Part 3: Hemoglobinopathies
Katharine Batt, MD, MSc, and Thomas Reske, MD
Dr. Batt is a fellow in Hematology/Oncology, Mount Sinai Hospital New York, NY, and Dr. Reske is a fellow in Hematology/Oncology, Boston University Medical Center, Boston, MA
Question 2
A 24-year-old man with HbSS is found in a shopping mall with facial droop and complaining of headaches and is transferred to the nearest ED. A family member accompanies the patient and gives a recent history of joint pain exacerbation prior to onset of his neurologic symptoms. Review of his prior medical chart in the ED reveals that he has had frequent ED visits for increased sickle cell pain and an admission for crisis 6 months ago. A computed tomography scan of the head is performed and reveals no brain bleed. Magnetic resonance imaging is performed and is consistent with an ischemic lesion in his brain. He is alert and responsive on examination but complains of 8/10 spine pain. There is no evidence of gross bleeding or bruising. Vitals signs include a blood pressure of 182/89 mm Hg, temperature of 37.9ºC, heart rate of 115 bpm, respiratory rate of 23 breaths/min, and oxygen saturation on room air of 92%. His hemoglobin on admission is 8.7 mg/dL (baseline hemoglobin is 9 g/dL). What sickle cell-related treatment has shown superior outcome in the acute management of stroke?
