Treatment of Sickle Cell Pain

Karen F. Marlowe, PharmD., Michael F. Chicella, PharmD.


Pharmacotherapy. 2002;22(4) 

In This Article

Pathophysiology and Presentation

Sickle cell pain is the result of tissue ischemia caused by occlusion of vascular beds with sickled erythrocytes. Under hypoxic conditions, hemoglobin S in the erythrocytes of patients with sickle cell disease reacts to form rigid polymers, leading to sickling or distortion of erythrocytes. Concurrently, polymorphonuclear leukocytes release cytokines, leading to expression of adhesion molecules on the vascular endothelium. These receptors bind passing erythrocytes.[9] The abnormal erythrocytes adhere to the vascular endothelium, which narrows the vascular lumen, traps more cells, and creates a hypoxic environment, encouraging further sickling.[10] In addition, interaction between erythrocytes and endothelium leads to release of vasoconstrictors, further inhibiting regional blood flow.[11] This cycle results in occlusion of certain vascular beds, most commonly in vertebrae, femur, hip, and ribs. Regional hypoxia can cause serious consequences, such as vascular necrosis of bone marrow or tissue infarcts. Acute inflammatory response to tissue injury may contribute to acute bone pain.[6,12]


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