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Victor C. Urrutia, MD, FAHA Assistant Professor Director, The Johns Hopkins Hospital Stroke Center

Sickle Cell Disease and Stroke. Victor C. Urrutia, MD, FAHA Assistant Professor Director, The Johns Hopkins Hospital Stroke Center Department of Neurology Johns Hopkins University School of Medicine vurruti1@jhmi.edu. Objectives. Review risk of stroke in the sickle cell disease population

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Victor C. Urrutia, MD, FAHA Assistant Professor Director, The Johns Hopkins Hospital Stroke Center

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  1. Sickle Cell Disease and Stroke Victor C. Urrutia, MD, FAHA Assistant Professor Director, The Johns Hopkins Hospital Stroke Center Department of Neurology Johns Hopkins University School of Medicine vurruti1@jhmi.edu

  2. Objectives • Review risk of stroke in the sickle cell disease population • Review treatment, acute and prevention, for sickle cell disease.

  3. Disclosures • Atrial fibrillation advisory board (1/30/12). Janssen Pharmaceuticals • Johns Hopkins Hospital site PI for DIAS 4 (Lundbeck) and POINT (NINDS) • PI for SAIL ON, funded by Genentech • I will mention off label use of approved medications

  4. Introduction • Stroke is common in Sickle Cell Disease • 11% of patients with HbSS suffer a stroke by age 20, 15% by age 30, 24% by age 45 • Silent stroke/cognitive deficit in 5 to 9 times as many patients • One of every 600 African Americans in the US have SCD

  5. Epidemiology • The Cooperative Study of Sickle Cell Disease, a prospective cohort of 4000 children and adults • 3.8% had a history of stroke • Hemorrhagic stroke was more frequent in young adults. 20-29 years, 440 per 100,000 person • The Hemorrhagic stroke rate in African Americans, in the Northern Manhattan Stroke Study , was 14 per 100,000

  6. Epidemiology • Incidence of ischemic stroke in African Americans • 270 per 100,000 in 35-64 year olds • 1500 per 100,000 in 65-74 year olds • Incidence of ischemic stroke in SCD • 740 per 100,000 in 35-64 year olds • 3500 per 100,000 in >65 year olds • Stroke recurs in 67% of untreated children within 24 months

  7. Age of First CVA by Hb Genotype Ohene-Frempong K et al. Blood 1998;91:288-94.

  8. Incidence of Stroke in SCD California discharge database 1998-2007

  9. Pathophysiology Steinberg, MH. Management of Sickle Cell Disease. New Engl J Med 2009, 340:13;1021-1031

  10. Risk Factors • Risk Factors for ischemic stroke in adults • HbSS • Increasing age • Hypertension • Lower baseline hemoglobin • Acute Chest Syndrome with Aplastic crisis , secondary to parvovirus infection(children) • The presence of MoyaMoya increases risk despite transfusion therapy (58% vs. 28%)

  11. Risk Factors • In adults • Smoking • Hypertension • Diabetes • Obesity • Elevated cholesterol

  12. Risk Factors for Hemorrhagic Stroke • Low hemoglobin • High leukocyte count • Renal disease • Hypertension • Coagulopathy

  13. Primary Stroke Prevention • TCD is effective in detecting children with SCD at high risk of stroke • Transfusion to achieve a Hb S <30% • 90% relative risk reduction • Not validated in adults N Engl J Med 2005;353:2769-78.

  14. Non-contrast head CT Stroke labs: ESR, RPR/VDRL, fasting lipid profile, fasting blood glucose, CRP, PT, PTT, CBC MRI MRA brain and neck Carotid Duplex TTE TEE/TCD Bubble Hypercoagulable work-up: protein C, protein S, antithrombin III, factor V Leiden, prothrombin gene mutation, dilute Russell viper venom, kaolin clotting time, anticardiolipin antibody, B2-glycoprotein antibody assay Angiogram Acute Stroke Evaluation

  15. Acute Stroke Evaluation • There is risk with administration of hyperosmolar IV contrast • It is recommended that patients be treated with exchange transfusion to a Hb S level less than 30% prior to contrast studies

  16. Treatment of Acute Stroke • Exchange Transfusion (Class IIb; level C) • IV tPA • Antithrombotic therapy (Class IIa; level B) • Aspirin • Clopidogrel • Ticlopidine • Aspirin/Dipyridamole

  17. SCD specific treatments • Transfusion to keep Hb SS less than 30% • Reduced recurrence rate to 2.1 episodes per 100 person-years • If transfusion is stopped, 70% have a stroke within 12 months • Hydroxyurea after 6 months of transfusion • Reduced recurrence rate to 4.6 per 100 person-years • Other studies are conflicting

  18. SCD Specific treatments • Hematopoietic Stem cell Transplantation (HSCT) • Shows promise in a few small studies • Encephaloduromyoarteriosynangiosis • May be useful in MoyaMoya

  19. 21 year old young woman with sickle cell disease • Previous strokes in 2002 and 2010 • Under treatment with erithrocytapheresis every 4-6 weeks • Has a mild aphasia at baseline from her stroke in 2010 • In preparation for bone marrow transplant an MRI revealed an acute stroke

  20. Started on aspirin 81mg daily • Concern about risk of bleeding with BMT • Statin considered but not started at this time • Underlying mechanism not likely to be atherosclerosis

  21. Conclusion • Stroke is a significant problem for patients with sickle cell disease • It affects disproportionately an African American population • It has been better studied in children than in adults • The management of these patients should be a collaborative effort between stroke neurologists and hematologists

  22. Acknowledgement John J. Strouse, MD, PhD Sophie Lanzkron, MD MHS

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