallmarks of sickle cell disease .patients with sickle cell at risk of iron overload due to chronic blood transfusion treating complications of the disease ,in addition to increasing iron absorption from gut and chronic hemolysis. Material and Methods: This is retrospective study on total of 174 adult sickle cell disease patients were included in this study, attending hematology clinic in king Fahad Hofuf hospital and inherited blood disorder center-Alhassa. for each patient demographic data was obtained (age, sex, nationality), history of previous blood transfusion, hemoglobin level, serum ferritin level. In total, 174 naïve Saudi adult patients (113 males, 61 females) were included in this study. Mean age was 28.2 ±10 years (range 12- 62 years), sex ratio (M/F) was 1.8 :1.0 . 93 (53%) patients had history of previous blood transfusion. The overall mean serum ferritin concentration was 587 ± 547(18.49 - 2660 ng\dl). The mean serum ferritin level for male is 649 ± 606 ng\dl and mean serum ferritin level for female is 473± 440ng\dl. Conclusion: Iron overload in sickle cell disease patients from eastern province remains mild to moderate, with significant sex difference. The repetitive assessment of serum ferritin level should be consider to sickle cell patients whom frequent blood transfusion is needed like stroke and renal failure.
Keywords: Ferritin, red blood cells, sickle cell
|How to cite this article:|
Alsuliman AM, Albagshi M, Algadeeb KB, Aldanden A, Alabdultif AI. Serum ferritin level in adult sickle cell anemia in Saudi population. J Appl Hematol 2014;5:148-50
|How to cite this URL:|
Alsuliman AM, Albagshi M, Algadeeb KB, Aldanden A, Alabdultif AI. Serum ferritin level in adult sickle cell anemia in Saudi population. J Appl Hematol [serial online] 2014 [cited 2020 Jan 25];5:148-50. Available from: http://www.jahjournal.org/text.asp?2014/5/4/148/146949
| Introduction|| |
Vaso-occlusive and hemolysis are the clinical hallmarks of sickle cell disease (SCD). Patients with sickle cell at risk of iron overload due to chronic blood transfusion are treating complications of the disease, in addition to increasing iron absorption from gut and chronic hemolysis. ,,,
Ferritin is the primary iron storage protein in tissues; it is also an acute phase reactant with elevated serum levels in the presence of chronic inflammation, infection, and liver disorders associated with SCD in crisis. ,,
In this study, we examined the extent of iron overload and the predictive value of ferritin in estimating iron overload in adult with SCD.
| Materials and Methods|| |
In this retrospective study, the total of 174 adult SCD patients were included, attending hematology clinic for each patient demographic data (age, sex, nationality), and history of previous blood transfusion, hemoglobin (Hb) level, alanine aminotransferase (ALT), aspartate aminotransferase (AST) alkaline phosphates bilirubin level, hepatitis B and C, serum ferritin level were obtained. Including criteria were adult patients (age ≥ 12 years) in their usual state of health with no admission for 1-month serum ferritin values measure by immunoenzyme and normal range is adult males (17-323 ug/L) adult females (7-283 ug/L) data were analyzed using Chi-square analysis, and P < 0.05 were consider to be as significant.
| Results|| |
A total of 174 naïve Saudi adult patients (113 males, 61 females) were included in this study. The mean age was 28.2 ± 10 years (range: 12-62 years), sex ratio (male/female) was 1.8:1.0 [Table 1]. About 93 (53%) patients had a history of previous blood transfusion during their course, but no one of them was in chronic blood transfusion program.
The overall mean serum ferritin concentration was 587 ± 547 (18.49-2660 ng\dl). The mean serum ferritin level for male is 649 ± 606 ng\dl, and mean serum ferritin level for female is 473 ± 440 ng\dl.
Moreover, 10 of our patients are positive for the hepatitis marker, with mean serum ferritin level of 549.1 ± 462.7 (176-1118 ng\dl). ALT mean 41 ± 33.3, AST 35.7 ± 31.2. No one of patients had received iron chelating therapy.
| Discussion|| |
This study evaluates the magnitude of iron overload in SCD patients in the eastern province of Saudi Arabia. This study shows that mean serum ferritin levels of 174 patients with SCD is 587 ± 547 ng\dl (18.49-2660 ng\dl), with significant differences between both sex as male has higher mean serum ferritin as compared to female (male 649 ± 606 ng\dl, female 473 ± 440 ng\dl) [Table 2].
Majority (80%) of patients have serum ferritin level < 1000 ng\dl [Table 3] with no serious health problems related to iron overload comparing to patients with B-thalassemia major. Many studies showed behavior of iron overload SCD comparing to thalassemia major is different and suggest SCD patients are relatively protected from iron-induced cardiac and endocrine organ damage. ,,,, In fact, one study report one possible mechanism of protection of patient with SCD increase iron elimination rates through renal system. 
