Prevalence of Splenomegaly and Splenic Complications in Adults with Sickle Cell Disease and Its Relation to Fetal Hemoglobin
Background: Spleen has been found to be the earliest organ involved in sickle cell disease (SCD) patients with variable manifestations in different geographical regions. It usually undergoes autosplenectomy by adolescence but in countries like India, the course of the disease and splenic manifestations are different. And here we aim to study these differences and the relationship between spleen size and fetal hemoglobin (HbF) and various splenic complications in our patients with sickle cell disease.
Materials and Methods: This is an observational study of 62 adult patients with sickle cell disease admitted in our prestigious institute in the northwestern part of India, mostly hailing from the tribal population. The clinical and ultrasonographic methods have been used to identify splenomegaly and spleen size and prevalence have been calculated. The correlation coefficient has been calculated between fetal hemoglobin, sickle hemoglobin, and spleen size.
Results: The analysis done revealed that 77.4% of patients had abnormal spleen with high average HbF(14.9±5.0) values compared to those who had normal spleen(12.12±4.1). Only 2 patients were found to have no spleen and 3.3% had splenic infarct. All patients with splenomegaly had anemia, 51.6% of patients were in sickle cell crisis and 22.5% were having infections. We also found a weak but positive correlation between spleen size and HbF.
Conclusion: This study revealed the persistence of the spleen, the high prevalence of splenomegaly in the Indian adult population with sickle cell disease, and higher levels of fetal hemoglobin, the exact reason for which is still a subject of speculation that needs research. But this paper provides clear evidence of different natural courses of SCD in India.
2. Ryan K, Bain B, Worthington D, et al. Significant haemoglobinopathies: guidelines for screening and diagnosis. Br J Haematol. 2010;149(1):35-49.
3. Akinsheye I, Alsultan A, Solovieff N, et al. Fetal haemoglobin in sickle cell anemia. Blood. 2011;118(1):19-27.
4. Al-Salem AH. Splenic complications of sickle cell anemia and the role of splenectomy. ISRN Hematol. 2011;2011: 864257.
5. Babadoko AA, Ibinaye PO, Hassan A, et al. Autosplenectomy of sickle cell disease in Zaria, Nigeria: an ultrasonographic assessment. Oman Med J. 2012; 27(2):121-3.
6. Asnani MR, Williams A, Reid M. Splenic enlargement in adults with homozygous sickle cell disease: the Jamaican experience. Hematology. 2013;18(1):46-9.
7. Ahmed MMAO, Ayad CE. The Spleen and Sickle Cell Anaemia: A Contrast Enhanced Computerized Tomography Based Study. Open J Radiol. 2016; 6:168-180.
8. Tubman VN, Makani J. Turf wars: exploring splenomegaly in sickle cell disease in malaria-endemic regions. Br J Haematol. 2017;177(6):938-946.
9. Rumaney MB, Ngo Bitoungui VJ, Vorster AA, et al. The co-inheritance of alpha-thalassemia and sickle cell anemia is associated with better hematological indices and lower consultations rate in Cameroonian patients and could improve their survival. PLoS One. 2014;9(6):e100516.
10. Al-Salem AH. Indications and complications of splenectomy for children with sickle cell disease. J Pediatr Surg. 2006;41(11):1909-15.
11. Brousse V, Buffet P, Rees D. The spleen and sickle cell disease: the sick(led) spleen. Br J Haematol. 2014; 166(2): 165-76.
12. Pearson HA, Spencer RP, Cornelius EA. Functional asplenia in sickle-cell anemia. N Engl J Med.1969; 281(17):923-6.
13. Rogers DW, Clarke JM, Cupidore L, et al. Early deaths in Jamaican children with sickle cell disease. Br Med J. 1978;1(6126):1515-6.
14. Babadoko AA, Ibinaye PO, Hassan A, et al. Autosplenectomy of sickle cell disease in Zaria, Nigeria: An ultrasonographic assessment. Oman Med J. 2012;27(2):121-3.
15. Chopra R, Al‐Mulhim AR, Al‐Baharani AT. Fibrocongestive splenomegaly in sickle cell disease: a distinct clinicopathological entity in the Eastern province of Saudi Arabia. Am J Hematol. 2005;79(3):180-6.
