Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Thalassemia (beta thalassemia major)
- Radiologic Findings
- Chest radiograph shows marked ballooning of ribs with 'rib-within-a-rib' appearance. CT shows diffuse osteopenia with ballooning, cortical thinning, and erosion of the ribs. Soft tissue inside the ribs and paravertebral region indicate extramedullary hematopoiesis.
High-attenuation of liver (95HU), myocardium (56 HU), abdominal lymph nodes (190 HU) are the findings of hemochromatosis caused by prolonged RBC destruction. Splenectomy and polycystic kidney are additional findings.
- Brief Review
- Thalassemia, which is most prevalent in Mediterranean region, is a kind of chronic, inherited, microcytic anemia characterized by defective hemoglobin synthesis and ineffective erythropoiesis. In all thalassemias, clinical features that result from anemia, transfusional, and absorptive iron overload are similar but vary in severity. The radiographic features of beta thalassemia are largely due to marrow hyperplasia. Markedly expanded marrow space lead to various skeletal manifestations involving the spine, skull, facial bones, and ribs. Extramedullary hematopoiesis, hemosiderosis, and cholelithiasis are among the non-skeletal manifestations of thalassemia. The skeletal X-ray findings show characteristics of chronic overactivity of the marrow.
Sickle cell anemia, which is another type of hereditary anemia, may also cause ballooning of ribs and findings of extramedullary hematopoiesis. However, its most common thoracic manifestation is pulmonary infection and complex pulmonary abnormalities causing acute chest syndrome.
- References
- 1. Tunaci A et al. Imaging features of thalassemia. Eur Radiol 1999;9(9):1804-9.
2. Lonergan GL et al. Sickle cell anemia. RadioGraphics 2001;21(4);971-94
- Keywords
- Thalassemia, Thalassemia (beta thalassemia major), Bone, Extramedullary hematopoiesis,