The two primary causes of death from sickle cell disease in the first decade of life are which of the following?

b. Splenic sequestration crisis

Sickle cell disease and thalassemia are the principal hemoglobinopathies that cause clinically important disease. Clinical symptoms are directly dependent upon the amount of HgbS present, and this is variable. Patients who are homozygous for HgbS usually have small sequential splenic infarcts as a result of microvascular occlusion and the spleen usually becomes small, fibrotic, and dysfunctional by the age of 5 years. Splenic sequestration crisis is a cause of anemia that can be acute and life-threatening. Sepsis associated with functional asplenia can be lethal as well. These are the two primary causes of death in sickle cell disease during the first decade of life.