Histopathologic and molecular evidence of splenic infarction associated with sickle cell trait: An instructive case in Central America

Abstract

Sickle cell trait is generally considered asymptomatic; there are nevertheless potential complications. The spleen is vulnerable to infarction due to its role in trapping and removing sickle cells and its hypoxic environment. Here we report a case of a 30-year-old physically active man, who experienced severe abdominal pain near the peak of the Acatenango Volcano in Guatemala. At the hospital, he was diagnosed with splenic infarction, requiring splenectomy. Pathological analysis of the spleen showed interstitial hemorrhage and marked congestion of capillaries by sickle-shaped erythrocytes. Laboratory studies showed no evidence of anemia or any alteration in the red blood cell formula. Leukocytosis was observed at the time of the event but rapidly decreased on subsequent days. In contrast, platelets increased after the spleen removal. Capillary electrophoresis revealed the sickle cell carrier state. Genetic alterations associated with thrombophilia, alpha thalassemia and other beta globin hemoglobinopathies were absent. This case reinforces with evidence that high-altitude hypoxia can trigger sickle cell formation in heterozygous carriers and lead to splenic damage.