Hematological profile of children with sickle cell Disease in special reference to body iron stores
Abstract
Introduction: Sickle cell anemia is a genetic disorder commonly seen in rural population of western and central India. Being an important cause of hemolytic anemia, repeated blood transfusions are frequently required for the sickle cell anemia cases. Few studies have exclusively addressed hematological profile of sickle cell disease patients. This study was planned to study the hematological profile of children with special reference to body iron stores.
Methods: This was a prospective observational study carried out in department of pediatrics S.B.K.S MIRC. All the confirmed cases of children diagnosed with sickle cell disease or sickle β thalassemia were subjected to complete hematological analysis in special reference to body iron store by evaluation of serum iron, TIBC, % transferrin saturation and serum ferritin to find out total body iron store.
Results: About 110 patients were included in the study. In our study low MCV values were seen in 60.4% of patients, low MCH values in 84.9% of patients while MCHC was normal in 94.3% of patients. Serum iron levels showed normal values in majority of patients (94.3%) while transferrin saturation was low in 62.3% patients thus showing the discrepancy on correlating both values for diagnosis of iron deficiency.
Conclusion: Out of various parameters for diagnosis of iron status in SCD-SS patients, low MCV, MCH, Serum iron level and transferrin saturation more consistently associated with iron deficient state.
Downloads
References
2. The Georgia Comprehensive Sickle Cell Center at Grady Health System, The Sickle Cell Foundation of Georgia, Inc., Emory University School of Medicine,Department of Pediatrics. (1997). Sickle cell information center. http://www.scinfo.org/sicklept.htm
3. Shapiro BS. The management of pain in sickle cell disease. Pediatr Clin North Am. 1989 Aug;36(4):1029-45. [PubMed]
4. Wood, R.A., Fosarelli, P., Hudak, M., Lake, A., & Modlin, L. Pediatrics. Philadelphia: Lipincott. 1989.
5. Hussain MA, Davis LR, Laulicht M, Hoffbrand AV. Value of serum ferritin estimation in sickle cell anaemia. Arch Dis Child. 1978 Apr;53(4):319-21. [PubMed]
6. Erlandson ME, Walden B, Stern G, Hilgartner MW,Wehman J, Smith CH, et al. Studies on congenital haemolytic syndromes IU. Gastrointestinal absorption of iron. Blood 1962;19:359.
7. Cohen A, Schwartz E. Excretion of iron in response to deferoxamine in sickle cell anemia. J Pediatr. 1978 Apr;92(4):659-62. [PubMed]
8. Vichinsky E, Kleman K, Embury S, Lubin B. The diagnosis of iron deficiency anemia in sickle cell disease. Blood. 1981 Nov;58(5):963-8. [PubMed]
9. Mohanty D, Mukherjee MB, Colah RB, Wadia M, Ghosh K, Chottray GP, Jain D, Italia Y, Ashokan K, Kaul R, Shukla DK, Muthuswamy V. Iron deficiency anaemia in sickle cell disorders in India. Indian J Med Res. 2008 Apr;127(4):366-9. [PubMed]
10. Khan Y, Thakur AS, Mehta R, Kundu RK, Agnihotram G. Hematological profile of sickle cell disease: a hospital based study at CIMS, Bilaspur, Chhattisgarh. IJABPT 2010;1(2): 717-721.
11. Sahu T, Sahani NC, Das S, Sahu SK. Sickle cell anaemia in tribal children of gajapati district in south orissa. Indian Journal of Community Medicine 2003;28(4).
12. Kar BC. Sickle cell disease in India. J Assoc Physicians India. 1991 Dec;39(12):954-60. [PubMed]
13. Mandot S, Khurana VL, Sonesh JK. Sickle cell anemia in Garasia tribals of Rajasthan. Indian Pediatr. 2009 Mar;46(3):239-40. Epub 2009 Jan 1. [PubMed]
14. Walke VA, Walde MS. Haematological study in sickle cell homozygous and heterozygous children in the age group 0-6 years. Indian J Pathol Microbiol. 2007 Oct;50(4):901-4.
15. Kaur M, Das GP, Verma IC. Sickle cell trait & disease among tribal communities in Orissa, Madhya Pradesh & Kerala. Indian J Med Res. 1997 Mar;105:111-6.
16. Juwah AI, Nlemadim EU, Kaine W. Types of anaemic crises in paediatric patients with sickle cell anaemia seen in Enugu, Nigeria. Arch Dis Child. 2004 Jun;89(6):572-6. [PubMed]
17. Haddy TB, Castro O. Overt iron deficiency in sickle cell disease. Arch Intern Med. 1982 Sep;142(9):1621-4. [PubMed]
18. Davies S, Henthorn J, Brozović M. Iron deficiency in sickle cell anaemia. J Clin Pathol. 1983 Sep;36(9):1012-5. [PubMed]
19. Lulla RR, Thompson AA, Liem RI. Elevated soluble transferrin receptor levels reflect increased erythropoietic drive rather than iron deficiency in pediatric sickle cell disease. Pediatr Blood Cancer. 2010 Jul 15;55(1):141-4. doi: 10.1002/pbc.22471.
20. Oluboyede OA, Usanga EA, Lukanmbi FA, Ajayi OA. Evaluation of serum ferritin levels and other hematological parameters in a Nigerian population. J Natl Med Assoc. 1983 September; 75(9): 885–889.
21. Rao KR, Patel AR, McGinnis P, Patel MK. Iron stores in adults with sickle cell anemia. J Lab Clin Med. 1984 May;103(5):792-7.
22. Guyatt GH, Oxman AD, Ali M, Willan A, McIlroy W, Patterson C. Laboratory diagnosis of iron-deficiency anemia: an overview. J Gen Intern Med. 1992 Mar-Apr;7(2):145-53.
Copyright (c) 2017 Author (s). Published by Siddharth Health Research and Social Welfare Society
This work is licensed under a Creative Commons Attribution 4.0 International License.