= autosomal dominant congenital hemolytic anemia

Age: anemia begins in early infancy to late adulthood

• rarely severe anemia; jaundice; spherocytes in peripheral smear
• bone changes rare (← mild anemia); long bones rarely affected
• widening of diploe with displacement + thinning of outer table
• hair-on-end appearance

Rx: splenectomy corrects anemia even though spherocytemia persists

• improvement in skeletal changes following splenectomy