Features of a Sickle Cell Crisis
Sickle-cell disease - an autosomal recessive blood disorder.
Characterized by red blood cells that adopt an abnormal, rigid, sickle shape.
These cells can go on to block blood vessels leading to a "sickle cell crisis".
Crises can be precipitated by a variety of factors and are usually associated with severe pain, lasting from 5-7 days.
Sickle cell disease almost exclusively affects black individuals.
The underlying pathophysiology of the crisis (4 different mechanism exist):
1) Vaso-ocdusion- Obstruction of capillaries leading to ischaemia.
2) Aplasia - Acute worsening of the baseline anaemia. Can be triggered by Parvovirus B 19, a virus that invades & destroys red blood cell precursors).
3) Splenic Sequestration - Major sequestration of sickle cells in the spleen.
4) Haemolysis - An accelerated drop in haemaglobin levels. (Accelerated red blood cell breakdown - more in patients with co-existent G6PD deficiency
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