Sickle Cell Anaemia


Sickle cell anaemia is an autosomal recessive genetic disease leading to production of a defective form of haemoglobin, haemoglobin S (HbS) causing sickle shaped erythrocytes. The major consequence of these sickled erthrocytes is that they much less deformable causing obstruction in the microcirculation.

Manifestations in the spleen include splenomegaly in childhood, with splenic infarction and autosplenectomy in adults.

Skeletal manifestations include avascular necrosis of the femoral head and H -shaped vertebrae.

This AXR shows massive splenomegaly and bilateral femoral head AVN.

Reference: eMedicine

Credit: Dr Ian Bickle