thalassemia

hereditary disorder of hemoglobin synthesis

symptoms: anemia, jaundice, splenomegaly, hemosiderosis, cardiomegaly, rodent-like face, big head
erythroid hyperplasia
=> increased medullary pressure
=> atrophy of cortical and cancellous bone
- shafts flatten then become convex
- "hair-on-end" skull
- "honeycomb trabecular bone"

In puberty, peripheral manifestations regress as inactive yellow marrow replaces active red marrow. Thus the skull, spine and pelvis demonstrate the most severe changes in adulthood.

Brian Funaki, MD - 6 February 1995
Last updated: 1 September 2006