Pediatric Thalassemia

  • Etiology: Erythroblastemia and marked changes in size and shape of red blood cell leads to bone marrow hyperstimulation
  • Imaging: Radiographic findings reflect increased hematopoiesis
  • Imaging Bones:
    — Generalized osteopenia with decreased trabecular prominence and widened medullary space and thin cortices and these findings are most marked in hands and femurs
    — Thickened dipoloic space in skull = hair on end appearance
    — 23% of patients greater than 10 years old have premature epiphyseal fusion
  • Imaging Chest:
    — Cardiomegaly often seen secondary to high output congestive heart failure
    — Extramedullary hematopoiesis can present as lobulated masses in mediastinum contiguous to the spine
  • Imaging Gastrointestinal: Hemosiderin is deposited in various organs particularly liver
  • DDX:
  • Complications:
  • Treatment: Splenectomy
  • Clinical:
    — Thalassemia major is a progressive anemia and jaundice that begins in the first 2 years of life with death prior to adolescence
    — Thalassemia minor has less prominent symptoms

Radiology Cases of Thalassemia

Radiograph of thalassemia in the skull
Lateral radiograph of the skull shows widening of the diploic space, thinning of the inner and outer tables of the skull, and an overall hair-on-end appearance with sparing of the occipital bone.
CXR of extramedullary hematopoesis in thalassemia
CXR AP shows diffuse osteopenia of the bones, lobulated masses projecting over the posterior portions of the ribs bilaterally, and an expanded appearance of the anterior and posterior aspects of the ribs.