Sickle Cell Disease
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Vivien A. Sheehan, MD, PhD
Assistant Professor of Pediatrics, Baylor College of Medicine/Texas Children’s Hematology Center, Houston, TX

Question 1 of 5

A 23-year-old African American woman with homozygous hemoglobin SS (HbSS) disease presents to initiate care. She has been on monthly simple transfusion therapy since she was found to have a transcranial Doppler (TCD) velocity of 220 cm/sec in her right middle cerebral artery at age 7 years. She has not had an overt stroke, and her last magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) evaluation, at age 16, was negative for moyamoya or significant stenosis. There were mild ischemic changes in the frontal lobe consistent with silent infarcts. She has no allo- or autoantibodies, and is on an iron chelator, with a ferritin level under 1000 ng/mL. She wants to know when she can discontinue transfusion therapy.

How should the physician respond to this patient's question regarding stopping transfusion therapy?

She can stop now, as she is an adult and is no longer at risk for stroke

She can safely switch to hydroxyurea at any time, as it has been shown conclusively to prevent stroke with efficacy equal to that of chronic transfusions

She can undergo dural inversion therapy and discontinue transfusions

She should continue transfusion therapy indefinitely

Hospital Physician: Hematology/Oncology. 2017 January;12(1)

This quiz is not accredited for CME.

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