Unusually High Prevalence of Stroke and Cerebral Vasculopathy in Hemoglobin SC Disease: A Retrospective Single Institution Study

Bindu Kanathezhath Sathi; Yilin Yoshida; Michael Raymond Weaver; Lila S Nolan; Barbara Gruner; Vinod Balasa; Talissa Altes; Carlos Leiva-Salinas

doi:10.1159/000519360

Unusually High Prevalence of Stroke and Cerebral Vasculopathy in Hemoglobin SC Disease: A Retrospective Single Institution Study

Acta Haematol. 2022;145(2):160-169. doi: 10.1159/000519360. Epub 2021 Nov 8.

Authors

Bindu Kanathezhath Sathi^{1

2}, Yilin Yoshida³, Michael Raymond Weaver^{2

4}, Lila S Nolan^{2

5}, Barbara Gruner², Vinod Balasa¹, Talissa Altes^{2

6}, Carlos Leiva-Salinas⁶

Affiliations

¹ Pediatric Hematology Oncology, Valley Children's Hospital, University of San Francisco-Fresno Program, Madera, California, USA.
² Department of Child Health, University of Missouri, Columbia, South Carolina, USA.
³ Department of Internal Medicine, Tulane University, New Orleans, Louisiana, USA.
⁴ University of South Florida, Tampa, Florida, USA.
⁵ Department of Pediatrics, Washington University, St. Louis, Missouri, USA.
⁶ Department of Radiology, University of Missouri, Columbia, South Carolina, USA.

Abstract

Introduction: Unlike homozygous hemoglobin SS (HbSS) disease, stroke is a rare complication in hemoglobin SC (HbSC) disease. However, recent studies have demonstrated a high prevalence of silent stroke in HbSC disease. The factors associated with stroke and cerebral vasculopathy in the HbSC population are unknown.

Methods: We conducted a retrospective study of all patients with sickle cell disease treated at the University of Missouri, Columbia, over an 18-year period (2000-2018). The goal of the study was to characterize the silent, overt stroke, and cerebral vasculopathy in HbSC patients and compare them to patients with HbSS and HbS/β thalassemia1 (thal) in this cohort. We also analyzed the laboratory and clinical factors associated with stroke and cerebral vasculopathy in the HbSC population.

Results: Of the 34 HbSC individuals, we found that the overall prevalence of stroke and cerebral vasculopathy was 17.7%. Only females had evidence of stroke or cerebral vasculopathy in our HbSC cohort (33.3%, p = 0.019). Time-averaged means of maximum velocities were lower in the HbSC group than the HbSS group and did not correlate with stroke outcome. Among HbSC individuals, those with stroke and cerebral vasculopathy had a marginally higher serum creatinine than those without these complications (0.77 mg/dL vs. 0.88 mg/dL, p = 0.08). Stroke outcome was associated with recurrent vaso-occlusive pain crises (Rec VOCs) (75 vs. 25%, p = 0.003) in HbSC patients. The predominant cerebrovascular lesions in HbSC included microhemorrhages and leukoencephalopathy.

Conclusion: There is a distinct subset of individuals with HbSC who developed overt, silent stroke, and cerebral vasculopathy. A female predominance and association with Rec VOCs were identified in our cohort; however, larger clinical trials are needed to identify and confirm specific clinical and laboratory markers associated with stroke and vasculopathy in HbSC disease.

Keywords: Cerebral vasculopathy; Hemoglobin SC disease; Silent cerebral infarction; Stroke.

MeSH terms

Anemia, Sickle Cell* / complications
Anemia, Sickle Cell* / epidemiology
Female
Hemoglobin SC Disease* / complications
Hemoglobin SC Disease* / epidemiology
Humans
Prevalence
Retrospective Studies
Stroke* / diagnosis
Stroke* / epidemiology
Stroke* / etiology