What is the term for severe pain due to vaso-occlusion in sickle cell disease?

What is the term for severe pain due to vaso-occlusion in sickle cell disease?

• A. Sickle crisis
• B. Aplastic crisis
• C. Hemolytic crisis
• D. Megaloblastic crisis

Answer: (A)

Severe pain from blockage of small vessels by sickled red cells is called a sickle crisis, also described as a vaso-occlusive crisis. In sickle cell disease, hemoglobin S polymers when deoxygenated cause red cells to become rigid and misshapen, promoting adhesion to the endothelium and occluding microcirculation. This leads to tissue ischemia and the characteristic acute, often intense pain that typically affects bones and joints but can involve the chest, abdomen, or back. Triggers include dehydration, infection, acidosis, and hypoxemia, which raise the risk of a vaso-occlusive episode. Management focuses on prompt analgesia, aggressive hydration, and addressing precipitating factors. Other crises described in sickle cell disease—such as aplastic crisis from Parvovirus B19 causing temporary cessation of erythropoiesis, hemolytic crisis from increased red cell destruction, or megaloblastic crisis from folate/B12 deficits—produce different patterns and do not present with the focal painful vaso-occlusion characteristic of a sickle crisis.

Severe pain from blockage of small vessels by sickled red cells is called a sickle crisis, also described as a vaso-occlusive crisis. In sickle cell disease, hemoglobin S polymers when deoxygenated cause red cells to become rigid and misshapen, promoting adhesion to the endothelium and occluding microcirculation. This leads to tissue ischemia and the characteristic acute, often intense pain that typically affects bones and joints but can involve the chest, abdomen, or back. Triggers include dehydration, infection, acidosis, and hypoxemia, which raise the risk of a vaso-occlusive episode. Management focuses on prompt analgesia, aggressive hydration, and addressing precipitating factors. Other crises described in sickle cell disease—such as aplastic crisis from Parvovirus B19 causing temporary cessation of erythropoiesis, hemolytic crisis from increased red cell destruction, or megaloblastic crisis from folate/B12 deficits—produce different patterns and do not present with the focal painful vaso-occlusion characteristic of a sickle crisis.