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This patient with presents with acute chest syndrome in the setting of sickle cell disease. Peripheral blood smear reveals sickle cells and reticulocytes, as seen in figure C. Sickle cell anemia is an autosomal recessive hematologic disorder caused by a single amino acid replacement of glutamic acid with valine at position 6 of the beta chain. Hemoglobin S (HbS) tetramers polymerize in low O2 settings, leading to sickling of deoxygenated RBCs. Sickle cell anemia is most commonly seen in African Americans, with approximately 8% of the population carrying the trait. Painful crisis is a common complication of sickle cell disease that occurs due to occlusion of the vasculature by the sickled erythrocytes in the setting of hypoxia, dehydration, or infection. It can present as acute chest syndrome, dactylitis, or avascular necrosis. Other complications include autosplenectomy, splenic sequestration crisis, renal papillary necrosis, aplastic crisis, and osteomyelitis. Yale et al. review the approach to vaso-occlusive crises in patients with sickle cell disease. Vaso-occlusive crises are the most common reason that patients with sickle cell disease present to the emergency department. They state that vaso-occlusive crises can lead to acute pain due to ischemic tissue injury and chronic pain due to damage to bones, joints, and organs. As vaso-occlusive crises can trigger further sickling and damage, pain crises need to be diagnosed early and treated quickly. The approach to treatment includes medical management with opioids, hydration, rest, and cognitive and behavioral therapies. Other therapies include oxygen management, fluid replacement, transfusions, and hydroxyurea. Wong et al. describe the use of hydroxyurea in patients with sickle cell disease. Hydroxyurea functions by increasing the amount of red blood cell fetal hemoglobin (HbF) in patients, which is protective against sickling. Hydroxyurea is approved for treatment of adults with recurrent moderate to severe painful crises and is the only FDA approved medication that modifies the disease course in sickle cell disease. This review supported the use of hydroxyurea by validating its efficacy, effectiveness, and safety. Figures A, B, D, and E are described below. Incorrect Answers: Answer 1: Figure A depicts a bite cell, which is commonly seen in G6PD deficiency. Answer 2: Figure B shows basophilic stippling, which is seen in thalassemias, anemias of chronic disease, and lead poisoning. Answer 4: Figure D illustrates target cells, which are seen in thalassemias, as well as in hemoglobin C disease, liver disease, and asplenia. Answer 5: Figure E shows spherocytes, which are seen in hereditary spherocytosis and autoimmune hemolysis.
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