A painful sickle cell crisis (or vaso-occlusive crises) is the commonest manifestation of SCD and is chracterised by recurrent episodes of acute, severe pain from tissue ischemia as a result of vaso-occlusion (1).
Microvascular occlusion can affect any organ in the body but it is particularly common in deep muscle, periosteum and in bone marrow (3):
In males the age is important, with clustering of events around puberty, and correlates with an increase in the haemoglobin levels, to the extent where some centres may venesect patients. In women there is an association with the first week post menstruation
An association between frequent episodes of acute pain and the following factors has been observed:
In sickle cell anemia patients older than 20 years, an increased frequency of pain is associated with an early death (4).
Reference:
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