Learning objectives
(1) to understand the different manifestations of thalassemias,
(2) to illustrate the skeletal and non-skeletal features of thalassemias through an iconography of our own practice in order to highlight the contribution of imaging to their diagnosis.
Background
Thalassemias are chronic microcytic anemias,
of an inherited autosomal recessive nature,
characterized by a defective synthesis of one of the α or β chains of hemoglobin and therefore ineffective hematopoiesis [1].
They were first described by Cooley et al.
in 1927 [2].
They are common in the Mediterranean Basin,
the Middle East and the Far East [3].
In all thalassemias,
clinical features that result from anemia,
transfusional,
and absorptive iron overload are similar but vary in severity.
Major ß-thalassemia or Cooley anemia is characterized by...
Findings and procedure details
We looked at the results of the different imaging modalities (Xray,
ultrasound,
CT scan and MRI) of patients with thalassemias and tried to use them to illustrate the skeletal and non-skeletal features of thalassemias.
I. Skeletal manifestations of thalassemia
The radiographic features of ß-thalassemia are due in large part to bone marrow hyperplasia that leads to various skeletal manifestations.
The skeletal system's response to this bone marrow proliferation consists of bone marrow expansion,
bone cortical thinning,
and bone resorption.
All these phenomena lead to a...
Conclusion
Manifestations of thalassemias are rich and variable.
Imaging contributes effectively to optimizing the care of thalassemic patients by allowing an early and accurate diagnosis of the different thalassemia’s features.
The choice of the modality is conditioned by the clinical examination.
X-ray is generally adequate in defining osseous abnormalities.
CT and MRI techniques can document the iron oveload,
extramedullary hematopoiesis,
and bone marrow changes.
References
Tunacõ M,
Tunacõ A,
Engin G,
Özkorkmaz B,
Dinçol G,
Acunas G,
Acunas B,
Imaging features of thalassemia,
Eur.
Radiol.,1999; 9:1804-1809.
Cooley TB,
Witwer ER,
Lee P,
Anemia in children with splenomegaly and peculiar changes in the bones.
Am J Dis Child,
1927; 34: 347.
Ben Brahim O,
Ladeb S,.
Ladeb MF,
Ben Hamouda M,
Imagerie des manifestations osseuses de la thalassémie,
Feuillets De Radiologie,
2003,
43(2):137-141.
Izadyar S,
Fazeli M,
Izadyar M,
Salamati P,
Gholamrezanezhad A.
Bone mineral density in adult patients with major...