A rare presentation of hypersplenism in an adult female with homozygous sickle cell disease

Hypersplenism in adults with homozygous sickle cell disease (HbSS) is rare, as most patients undergo autosplenectomy during early childhood. However, phenotypic variants such as high fetal hemoglobin, coexisting hemoglobinopathies, and different globin gene haplotypes can lead to splenic preservation and altered disease progression. These patients often exhibit milder symptoms, delayed complications, and increased morbidity. We report a rare case of a 43-year-old female with HbSS and elevated fetal hemoglobin who presented with hypersplenism, characterized by massive splenomegaly, pancytopenia, and compensatory bone marrow proliferation. She underwent an open splenectomy with an uneventful postoperative course. This case highlights the importance of recognizing atypical presentations in adult patients with sickle cell disease, in whom splenomegaly may persist. Clinicians should maintain a high index of suspicion to facilitate timely diagnosis and appropriate management, which can significantly improve patient outcomes.