Description

Hereditary spherocytosis may show a spectrum and clinical and laboratory severity.


Patient selection: hereditary spherocytosis

 

Parameters:

(1) clinical signs and symptoms (pallor, fatigue, hemolytic anemia, splenomegaly)

(2) hemoglobin concentration (degree of anemia)

(3) reticulocytosis

 

Clinical Findings

Anemia

Reticulocytosis

Severity

mild

mild (>= 11 g/dL)

mild to moderate (<= 8%)

mild

moderate

moderate (8 to 11.5 g/dL)

moderate to high (> 8%)

moderate

severe

severe (< 8 g/dL)

high (>= 10%)

severe

 

Splenectomy may need to be performed in more severe disease. The more severe the anemia and the greater the disability the earlier this is performed.


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