CEREBROVASCULAR RESERVE IN SICKLE CELL DISEASE ASSESSED WITH PERFUSION MAGNETIC RESONANCE IMAGING L. VÁCLAVŮ1, HJMM MUSTSAERTS1, CBLM MAJOIE1, ET VAN BAVEL1, AJ NEDERVEEN1, BJ BIEMOND1 1 Academic Medical Center, Amsterdam, The Netherlands Poster Ref: P372 Abstract identifier: EHA-3939. CONCLUSION Low CVR in SCD patients is in line with literature suggesting that maximum vasodilation has been reached in SCD1 Sickle hemoglobin in less able to carry oxygen due to this mutation. Reduced oxygen delivery causes elevated cerebral blood flow (CBF). Chronic high blood flow impairs the ability of the vessels to dilate further1. Since this was observed in SCD patients in a steady-state disease course, it is very imaginable that the CVR will become exhausted during times of increased metabolic demand (e.g. fever, infection) Impaired vessels are thought to induce Silent strokes due to local perfusion deficits. Perfusion is flow through the capillary network to deliver oxygen to the tissue. Perfusion measurements can provide local hemodynamic information CBF was significantly dependent on hematocrit CBF was significantly higher in patients with Sickle Cell Disease compared with healthy controls OBJECTIVES ACKNOWLEDGEMENTS REFERENCES 1. 2. 3. 4. Nur E et al. Blood:114:3473 - 3478 Alsop D et al. 2015 MRM:73:102-116 Petersen ET et al. 2012 ISMRM 20:472 Václavů L et al. AJNR 2016 In Press Table 1. Subject characteristics Healthy controls Sickle Cell Disease Patients n 9 25 Mean age ± SD 33 ± 14y 33 ± 12y Males/Females 5/4 16/9 Ethnicity Dutch Antilles and Africa Dutch Antilles and Africa Haemoglobin HbAA/HbAS HbSS or HbSß0 Haematocrit 39 ± 4 % 25 ± 5 % CONTACT INFORMATION Lena Václavů Department of Radiology, Academic Medical Center Meibergdreef 9 , 1105 AZ, Amsterdam, The Netherlands [email protected] Lena Vaclavu Cerebral blood flow was significantly higher in patients with Sickle Cell Disease, and was dependent on blood hematocrit. Thus, severity of anemia was a strong indicator of resting CBF. Moreover, The higher the baseline CBF, the lower the cerebrovascular reserve (CVR) in response to vasodilation. The CVR in patients was approximately one-third of that in controls. John C Wood, Children’s Hospital Los Angeles, LA, USA Red blood cells and iron - Clinical 1 CBF increases due to vasodilation induced by acetazolamide were significantly lower in patients compared to control, indicated by red arrows Esben Thade Petersen, Danish Research Centre for Magnetic Resonance, Hvidore Hospital 372-P Baseline cerebral blood flow was a significant predictor of cerebrovascular reserve Procedure • IRB approved study • All participants underwent MRI at 3T (Ingenia, Philips Healthcare, Best, The Netherlands) • Sequences included: T2-weighted and FLAIR anatomical scans, Arterial Spin Labelling (ASL) perfusion MRI2, and T1blood for improved quantification of CBF3,4 Data processing • Registration of perfusion images to population-based average • CBF quantification • CVR calculated from increase in CBF induced by acetazolamide (vasodilator) CVR could potentially be a cerebrovascular biomarker in SCD for treatment stratification in the future MRI-based cerebral perfusion measurements with acetazolamide show robust CVR results and indicate that cerebral hemodynamics may be impaired in SCD Our aim was to gain understanding of cerebral hemodynamics in SCD by using perfusion MRI. Our objective was to investigate the acute response of cerebral blood flow by inducing vasodilation in patients with SCD. This is called cerebrovascular reserve (CVR). We hypothesised that patients would have impaired vascular reactivity due to pre-existing increased vasodilation and elevated resting CBF. METHODS DOI: 10.3252/pso.eu.21EHA.2016 Sickle Cell Disease (SCD) is an inherited chronic form of anaemia, caused by a hemoglobin mutation. Poster presented at EHA 21 on: 10th June 2016 RESULTS 21eha INTRODUCTION Academic Medical Center
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