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Splenic sequestration crisis is a life-threatening illness common in pediatric patients with homozygous sickle cell disease and beta thalassemia.Up to 30% of these children may develop splenic sequestration crisis with a mortality rate of up to 15%.
Spleens are also removed for therapeutic purposes in conditions like hereditary spherocytosis, trauma to the spleen, and autosplenectomy caused by sickle cell anemia. Other causes are radiation therapy involving the spleen, such as that used to treat Hodgkin lymphoma. Howell–Jolly bodies inside of two normoblasts (center) in bone marrow.
Treatment is the same as for patients with sickle cell disease. Patients may receive hydroxyurea to induce the protective effects of increased fetal hemoglobin production. . They may also benefit from blood transfusions especially during vaso-occlusive cris
The acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in people with sickle cell anemia. This condition commonly manifests with a new opacification of the lung(s) on a chest x-ray .
A single point mutation in this polypeptide chain, which is 147 amino acids long, results in the disease known as Sickle Cell Anemia. [18] Sickle-cell anemia is an autosomal recessive disorder that affects 1 in 500 African Americans, and is one of the most common blood disorders in the United States. [17]
Thalassemias are inherited blood disorders that result in abnormal hemoglobin. [7] Symptoms depend on the type of thalassemia and can vary from none to severe. [1] Often there is mild to severe anemia (low red blood cells or hemoglobin) as thalassemia can affect the production of red blood cells and also affect how long the red blood cells live. [1]