Classically, serum ferritin has been used to monitor patient with high serum ferritin and to less extent liver biopsy. So high serum ferritin concentration need to be deal cautiously in SCD patients with no history of chronic blood transfusion, as serum ferritin can be elevated in different medical conditions (fever, acute painful crisis, infection, inflammation, and hepatic dysfunction). Recently, magnetic resonance imaging has proven effectively in detecting and quantifying iron deposition in liver and heart. ,,,,,
Iron overload is toxic to many tissues, particularly the heart and endocrine system. About 53% of our patients had history of blood transfusion for complication events or surgical indication, but not reaching to intensity of β - thalassemia major blood transfusion.it is known SCD - patients in Eastern part of Saudi Arabia maintaining high level of Hb at steady - state, milder disease phenotype comparing to African subtype due to genetic factors including alpha-thalassemia and high levels of fetal Hb, so there are not routinely transfused except for acute complications or stroke and renal failure. ,,,
Lower mean serum ferritin level in Female was reported in different studies. It may contribute to dietary deficiency, blood loss with the menstrual cycle, multi pregnancy, uterine dysfunction in female. ,,,,,
There is one study came from AL-Hassa area of Saudi Arabia supporting gender differences in acute pain, and showed that female do better than male in acute SCD pain crisis and these gender differences were not explained in that study. ,
Sickle cell disease patients are suffering from frequent painful crises; several studies showed iron depletion or less iron in the body is better by decreasing episode of painful crises and hemolysis. These studies suggested the mechanism of this effect is probably multifactorial: (a) The concentration of Hb level is known to influence the blood viscosity and its decrease always improved rheology in SCD patients; (b) the mean corpuscular Hb concentration is a critical factor concerning the HbS molecule polymerization in SCD, and its slight reduction may have an important biological effect. Prospective controlled studies are needed to evaluate further this observation. ,,,,,,
| Conclusion|| |
Iron overload in SCD patients from the eastern province remains mild to moderate, with significant sex difference. The repetitive assessment of serum ferritin level should be considered to sickle cell patients whom frequent blood transfusion is needed like stroke and renal failure.
| References|| |
Rechavi G, Rivella S. Regulation of iron absorption in hemoglobinopathies. Curr Mol Med 2008;8:646-62.
Akinbami AA, Dosunmu AO, Adediran AA, Oshinaike OO, Osunkalu VO, Ajibola SO, et al
. Serum ferritin levels in adults with sickle cell disease in Lagos, Nigeria. J Blood Med 2013;4:59-63.
Gyang E, Yeom K, Hoppe C, Partap S, Jeng M. Effect of chronic red cell transfusion therapy on vasculopathies and silent infarcts in patients with sickle cell disease. Am J Hematol 2011;86:104-6.
Adams RL, Bird RJ. Safety and efficacy of deferasirox in the management of transfusion-dependent patients with myelodysplastic syndrome and aplastic anaemia: A perspective review. Ther Adv Hematol 2013;4:93-102.
Wang W, Knovich MA, Coffman LG, Torti FM, Torti SV. Serum ferritin: Past, present and future. Biochim Biophys Acta 2010;1800:760-9.
Walter PB, Harmatz P, Vichinsky E. Iron metabolism and iron chelation in sickle cell disease. Acta Haematol 2009;122:174-83.
Claster S, Wood JC, Noetzli L, Carson SM, Hofstra TC, Khanna R, et al
. Nutritional deficiencies in iron overloaded patients with hemoglobinopathies. Am J Hematol 2009;84:344-8.
Mariani R, Trombini P, Pozzi M, Piperno A. Iron metabolism in thalassemia and sickle cell disease. Mediterr J Hematol Infect Dis 2009;1:e2009006.
Ghugre NR, Gonzalez-Gomez I, Butensky E, Noetzli L, Fischer R, Williams R, et al.
Patterns of hepatic iron distribution in patients with chronically transfused thalassemia and sickle cell disease. Am J Hematol 2009;84:480-3.
Hankins JS, Smeltzer MP, McCarville MB, Aygun B, Hillenbrand CM, Ware RE, et al.
Patterns of liver iron accumulation in patients with sickle cell disease and thalassemia with iron overload. Eur J Haematol 2010;85:51-7.
Harmatz P, Butensky E, Quirolo K, Williams R, Ferrell L, Moyer T, et al.
Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy. Blood 2000;96:76-9.
Raghupathy R, Manwani D, Little JA. Iron overload in sickle cell disease. Adv Hematol 2010;2010 :272940.
Inati A, Musallam KM, Wood JC, Taher AT. Iron overload indices rise linearly with transfusion rate in patients with sickle cell disease. Blood 2010;115:2980-1.
