Challenging case of hemolytic anemia and splenic sequestration in sickle cell disease

Abstract A 5-year-old female with sickle cell disease (SCD) (Hb SS) and poor compliance with hydroxyurea (HU) presented with a decline in hemoglobin (Hb), splenomegaly, and vaso-occlusive crisis. All of which become overlapping clinical features of different complications of SCD, including delayed hemolytic transfusion reaction (DHTR), and warm autoimmune hemolytic anemia (wAIHA). This patient’s initial presentation was with an orbital bone infarct for which she received an Rh/Kell antigens-unmatched transfusion. Because of subsequent alloimmunization with anti-D, anti-C, anti-E, and nonspecific IgG antibodies, she was started on a chronic transfusion regimen. At follow-up, she was noted to have splenomegaly and critically low Hb levels. She was then found to have developed wAIHA, splenic sequestration, and hyperhemolysis syndrome/possible DHTR. Through multidisciplinary decision-making, she was treated with intravenous immunoglobulin, methylprednisolone, and rituximab to stabilize her Hb and manage splenic sequestration to then allow for fully phenotypically matched red blood cells’ transfusion. Her last Hb during the admission was 7.8 g/dL. At this point, splenectomy was done in a safer context as definitive treatment for recurrent sequestration and immune hemolysis. This case highlights several important aspects of the care of patients with SCD, including multiple complications, treatment, and the importance of multidisciplinary coordination in high-risk or complex cases.