Main liver manifestations in sickle cell disease




Sickle cell anemia; Liver diseases.


Sickle cell disease (SC) is an autosomal recessive genetic disease that occurs due to a mutation in the β-globin gene. Among the main genotypes are hemoglobin SS (HbSS), a homozygous form responsible for a series of complications, which vary according to the affected system. Within the digestive tract, the hepatobiliary system is the most affected. Sickle cell liver diseases, so called hepatic manifestations in sickle cell disease, can be classified into acute and chronic, which can generate serious consequences for carriers. This literature review, conducted between July and October 2022, has as its main objective to elucidate the main liver manifestations in sickle cell disease, as well as to discuss possible behaviors to reduce mortality in this group. For the composition of this review, articles published between the years 2017 and 2022 were selected, from the search for keywords through the following electronic databases: PUBMED Central (PMC) and Scielo. In total, 21 articles were selected. It can be concluded from the analysis of these that the pathophysiology and clinical manifestations of sickle cell liver diseases are well clarified in the literature. However, the ways to prevent these complications and treatment still need more studies.


Alkhayyat, M., Saleh, M. A., Zmaili, M., Sanghi, V., Singh, T., Rouphael, C., Linares, C. R. S., Marrero, C. R., Carey, W. D., & Lindenmeyer, C. C. (2020). Successful liver transplantation for acute sickle cell intrahepatic cholestasis: A case report and review of the literature. World Journal of Gastrointestinal Pathophysiology, 12(7), 108–115.

Allali, S., Taylor, M., Brice, J., & Montalembert, M. d. M. (2021). Chronic organ injuries in children with sickle cell disease. Haematologica, 106(6), 1535–1544.

Cisneros, G. S., & Thein, S. L. (2020). Recent Advances in the Treatment of Sickle Cell Disease. Frontiers in Pediatrics, 11, 435.

Drasar, E., Fitzpatrick, E., Gardner, K., Awogbade, M., Dhawan, A., Bomford, A., Suddle, A., & Thein, S. L. (2017). Interim assessment of liver damage in patients with sickle cell disease using new non-invasive techniques. British Journal of Haematology, 176(4), 643–650.

Hogen, R., Kim, M., Lee, Y., Lo, M., Kaur, N., Kahn, J., Chopra, S., Qazi, Y., Sedra, A., Kim, J., O’Brien, L., Genyk, Y., Sher, L., & Emamaullee, J. (2020). Liver Transplantation in Patients with Sickle Cell Disease in the U.S. Journal of Surgical Research, 255, 23–32.

Inah, G. B., & Ekanem, E. E. (2019). Sonographic diagnosis and clinical correlates of gallbladder stones in patients with sickle cell disease in calabar, nigeria. Open Access Macedonian Journal of Medical Sciences, 7(1), 68–72.

Khan, A., Nashed, B., Issa, M., & Khan, M. Z. (2022). Sickle cell intrahepatic cholestasis: Extremely rare but fatal complication of sickle cell disease. Cureus Journal of Medical Science, 14(2).

Kyrana, E., Rees, D., Lacaille, F., Fitzpatrick, E., Davenport, M., Heaton, N., Height, S., Samyn, M., Mavilio, F., Brousse, V., Suddle, A., Chakravorty, S., Verma, A., Gupte, G., Velangi, M., Inusa, B., Drasar, E., Hadzic, N., Grammatikopoulos, T., & Dhawan, A. (2020). Clinical Management of Sickle Cell Liver Disease in Children and Young Adults. Archives of Disease in Childhood, 106(4), 315–320.

Martins, R. A., Soares, R. S., De Vito, F. B., Barbosa, V. d. F., Silva, S. S., Souza, H. M., & Martins, P. R. J. (2017). Cholelithiasis and its complications in sickle cell disease in a university hospital. Revista Brasileira de Hematologia e Hemoterapia, 39(1), 28–31.

Mawuli, G., Dzudzor, B., Tachi, K., Kuma, A. A. B.-A., Odame-Aboagye, J., Obeng, B. M., Boateng, A. T., Edu-Quansah, E. P., Attiku, K. O., Agbosu, E., Arjarquah, A., & Bonney, J. H. K. (2022). Hepatitis C virus (HCV) infection among patients with sickle cell disease at the Korle-Bu teaching hospital. Virology Journal, 19, 73.

Mehrabani, S., Tammadoni, A., & Osia, S. (2019). Abdominal pain in a patient with sickle cell disease with multiple complications. Turkish Archives of Pediatrics, 54(4), 267–271.

Mendes, K. D. S., Silveira, R. C. D. C. P., & Galvão, C. M. (2008). Revisão integrativa: Método de pesquisa para incorporação de evidências na saúde e na enfermagem. Texto & Contexto - Enfermagem, 17(4), 758-764.

Ochocinski, D., Dalal, M., Black, L. V., Carr, S., Lew, J., Sullivan, K., & Kissoon, N. (2020). Life-Threatening infectious complications in sickle cell disease: A concise narrative review. Frontiers in Pediatrics, 8, 38.

Praharaj, D. L., & Anand, A. C. (2021). Sickle hepatopathy. Journal of Clinical and Experimental Hepatology, 11(1), 82–96.

Rambaud, E., Ranque, B., Tsiakyroudi, S., Joseph, L., Bouly, N., Douard, R., François, A., Pouchot, J., & Arlet, J.-B. (2022). Risks and benefits of prophylactic transfusion before cholecystectomy in sickle cell disease. Journal of Clinical Medicine, 11(14), 3986.

Rodrigues, L., Almeida, S., Salgado, C., & Gonçalves, C. (2020). Pediatric acute liver failure in sickle cell disease. GE - Portuguese Journal of Gastroenterology, 29(3), 192–196.

Samuel, S. S., & Jain, N. (2022). Sickle cell hepatopathy. In StatPearls. StatPearls Publishing LLC.

Shah, R., Taborda, C., & Chawla, S. (2017). Acute and chronic hepatobiliary manifestations of sickle cell disease: A review. World Journal of Gastrointestinal Pathophysiology, 8(3), 108–116.

Suddle, A. R. (2019). Management of liver complications in sickle cell disease. Hematology: The American Society of Hematology Education Program, 2019(1), 345–350.

Thein, S. L., & Howard, J. (2018). How I treat the older adult with sickle cell disease. Blood, 132(17), 1750–1760.

Williams, T. N., & Thein, S. L. (2018). Sickle cell anemia and its phenotypes. Annual Review of Genomics and Human Genetics, 19, 113–147.



How to Cite

CHAGAS, M. do S.; FRIGGI, J. R. Main liver manifestations in sickle cell disease. Research, Society and Development, [S. l.], v. 11, n. 15, p. e441111537486, 2022. DOI: 10.33448/rsd-v11i15.37486. Disponível em: Acesso em: 31 jan. 2023.



Health Sciences