Page 115 - Essential Haematology
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Chapter 7  Genetic disorders of haemoglobin   /  101





























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                                Figure 7.15   Sickle cell anaemia.
                        (a)  Radiograph of the pelvis of a
                      young man of West Indian origin
                      which shows avascular necrosis
                      with fl attening of the femoral
                      heads, more marked on the right
                      hip, coarsening of the bone
                      architecture and cystic areas in
                      the right femoral neck caused by
                      previous infarcts.  (b)  Coronal hip
                      MRI image revealing established
                      osteonecrosis of femoral heads
                      bilaterally (yellow arrow) with
                      crescentric sclerotic margin (blue
                      dot) as a consequence of sickle
                      cell. (Courtesy of Dr A. Malhotra.)     (b)



                                                                    Other  c linical  f eatures
                          Haemolytic  c rises
                       These are characterized by an increased rate of   Ulcers of the lower legs are common, as a result of

                      haemolysis with a fall in haemoglobin but rise  vascular stasis and local ischaemia (Fig.  7.17 ). Th e
                      in reticulocytes and usually accompany a painful  spleen is enlarged in infancy and early childhood but
                      crisis.                                   later is often reduced in size as a result of infarcts
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