Pregnancy-Triggered Vaso-Occlusive Crises in Hemoglobin SE Disease Complicated by Glucose-6-Phosphate Dehydrogenase Deficiency: A Case Report.

Hemoglobin SE (HbSE) disease is a rare hemoglobinopathy that may be associated with significant clinical complications despite its traditional characterization as a mild condition, and concomitant glucose-6-phosphate dehydrogenase (G6PD) deficiency can further complicate diagnosis and management. We report the case of a 40-year-old Saudi woman with known G6PD deficiency who experienced recurrent vaso-occlusive crises triggered by pregnancy. She first presented postpartum in 2017 with severe joint pain, and initial hemoglobin electrophoresis suggested sickle cell trait (HbS: 27.1%, HbA: 67.9%). Six years later, during her fourth pregnancy complicated by gestational diabetes, she developed severe lower limb pain. Repeat hemoglobin electrophoresis demonstrated HbS of 66.3%, HbE of 27.1%, and HbA of 0%, confirming compound heterozygous HbSE disease. The delay in diagnosis was attributed to discrepant electrophoresis findings and a low index of clinical suspicion. This case underscores the importance of considering HbSE disease in patients with unexplained vaso-occlusive symptoms, particularly when pregnancy serves as a physiological stressor, and highlights that HbSE is not invariably benign. Awareness of the potential impact of coexisting G6PD deficiency on laboratory interpretation is essential, and comprehensive hemoglobin analysis, along with genetic counseling, is crucial for accurate diagnosis and appropriate family screening.