16. Lakhani S, Pandit N, Lakhani JD. Sickle cell disorder and their relation to geographical location: - Relevance to malaria and comprehensive care programs at local and global level. J Integr Health Sci. 2017;5(1):1-4.
17. Grover SA, Barkun AN, Sackett DL. The rational clinical examination. Does this patient have splenomegaly? JAMA. 1993; 270(18): 2218–21.
18. Castell DO. The spleen percussion sign. A useful diagnostic technique. Ann Intern Med. 1967; 67(6):1265-7.
19. Piplani S, Manan R, Lalit M, et al. NESTROFT-A Valuable, Cost Effective Screening Test for Beta Thalassemia Trait in North Indian Punjabi Population. J Clin Diagn Res. 2013;7(12):2784-687.
20. Hosey RG, Mattacola CG, Kriss V, et al. Ultrasound assessment of spleen size in collegiate athletes. Br J Sports Med. 2006;40(3):251-4.
21. Chapman J, Bansal P, Goyal A, et al. Splenomegaly. [Updated 2020 Aug 10]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430907/ assessed on 5th september 2020.
22. Alvarez-Uria G, Naik PK, Midde M, et al. Prevalence and severity of anaemia stratified by age and gender in rural India. Anemia. 2014;2014:176182.
23. Colah RB, Mukherjee MB, Martin S, et al. Sickle cell disease in tribal populations in India. Indian J Med Res. 2015;141(5):509-15.
24. Lehman H, Cutbush M. Sickle cell trait in southern India. Brit Med J. 1952;1 (4755):404–5.
25. Jain D, Tokalwar R, Upadhye D, et al. Homozygous sickle cell disease in Central India & Jamaica: A comparison of newborn cohorts. Indian J Med Res. 2020;151(4):326-332.
26. Serjeant GR, Ghosh K, Patel J. Sickle cell disease in India: A perspective. Indian J Med Res. 2016;143(1):21-4.
27. Kar BC, Satapathy RK, Kulozik AE, et al. Sickle cell disease in Orissa State, India. Lancet 1986; 2(8517):1198-201.
28. Lakhani JD, Shah V, Gandhi D, et al. Sickle cell disorders in females: Screening of sickle hemoglobinopathy be part of Antenatal and Intensive care?. J Integr Health Sci. 2016;4(2):31-6.
29. Akinlosotu MA, Adeodu OO, Adegoke SA, et al. Fetal hemoglobin level and its relationship with spleen size and malaria parasite density in Nigerian children with sickle cell anemia. Ann Trop Med Public Health. 2018;11(4):133-9.
30. Gupta P, Acharya S, Shukla S. Comparison of adverse prognostic haematological parameters in sickle cell anaemia patients with splenomegaly and without splenomegaly. IOSR J Dent Med Sci. 2016;15(2):48-52.
31. Parmar D, Likhar KS. Prevalence of splenomegaly in Sickle cell anemia patients in relation to Hemoglobin. F. IJRRMS. 2013;3(3):18-20.
32. MishraB, Nayak MK,MishraS, et al. Splenectomy in sickle cell haemoglobinopathies. Int Surg J. 2019;6(4):1371-5.
33. Pembrey ME, Wood WG, Weatherall DJ, et al. Fetal haemoglobin production and the sickle gene in the oases of Eastern Saudi Arabia. Br J Haematol. 1978;40(3):415-29.
34. Lakhani JD, Vaswani VN, et al . A study of blood indices in sickle cell disorder. Ann Trop Med & Public Health. 2020; 23(S23): SP2323136.
35. Balci A, Karazincir S, Sangün Ö, et al. Prevalence of abdominal ultrasonographic abnormalities in patients with sickle cell disease. Diagn Interv Radiol. 2008;14(3):133-7.
36. Lakhani JD, Vaswani VN, Kalaria TK, et al. A study of Blood indices in Sickle Cell Disorder. Annals of Tropical Medicine and Health. 2020 Dec;23:232-3136.
37. AlFadhli SM, Al-Awadhi AM, AlKhaldi DA. Validity assessment of nine discriminant functions used for the differentiation between iron deficiency anemia and thalassemia minor. J Trop Pediatr. 2007;53(2):93-7.
|Issue||Vol 16, No 4 (2022)|
|Sickle cell disease; Spleen; Splenomegaly; Fetal hemoglobin|
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