Abdominal Pain and Anemia in a Child

Allison King, MD, MPH and John DiPersio, MD, PhD

Washington University School of Medicine, St. Louis, MO

This case was reviewed and updated in June 2013 by Dr. Ted Wun and members of the Teaching Cases Subcommittee.

Copyright of the American Society of Hematology, 2006. ISSN: 1931-6860.

VII. TEACHING POINTS

  1. Sickle cell disease is an autosomal recessive disorder caused by the inheritance of a mutated beta globin gene that under low oxygen conditions forms polymers and alters the red cell membrane. It is associated with chronic hemolytic anemia and recurrent vaso-occlusive crises (VOC). These crises can be associated with tissue infarct in the bones, lungs, liver, central nervous system, and kidneys.

  2. Patients with sickle cell disease may have a variable clinical course due in part to the amount of HbS and fetal hemoglobin in their red cells. Recent data suggest two sub-phenotypes defined by the degree of hemolysis as denoted by LDH level. Patients with higher LDH levels may have marked anemia, pulmonary arterial hypertension, leg ulcers, and episodes of priapism. Patients with lower LDH levels may have more frequent VOC and avascular necrosis. However, there is considerable overlap in these sub-phenotypes and this represents a continuum rather than a dichotomy.

  3. Initial management of acute chest syndrome requires recognition of the clinical syndrome: chest pain, hypoxemia, fever, tachypnea, and infiltrate(s) on CXR. Hematologists should also consider asthma as a contributing factor to acute chest syndrome. Asthma is a common disease in African American children, and asthma has been associated with an increased frequency of VOC as well as an increased incidence of acute chest syndrome in children with sickle cell disease.

  4. If a child with sickle cell disease needs a transfusion, the physician should communicate with the local blood bank. Children with sickle cell disease are at higher risk for developing allo-antibodies to C, E, and Kell. Extended typing and transfusion with partially phenotypically matched red blood cells is usually possible.

  5. Close to 25% of sickle cell anemia patients will have a clinical stroke by age 40; the peak incidence is in the late childhood and early teenage years. Strokes often occur in the setting of acute chest syndrome and VOC. Another third of patients may have silent cerebral infarcts.

  6. Even though infarcts without a focal correlating neurological finding are deemed “silent,” affected children still may have long-term sequelae. Almost 75% of children with silent infarcts may have cognitive deficits or receive special education services in school. Families, medical teams, and educators need to communicate with each other to address these children’s needs.

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