What is hereditary spheocytosis?

Abnormality in gene of spectrin (ankyrin protein band 3.0 and 4.1) Red cell is smaller and more dense than usual, tx folic acid and when necessary splenectomy, other examples are hereditary elliptocytosis (mutation in spectrin/ankyrin/band 3/band 4.1/4.2 defects impair elastic deformability of RBC lead to spherocytic shape of RBC (not biconcave disc) We see MCHC >36 (CBC), smear see spheocytes and some polychromatophilia (reticulocytosis), increased LDH and bilirubin, decreased haptoglobin, elevated reticulocyte count (in hemolysis testing), Coombs test negative (not autoimmune) Confirmatory test (EMA binding (eosin-5-maleimide, binds membrane proteins of RBC), or osmotic fragility test (OFT)