Acute splenic sequestration in an adult patient with sickle cell trait and beta-thalassemia

Secuestro esplénico agudo en un paciente adulto con rasgo falciforme y beta-talasemia

Introduction: Splenic sequestration (SE) crisis with sickle cell disease is a rare disease in adults. Its occurrence in patients with sickle cell trait and beta-thalassemia is unusual, particularly during pregnancy. This report aims to describe the case of an adult patient with a mixed hemoglobinopathy (HbS/β-thalassemia) who presented with SE crisis as the first manifestation of the disease. Clinical case:  A descriptive study case report of a 35-year-old female patient with a history of anemia during her second pregnancy, presented two years later referring fatigue, weight loss, and dyspnea. During hospital stay, laboratory tests, including hemoglobin electrophoresis, and imaging studies revealed arregenarative anemia secondary to hemolysis, a mixed phenotype for sickle cell disease with B thalassemia and hepatosplenomegaly. Despite transfusional support, hemolysis persisted, requiring emergency splenectomy which improved symptoms and decreased hemolytic activity. Histopathological analysis from splenic biopsy showed red pulp expansion with diminished white pulp. Conclusions: Splenic sequestration is a life-threatening complication primarily observed in children with sickle cell disease, but its occurrence in adults is exceptionally rare, particularly in patients with mixed hemoglobinopathy. In this case, splenic function was preserved due to high percentage of circulating HbA, which explained late presentation of the disease. In adult patients with severe anemia refractory to treatment, splenectomy should be considered to prevent severe complications and improve patient outcomes.