Patra PK, Khodiar PK, Panigrahi S, Srivastava N. Study of serum ferritin, iron and total iron binding capacity in sickle cell disease. J Adv Res Biol Sci 2012;4:340-4.
Tsitsikas DA, Nzouakou R, Ameen V, Sirigireddy B, Amos RJ. Comparison of serial serum ferritin measurements and liver iron concentration assessed by MRI in adult transfused patients with sickle cell disease. Eur J Haematol 2014;92:164-7.
Inati A, Musallam KM, Wood JC, Sheikh-Taha M, Daou L, Taher AT. Absence of cardiac siderosis by MRI T2* despite transfusion burden, hepatic and serum iron overload in Lebanese patients with sickle cell disease. Eur J Haematol 2009;83:565-71.
Neto JP, Lyra IM, Reis MG, Goncalves MS. The association of infection and clinical severity in sickle cell anaemia patients. Trans R Soc Trop Med Hyg 2011;105:121-6.
Puliyel M, Sposto R, Berdoukas VA, Hofstra TC, Nord A, Carson S, et al.
Ferritin trends do not predict changes in total body iron in patients with transfusional iron overload. Am J Hematol 2014;89:391-4.
Akohoue SA, Shankar S, Milne GL, Morrow J, Chen KY, Ajayi WU, et al
. Energy expenditure, inflammation, and oxidative stress in steady-state adolescents with sickle cell anemia. Pediatr Res 2007;61:233-8.
Jastaniah W. Epidemiology of sickle cell disease in Saudi Arabia. Ann Saudi Med 2011;31:289-93.
Adekile A, Al-Kandari M, Haider M, Rajaa M, D'Souza M, Sukumaran J. Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients. Med Princ Pract 2007;16:286-90.
el Mouzan MI, al Awamy BH, al Torki MT. Clinical features of sickle cell disease in eastern Saudi Arab children. Am J Pediatr Hematol Oncol 1990;12:51-5.
Quadri MI, Islam SI, Nasserullah Z. The effect of alpha-thalassemia on cord blood red cell indices and interaction with sickle cell gene. Ann Saudi Med 2000;20:367-70.
Kassim A, Thabet S, Al-Kabban M, Al-Nihari K. Iron deficiency in Yemeni patients with sickle-cell disease. East Mediterr Health J 2012;18:241-5.
Rodrigues PC, Norton RC, Murao M, Januario JN, Viana MB. Iron deficiency in Brazilian infants with sickle cell disease. J Pediatr (Rio J) 2011;87:405-11.
Mohanty D, Mukherjee MB, Colah RB, Wadia M, Ghosh K, Chottray GP, et al.
Iron deficiency anaemia in sickle cell disorders in India. Indian J Med Res 2008;127:366-9.
Roopnarinesingh S. Iron stores in pregnant patients with anaemia and haemoglobin S. Br J Obstet Gynaecol 1976;83:375-7.
King L, Reid M, Forrester TE. Iron deficiency anaemia in Jamaican children, aged 1-5 years, with sickle cell disease. West Indian Med J 2005;54:292-6.
Mohamed AO, Bayoumi RA, Hofvander Y, Omer MI, Ronquist G. Sickle cell anaemia in Sudan: Clinical findings, haematological and serum variables. Ann Trop Paediatr 1992;12:131-6.
Udezue E, Girshab AM. Differences between males and females in adult sickle cell pain crisis in eastern Saudi Arabia. Ann Saudi Med 2004;24:179-82.
Udezue E, Herrera E. Pain management in adult acute sickle cell pain crisis: A viewpoint. West Afr J Med 2007;26:179-82.
Koduri PR. Iron in sickle cell disease: A review why less is better. Am J Hematol 2003;73:59-63.
Giordano PC, Huisman W, Harteveld CL. Iron depletion: An ameliorating factor for sickle cell disease? ISRN Hematol 2011;2011:473152.
Rao KR, Ray VH, Patel AR. Serum ferritin and sequestered stores of body iron. Am J Clin Pathol 1983;80:743-5.
Haddy TB, Castro O. Overt iron deficiency in sickle cell disease. Arch Intern Med 1982;142:1621-4.
Rao KR, Patel AR, McGinnis P. Iron deficiency anemia in an adult with sickle cell disease. Blood 1978;52 Suppl:87.[abstract].
Castro O, Poillon WN, Finke H, Massac E. Improvement of sickle cell anemia by iron-limited erythropoiesis. Am J Hematol 1994;47:74-81.
Bouchaïr N, Manigne P, Kanfer A, Raphalen P, de Montalembert M, Hagege I, et al.
Prevention of sickle cell crises with multiple phlebotomies. Arch Pediatr 2000;7:249-55.
[Table 1], [Table 2], [Table 3]