Pediatric emergency medicine trisk 2190 2190

Promptly evaluate patients with acute onset of pallor and jaundice for
hemolytic anemia.
Autoimmune hemolytic anemia (AIHA) can be life threatening, especially in
cases of severe anemia and reticulocytopenia.
Early consultation with hematology and the blood bank helps to optimize
patient management and to provide sufficient time for compatibility testing if
transfusion is needed.
With evidence of hemolysis, transfusion indications include symptomatic
anemia or low presenting hemoglobin (<5 to 6 g/dL in children, <6 to 7 g/dL in
adolescents).
Avoid excessive crystalloid fluid resuscitation in severely anemic patients with
hemolytic processes.

Current Evidence
The premature destruction of RBCs in circulation (hemolytic anemia) is most commonly
due to intrinsic defects of erythrocytes (membranopathies, enzymopathies, or
hemoglobinopathies) in pediatric patients; however, extrinsic or extracorpuscular factors
such as antibodies, environmental stresses, infection, or microangiopathic damage may
also cause hemolysis. The severity of anemia can range from mild to life threatening and
is often influenced by the underlying mechanism. For example, erythrocyte membrane
disorders (hereditary spherocytosis, hereditary elliptocytosis, hereditary stomatocytosis)
and metabolic abnormalities (glucose-6-phosphate dehydrogenase [G6PD] deficiency,
pyruvate kinase deficiency) do not usually cause severe anemia and rarely constitute a
life-threatening emergency. AIHA, on the other hand, may present with a precipitously
falling hemoglobin level in a child who appears critically ill with signs of congestive
heart failure. Reported mortality rates for pediatric patients with hemolytic anemia range
from 4% to 10%. Fortunately, this is a rare condition in children.

Goals of Treatment
Successful treatment of hemolytic anemia requires stabilization of the hemoglobin level
and maintenance of sufficient oxygen-carrying capacity and cardiac output; clinical

outcomes depend on early recognition and intervention for uncompensated anemia and
ultimately diagnosis of the underlying condition.

Clinical Considerations
Clinical Recognition
Hemolytic anemia arises in all age groups. The typical presenting features include
pallor, jaundice, and dark urine. Other symptoms include fatigue, malaise, dizziness,
fever, and abdominal or back pain. Symptoms often develop suddenly, and in cases of
rapid hemolysis, the presenting hemoglobin level may be as low as 3 to 4 g/dL. Some