A 9-year-old boy with sickle cell anemia (HbSS) is brought to the emergency department for a prolonged, painful erection lasting over 5 hours. His parents have tried supportive measures at home without success. Which of the following is the most appropriate initial management step?
A 17-year-old female presents with fever, confusion, and petechiae. Laboratory studies reveal a hemoglobin of 8 g/dL with schistocytes on the peripheral smear, a platelet count of 15 × 10⁹/L, and elevated creatinine. A severe deficiency of which of the following is the most likely underlying pathophysiologic mechanism?
A healthy, full-term infant is seen at his 2-month well-child visit. His parents are concerned because he seems paler than he was at birth. A complete blood count shows a hemoglobin of 11.2 g/dL. What is the primary mechanism responsible for this “physiologic anemia of infancy”?
A 10-year-old female with stage 4 chronic kidney disease presents with fatigue and pallor. Her lab work shows a normocytic, normochromic anemia with a low reticulocyte count. Her hemoglobin is 9.8 g/dL. Which of the following is the predominant cause of her anemia?
A 14-year-old male with a history of mild macrocytic anemia, jaundice, and splenomegaly is diagnosed with a congenital dyserythropoietic anemia (CDA). His bone marrow shows erythroid hyperplasia with 15% binucleated erythroblasts. Red blood cell membrane protein analysis by SDS-PAGE is ordered. Which finding would be most consistent with congenital dyserythropoietic anemia type II?
A 6-year-old boy with parental consanguinity has recurrent epistaxis and gum bleeding since infancy. Platelet count and morphology are normal, but aggregation studies show absent response to ADP, epinephrine, collagen, and thrombin, with normal ristocetin-induced aggregation. What is the most likely diagnosis?
A pediatrician is using a diagnostic algorithm for a 3-year-old child with anemia. The initial labs show a normocytic anemia and a low reticulocyte count. Which of the following conditions is a primary consideration in this category?
A 14-year-old male with sickle cell anemia (HbSS) and a history of frequent vaso-occlusive crises presents with a new limp and persistent pain in his right hip. Physical exam reveals limited range of motion. An MRI is ordered. Which of the following is the most likely diagnosis?
A 16-year-old male with a recently diagnosed aplastic anemia develops intermittent dark-colored morning urine, abdominal pain, and evidence of intravascular hemolysis. Flow cytometry is performed on his granulocytes. Which finding would be diagnostic for the suspected underlying diagnosis?
A 12-year-old female of African descent is found to have elliptocytes on a peripheral blood smear during a workup for an unrelated issue. She is asymptomatic with a normal complete blood count. Her father has a similar finding on his blood smear. This is most characteristic of which condition?
