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the hemoglobin level may continue to fall precipitously despite initial therapy. Patients
with symptomatic or severe anemia (hemoglobin <5 to 6 g/dL in children, <6 to 7 g/dL
in adolescents) should receive a transfusion of pRBCs. Hemolytic transfusion reactions
are a potential hazard. Transfuse an initial 5 to 10 mL of blood slowly to test for a
significant hemolytic transfusion reaction associated with the selected aliquot of blood.
If the patient does not manifest signs or symptoms of acute worsening during the initial
infusion, increase the transfusion rate. Ultimately, the transfusion rate must exceed the
rate of ongoing hemolysis. Careful monitoring of hemoglobin levels is important, with a
stable hemoglobin goal of 6 to 8 g/dL.

FIGURE 93.3 Management of autoimmune hemolytic anemia.

The management strategy differs for those with a warm-reactive antibody versus
those with a cold-reactive antibody as highlighted in Figure 93.3 . Hemolysis and
erythrocyte clearance in the setting of a warm-reactive antibody is generally
extravascular, taking place in the spleen. These patients typically respond to
corticosteroid therapy, but may also benefit from concomitant IVIG or splenectomy in
more dire circumstances. Cold-reactive AIHA involves intravascular hemolysis,
dominated by complement activation and with limited response to corticosteroids or
splenectomy. These patients should be kept warm (i.e., avoid cold exposure), and they
may benefit from plasmapheresis in severe cases. In the setting of intravascular
hemolysis, accumulation of circulating free hemoglobin and cellular contents can be
toxic to the renal tubular system. Careful fluid management to ensure adequate renal
clearance without excessive dilution of the red cells is imperative.