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 #SickleCell #Crisis #Features #Signs #Symptoms
