Clinical manifestations

Clinical symptoms vary tremendously between patients with SCD for several reasons. The disease is more severe in patients with HbSS or HbS P0-thalassaemia than in those with HbS P+-thalassaemia or HbSC disease. The Arab-Indian haplotype produces a less severe disease than the African haplotypes. The

Biconcave Reticulocyte disc

Unobstructed flow

Irreversibly Initiation of sickled cell i vaso-occlusion

Propagation

Red cell CD36 ?

Biconcave Reticulocyte disc

Unobstructed flow

Red cell CD36 ?

avß3 gp VCAM-1 ? Ib-IX-V Endothelial cell

Vaso-occlusion and red cell sickling

Sickle cells

Sickle cells

Figure 7.2 Endothelial red cell adhesion and vaso-occlusion in sickle cell disease. Adhesive sickle reticulocytes initiate vaso-occlusion by becoming attached to the endothelium of vessel walls. Thereafter, poorly deformable red cells begin to accumulate behind the site of adhesion, ultimately resulting in an occluded vascular segment containing many sickled red cells. The inset shows the site of red cell attachment to an endothelial cell and several adhesion mechanisms that could participate in the vaso-occlusive process. On the red cell, the relevant adhesion receptors include CD36, co-inheritance of one or two a-gene deletions also modifies the clinical picture. The high HbF level observed in hereditary persistence of fetal haemoglobin (HPFH) is associated with very mild disease. However, for poorly recognized reasons, the disease severity varies enormously even within the subgroup of patients with HbSS.

In countries with inadequate healthcare, SCD is associated with high mortality in the first 3 years of life as a result of sepsis and splenic sequestration. In the developed world, the typical patient with SCD has moderately severe anaemia, leads a relatively normal life interrupted by 'crises' as a result of vaso-occlusion, and has a life expectancy of over 45 years.

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