Sickle cell trait: a cause of abdominal pain and pulmonary embolism
DOI:
https://doi.org/10.31053/1853.0605.v77.n4.25077Keywords:
sickle cell trait, splenic infarction, pulmonary embolism, lupus coagulation inhibitor.Abstract
Introduction: Sickle cell trait (SCT) is a rare and underdiagnosed disorder in the Argentinian population. In this condition, individuals carry the mutation of the HbS gene in one of the two beta-globin genes. In general, SCT does not present with the typical manifestations of sickle cell anemia. However, under certain circumstances, some clinical characteristics of the disease may develop. Methods: We discussed the case of a 39-Year old man who presented with persistent abdominal pain of unknown origin after traveling to a high-altitude place. He underwent laparotomy without a definite diagnosis. After that, the patient developed signs of splenic infarction and pulmonary thromboembolism that were confirmed by computed tomography. Results: A sickling test was positive, and a hemoglobin electrophoresis revealed an abnormal fraction at the HbS level. In this context a diagnosis of SCT was made. Additional, tests revealed a strongly positive lupus anticoagulant. Conclusion: SCT presentation as abdominal pain and thromboembolic disease in adult patients after exposure to high altitudes is a rarely suspected diagnosis.
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References
De Franceschi L, Cappellini MD, Olivieri O. Thrombosis and sickle cell disease. Semin Thromb Hemost. 2011;37:226-36.
Knorr M, Bienemann K, Walde G, Kaufhold A, Schündeln MM. A massive intestinal vaso-occlusive crisis or "girdle syndrome" in a 6-year-old boy observed as a first manifestation of sickle cell disease. Klin Padiatr. 2014;226:372-4.
Kotila TR. Sickle Cell Trait: A Benign State?. Acta Haematol. 2016;136:147-51.
Naik RP, Smith-Whitley K, Hassell KL et al. Clinical Outcomes Associated With Sickle Cell Trait: A Systematic Review. Ann Intern Med. 2018;169:619-627.
Huttle A, Maestre GE, Lantigua R, Green NS. Sickle cell in Latin America and the United States. Pediatr Blood Cancer. 2015;62:1131-6
Erramouspe, Beatriz, Eandi Eberle, Silvia Judith. Técnicas convencionales aplicadas al diagnóstico de las hemoglobinopatías. Acta Bioquímica Clínica Latinoamericana [online] 2017, 51 [Last acces: March 31, 2019] Available at: <http://www.redalyc.org/articulo.oa?id=53553013007> .
Noubiap JJ, Temgoua MN, Tankeu R, Tochie JN, Wonkam A, Bigna JJ. Sickle cell disease, sickle trait and the risk for venous thromboembolism: a systematic review and meta-analysis. Thromb J. 2018;16:27.
Ahmed S, Shahid RK, Russo LA. Unusual causes of abdominal pain: sickle cell anemia. Best Pract Res Clin Gastroenterol. 2005;19:297-310.
Yanamandra U, Das R, Malhotra P, Varma S. A Case of Autosplenectomy in Sickle Cell Trait Following an Exposure to High Altitude. Wilderness Environ Med. 2018;29:85-89.
Habibzadeh S, Maleki N. Sickle cell trait at high altitude. Int J Crit Illn Inj Sci. 2015;5:129-30.
Jebbin NJ, Adotey JM. Acute abdominal conditions in people with sickle cell disease: a 10-year experience in Port Harcourt, Nigeria. Ann Afr Med. 2011;10:165-70.
Goodman J, Hassell K, Irwin D, Witkowski EH, Nuss R. The splenic syndrome in individuals with sickle cell trait. High Alt Med Biol. 2014;15:468-71.
Seegars MB, Brett AS. Splenic infarction associated with sickle cell trait at low altitude. Hematology. 2015;20:607-9.
Ataga KI, Orringer EP. Hypercoagulability in sickle cell disease: a curious paradox. Am J Med. 2003;115:721-8.
Shet AS, Wun T. How I diagnose and treat venous thromboembolism in sickle cell disease. Blood. 2018;132:1761-1769.
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