Vol. 7, Issue 1, Part A (2025)

HbSE disease is a rare hemoglobinopathy characterized by the coexistence of hemoglobin S (HbS) and hemoglobin E (HbE) in a double heterozygous state. This case report highlights the clinical presentation, diagnosis, and implications of HbSE disease in a 37-year-old male with hypertension and type 2 diabetes mellitus. The patient presented with persistent abdominal pain, and subsequent investigations revealed bilateral nephrolithiasis and a left-sided polycystic kidney. Hematological analysis showed mild anemia with microcytosis, and high-performance liquid chromatography (HPLC) confirmed the presence of HbS (56%) and HbE (32.7%). While HbSE disease is often considered a benign condition, it can present with vaso-occlusive complications under stressful conditions. The case emphasizes the importance of screening for hemoglobinopathies in patients with unexplained vaso-occlusive events, anemia, or microcytosis to prevent misdiagnosis and unnecessary investigations. HPLC remains the gold standard for diagnosis. Early detection and appropriate disease management strategies, including family screening, are essential to prevent complications and improve patient outcomes.