αLEPRAin trans to a null SPTA1 allele (leading to a total α-spectrin production of about 8%) has been shown to cause severe autosomal recessive HS, with anemia and jaundice that resolve with splenectomy (Wichterle et al., 1996; Delaunay et al., 2004). The gene discussed is SPTA1; the disease is anemia (phenotype).