From the Guidelines
Sickle cell crises are acute complications of sickle cell disease that include several distinct types, primarily vaso-occlusive crisis, acute chest syndrome, splenic sequestration crisis, aplastic crisis, hemolytic crisis, and stroke. The main types of sickle cell crises are:
- Vaso-occlusive crisis (also called pain crisis), which is the most common and involves severe pain when sickled red blood cells block blood vessels
- Acute chest syndrome, characterized by chest pain, fever, and respiratory symptoms due to lung involvement, as described in the american society of hematology 2020 guidelines for sickle cell disease: transfusion support 1
- Splenic sequestration crisis, where the spleen rapidly enlarges by trapping blood cells
- Aplastic crisis, marked by a temporary halt in red blood cell production often triggered by infections
- Hemolytic crisis, involving accelerated destruction of red blood cells
- Stroke, which occurs when blood flow to the brain is blocked Other crisis types include priapism (painful, prolonged erection), acute kidney injury, and hepatic crisis affecting liver function. These crises require prompt medical attention and are typically managed with pain medication, hydration, oxygen therapy, antibiotics for infections, and sometimes blood transfusions, such as simple or exchange transfusion for acute chest syndrome, as mentioned in the american society of hematology 2020 guidelines for sickle cell disease: transfusion support 1. Prevention strategies include staying hydrated, avoiding extreme temperatures, managing stress, and taking medications like hydroxyurea that reduce crisis frequency. The clinical spectrum of acute chest syndrome is variable, and although there are no specific markers of disease severity, a significant decline in the hemoglobin concentration and/or oxygen saturations (SpO2 # 94% or several percentage points below the patient’s baseline) can suggest severe disease, as noted in the american society of hematology 2020 guidelines for sickle cell disease: transfusion support 1.
From the Research
Types of Sickle Cell Crises
There are several types of sickle cell crises, including:
- Vaso-occlusive crisis: the most common type, characterized by the occlusion of small blood vessels, leading to tissue ischemia and pain 2, 3, 4, 5
- Haemolytic crisis: a less common type, characterized by the rapid destruction of red blood cells, leading to anemia and other complications 2
- Sequestration crisis: a type of crisis where red blood cells become trapped in the spleen or liver, leading to a decrease in blood cell count 2
- Infarctive crisis: a type of crisis where tissue damage occurs due to the lack of blood flow, leading to infarction of organs such as the spleen or kidneys 2
- Aplastic crisis: a type of crisis where the bone marrow fails to produce new red blood cells, leading to anemia and other complications 2
- Bone pain crisis: a type of crisis characterized by severe pain in the bones, often due to bone infarction or ischemia 2
Prevalence of Sickle Cell Crises
The prevalence of sickle cell crises varies, with vaso-occlusive crisis being the most common, accounting for 91.6% of all crises 2. Haemolytic crisis is the least common, accounting for 0.5% of all crises 2.
Demographic Factors
Demographic factors such as age and sex can influence the type and frequency of sickle cell crises. Young adults (20-29 years) are more likely to experience vaso-occlusive crises, while children are less likely to experience crises 2. Females are more likely to experience vaso-occlusive crises than males, but the relationship between sex and crisis type is not significant 2.