Bindu Kanathezhath Sathi1,2, Yilin Yoshida3, Michael Raymond Weaver2,4, Lila S Nolan2,5, Barbara Gruner2, Vinod Balasa1, Talissa Altes2,6, Carlos Leiva-Salinas6. 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.
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.
Authors: Lori Luchtman-Jones; Sara Pressel; Lee Hilliard; R Clark Brown; Mary G Smith; Alexis A Thompson; Margaret T Lee; Jennifer Rothman; Zora R Rogers; William Owen; Hamayun Imran; Courtney Thornburg; Janet L Kwiatkowski; Banu Aygun; Stephen Nelson; Carla Roberts; Cynthia Gauger; Connie Piccone; Theodosia Kalfa; Ofelia Alvarez; Kathryn Hassell; Barry R Davis; Russell E Ware Journal: Am J Hematol Date: 2016-02 Impact factor: 10.047
Authors: Maite E Houwing; Rowena L Grohssteiner; Marjolein H G Dremmen; Ferdows Atiq; Wichor M Bramer; Anne P J de Pagter; C Michel Zwaan; Tonya J H White; Meike W Vernooij; Marjon H Cnossen Journal: BMC Med Date: 2020-12-22 Impact factor: 8.775