hematology and oncology
Sickle Cell Disease
Key Points
Patients homozygous for hemoglobin S have an abnormal beta chain, resulting in fragile, relatively inflexible red blood cells that can plug capillaries, causing tissue infarction and are prone to hemolysis, causing anemia. Patients have various acute exacerbations including painful crisis, sequestration crisis, aplastic crisis, and acute chest syndrome. Long-term consequences include pulmonary hypertension, chronic kidney disease, stroke, aseptic necrosis, and increased risk of infection. Diagnose using hemoglobin electrophoresis. For acute crises, give opioid analgesics for pain, check for worsening anemia (suggesting aplastic or sequestration crisis) and signs of acute chest syndrome or infection, restore normal intravascular volume using 0.9% saline and then give maintenance fluids. Prevent infection by using vaccinations and prophylactic antibiotics; limit painful crises and risk of disease complications by givinghydroxyurea. 