Hereditary Spherocytosis, Pediatric

Hereditary spherocytosis (HS) is the most common red blood cell (RBC) membrane disorder causing hemolytic anemia.

Hemolytic anemia with shortened RBC lifespan due to an inherent defec...

Fatigue (symptom secondary to anemia)

Jaundice, scleral icterus, dark urine (signs of hemolysis)

Phototherapy required in newborn period (50% of cases): hyperbilirubinemia due to hemoly...

Folic acid supplement

Penicillin prophylaxis (if splenectomized)

Pneumococcal, meningococcal, and Haemophilus influenza B vaccines (prior to splenectomy)

Physical exam

• Check for splenomegaly.

• Follow growth curves closely.

CBC with reticulocyte count as needed: if patient develops fatigue, pallor, or increased jaundice especially in setting of...

Barcellini W, Bianchi P, Fermo E, et al. Hereditary red cell membrane defects: diagnostic and clinical aspects. Blood Transfus.  2011;9(3):274–277. [View Abstract on OvidInsigh...

55995005 Hereditary spherocytosis (disorder)

25266006 Hereditary spherocytosis due to spectrin deficiency (disorder)

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Q: Will my child require blood transfusions?

A: It depends on the clinical severity of your child’s disease.

Q: If a parent has HS, how should the newborn be followed?

A: The infant has a 50% chance ...