Splenic sequestration crisis as an index manifestation of heterozygous hemoglobinopathy in an adult

Sickle β⁺-thalassemia rarely manifests with acute splenic sequestration crisis in adults. We report a case of a 20-year-old female who presented with fever and left upper quadrant abdominal pain. Laboratory studies revealed hemolytic anemia. Tests for autoimmune hemolysis and hemolytic diseases were negative except for Hemoglobin (Hb) electrophoresis, which revealed sickle cell trait (Hb AS). Infectious workup was unremarkable. Computed tomography scan of the abdomen showed marked splenomegaly. The patient received blood transfusions and empiric antibiotics with no improvement; thus, splenectomy was performed. Pathology specimen revealed peripheral serpiginous infarcts alternating with surrounding acute inflammation and small capillaries plugged with sickle cell shaped red blood cells consistent with splenic sequestration. DNA test later revealed beta-globin mutations consistent with sickle cell-beta⁺ thalassemia. Post-splenectomy, there was a gradual improvement in her clinical symptoms with concomitant rise in Hb to 10.6 g/dl at discharge.