Autoimmune hemolytic anemia (AIHA) is characterized by shortened red cell survival due to the presence of autoantibodies directed against antigens on the red blood cell membrane. It is generally diagnosed in relation to a viral or bacterial infection but has also been described in association with drugs, malignancy, autoimmune disease, and immunodeficiency states. The occurrence of AIHA in patients with sickle cell anemia is uncommon. An 8-month-old African American female with sickle cell anemia presented with congestion, irritability, and increased sleepiness. The patient had no previous history of blood transfusion. Examination revealed pallor and splenomegaly. Investigations revealed severe anemia with reticulocytosis, elevated lactate dehydrogenase, and indirect hyperbilirubinemia. Considering the splenomegaly and decrease in hemoglobin from baseline, the initial clinical diagnosis made was splenic sequestration crisis. However, a positive direct antiglobulin test for IgG at 37°C during routine cross matching for transfusion confirmed the diagnosis of warm AIHA. No compatible blood was available secondary to the presence of antibody. The infant was successfully treated with corticosteroids for approximately 3 weeks with a subsequent increase in hemoglobin to baseline and a negative direct antiglobulin test after discharge.
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