Monitoring
Children who are diagnosed with hereditary spherocytosis (HS) during infancy should be monitored with blood counts. These should be performed on a routine basis every 2 to 4 months during the first year of life if they have evidence of anemia. Once a baseline hemoglobin value is determined, children with mild-to-moderate HS should be evaluated on a yearly basis to monitor blood counts, spleen size, and potential signs and symptoms of anemia.
Patients with more severe hemolysis and anemia may require more frequent monitoring. Many practitioners recommend routine ultrasonographic screening for gallstones every 3 to 5 years after age 5 years, or sooner if symptomatic. No formal studies have established whether this is of benefit.[14]
During an acute exacerbation of symptoms, the patient should be evaluated and a complete blood count (CBC) and reticulocyte count performed to be certain that the hemoglobin concentration has not dropped to levels requiring a blood transfusion or other supportive care.
Children or adults with HS who have a known exposure to parvovirus infection (and who have not had a previous documented parvovirus infection or positive parvovirus serology) should be evaluated with a CBC and reticulocyte count to be certain that there is no evidence for an aplastic crisis. It may also be prudent to screen parvovirus titers in very young children. Serologic conversion following subclinical infection can be documented and it may be possible to reassure the family regarding the risk of aplastic crisis.
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