
2026 Clinician Corner - Positive neurological outcome after acute encephalopathy and multifocal stroke after early red blood cell exchange transfusion in a patient with hemoglobin SC sickle cell disease
Abstract
Sickle cell disease is a genetic disorder characterized by the presence of abnormal hemoglobin, leading to various complications, including vaso-occlusive crises and organ damage. While much research has focused on the homozygous hemoglobin SS genotype, less is known about the heterogeneous hemoglobin SC genotype (HbSC), particularly in adults. This case report details the presentation, clinical course, and management of a 27-year-old African American man with HbSC sickle cell disease, who presented with severe acute encephalopathy and multifocal acute ischemic stroke and had a positive neurological outcome due to early red blood cell exchange transfusion. The case underscores the need for vigilant clinical care considerations into the unique challenges for adults with HbSC disease.
Keywords: HbSC sickle cell disease; neurocritical care; red blood cell exchange transfusion
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Faculty credentials/disclosure
The planners and faculty for this activity have no relevant financial relationships to disclose. The patient consented to the publication of this report.
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Expiration date:
Credit eligibility for this article is set to expire on July 1, 2027.
Learning Objectives
After completing the article, the learner should be able to:
Recognize that hemoglobin SC sickle cell disease can lead to various neurological complications with differing severity.
Understand the role of red blood cell exchange transfusion in management of patients with hemoglobin SC sickle cell disease.
Key Points
HbSC sickle cell disease can cause a myriad of neurological complications, including acute encephalopathy, acute ischemic stroke, hemorrhagic stroke, cerebral fat embolism, cerebral venous sinus thrombosis, moyamoya syndrome, and posterior reversible encephalopathy syndrome.
Early consideration of red blood cell exchange transfusion with a goal to achieve HbS levels below 10% to 20% in patients with HbSC sickle cell disease can improve the clinical outcome in suspected sickle cell crisis.
Ekta Haria, PA-C1, Sidarrth Prasad, MBBS2, and Jia W. Romito, MD3
1 Division of Neurocritical Care, Department of Neurology and Neurological Surgery, University of Texas Southwestern Medical Center; Department of Neurology, Texas Health Presbyterian Hospital; and Peter O'Donnell Brain Institute, University of Texas Southwestern Medical Center, Dallas, Texas, USA
2 Department of Neurology, University of Texas Southwestern Medical Center, Dallas, Texas, USA
3 Division of Neurocritical Care, Department of Neurology and Neurological Surgery, University of Texas Southwestern Medical Center; Department of Neurology, Texas Health Presbyterian Hospital; Peter O'Donnell Brain Institute, University of Texas Southwestern Medical Center; Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Dallas, Texas, USA
The planners and faculty for this activity have no relevant financial relationships to disclose. The authors report no funding. The patient consented to publication of this case report.
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AMA PRA Category 1 Credit™
The A. Webb Roberts Center for Continuing Medical Education of Baylor Scott & White Health designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
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Available Credit
- 1.00 American Board of Internal Medicine (ABIM) MOC Part 2Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.00 MOC points in the American Board of Medicine’s (ABIM) Maintenance of Certification (MOC) program. It is the CME activity provider’s responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit.
- 1.00 American Board of Surgery (ABS) Accredited CME
Successful completion of this CME activity enables the learner to earn credit toward the CME requirement of the American Board of Surgery’s Continuous Certification program. It is the CME activity provider's responsibility to submit learner completion information to ACCME for the purpose of granting ABS credit.
- 1.00 AMA PRA Category 1 Credit™The A. Webb Roberts Center for Continuing Medical Education of Baylor Scott & White Health is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
- 1.00 Attendance

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