title: How I treat and manage strokes in sickle cell disease.
abstract: Neurologic complications are a major cause of morbidity and mortality in sickle cell disease (SCD). In children with sickle cell anemia, routine use of transcranial Doppler screening, coupled with regular blood transfusion therapy, has decreased the prevalence of overt stroke from ∼11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with SCD and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurologic deficit includes evaluation by a multidisciplinary team (a hematologist, neurologist, neuroradiologist, and transfusion medicine specialist); prompt neuro-imaging and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion) is recommended if the hemoglobin is >4 gm/dL and <10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in SCD.
authors.affiliation: Department of Hematology/Oncology, Vanderbilt University School of Medicine, Nashville, TN;
authors.forename: Adetola A
authors.initials: AA
authors.lastname: Kassim
authors.affiliation: Department of Hematology and Blood Transfusion, Bayero University/Aminu Kano Teaching Hospital, Kano, Nigeria;
authors.forename: Najibah A
authors.identifier: http://orcid.org/0000-0002-6853-5023
authors.initials: NA
authors.lastname: Galadanci
authors.affiliation: Department of Radiology, and.
authors.forename: Sumit
authors.initials: S
authors.lastname: Pruthi
authors.affiliation: Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN.
authors.forename: Michael R
authors.identifier: http://orcid.org/0000-0002-0574-1604
authors.initials: MR
authors.lastname: DeBaun
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D059906:Neuroimaging; D020521:Stroke / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*
references.citation: South Med J. 1993 Apr;86(4):377-9
references.pmid: 8465211
references.citation: Arch Intern Med. 1984 Mar;144(3):607-8
references.pmid: 6703831
references.citation: Blood. 2011 Jan 20;117(3):772-9
references.pmid: 20940417
references.citation: Eur J Haematol. 2010 Mar;84(3):259-65
references.pmid: 19912310
references.citation: JAMA. 2014 Sep 10;312(10):1033-48
references.pmid: 25203083
references.citation: N Engl J Med. 2000 Jun 22;342(25):1855-65
references.pmid: 10861320
references.citation: J Pediatr Hematol Oncol. 2005 Aug;27(8):420-4
references.pmid: 16096523
references.citation: J Assoc Acad Minor Phys. 1990;1(3):79-82
references.pmid: 2136621
references.citation: Am J Hematol. 2002 Nov;71(3):161-5
references.pmid: 12410569
references.citation: Stroke. 2011 Apr;42(4):1158-92
references.pmid: 21293023
references.citation: J Pediatr. 2006 Nov;149(5):710-2
references.pmid: 17095350
references.citation: J Child Neurol. 1999 May;14(5):277-81
references.pmid: 10342593
references.citation: Radiology. 2001 Jun;219(3):756-65
references.pmid: 11376265
references.citation: Stroke. 2009 Jun;40(6):2276-93
references.pmid: 19423857
references.citation: Pediatr Blood Cancer. 2010 Mar;54(3):461-4
references.pmid: 19813251
references.citation: Neurology. 2000 Apr 25;54(8):1557-61
references.pmid: 10762493
references.citation: J Neurosurg. 1991 Oct;75(4):552-8
references.pmid: 1885973
references.citation: Biol Blood Marrow Transplant. 2010 Feb;16(2):263-72
references.pmid: 19822218
references.citation: Niger Postgrad Med J. 2013 Sep;20(3):181-7
references.pmid: 24287747
references.citation: Lancet. 2005 Jul 30-Aug 5;366(9483):371-7
references.pmid: 16054936
references.citation: Fed Regist. 2007 Apr 9;72(67):17747-81
references.pmid: 17520754
references.citation: Stroke. 2010 Sep;41(9):2108-29
references.pmid: 20651276
references.citation: J Pediatr Hematol Oncol. 2013 Aug;35(6):434-6
references.pmid: 23887022
references.citation: Neurology. 1997 Jul;49(1):113-9
references.pmid: 9222178
references.citation: Bone Marrow Transplant. 1998 Jul;22(1):1-6
references.pmid: 9678788
references.citation: Lancet Neurol. 2006 Sep;5(9):755-68
references.pmid: 16914404
references.citation: Arch Neurol. 2004 Jun;61(6):926-8
references.pmid: 15210532
references.citation: Lancet. 1992 Mar 21;339(8795):699-701
references.pmid: 1347583
references.citation: Pediatr Hematol Oncol. 2012 Apr;29(3):254-60
references.pmid: 22303979
references.citation: Stroke. 2014 Jul;45(7):2160-236
references.pmid: 24788967
references.citation: Neuroradiology. 1979 Oct 31;18(4):185-92
references.pmid: 530429
references.citation: Neuroradiology. 2004 Aug;46(8):635-41
references.pmid: 15185055
references.citation: Am J Hematol. 2011 Apr;86(4):357-61
references.pmid: 21442640
references.citation: Lancet. 2003 Jan 11;361(9352):136
references.pmid: 12531581
references.citation: AJR Am J Roentgenol. 1999 Feb;172(2):537-9
references.pmid: 9930819
references.citation: Brain. 2007 Feb;130(Pt 2):346-56
references.pmid: 17142831
references.citation: Am J Dis Child. 1990 May;144(5):565-9
references.pmid: 2330923
references.citation: Lancet. 1978 Dec 2;2(8101):1207
references.pmid: 82175
references.citation: Ann Neurol. 2005 Aug;58(2):290-302
references.pmid: 16049936
references.citation: J Pediatr. 1991 Mar;118(3):377-82
references.pmid: 1999776
references.citation: Cell. 1996 Nov 1;87(3):543-52
references.pmid: 8898206
references.citation: J Neurosurg. 1991 Sep;75(3):356-63
references.pmid: 1869933
references.citation: AJNR Am J Neuroradiol. 2000 Sep;21(8):1434-40
references.pmid: 11003275
references.citation: Ann Neurol. 1997 Nov;42(5):699-704
references.pmid: 9392568
references.citation: Am J Med. 1978 Sep;65(3):461-71
references.pmid: 717414
references.citation: Ann Trop Paediatr. 1984 Mar;4(1):41-3
references.pmid: 6203473
references.citation: Stroke. 1994 Aug;25(8):1688-92
references.pmid: 8042222
references.citation: J Pediatr. 2002 Mar;140(3):348-54
references.pmid: 11953734
references.citation: Haematologica. 2013 Jan;98(1):129-35
references.pmid: 22875626
references.citation: Blood. 2012 Apr 26;119(17):3925-32
references.pmid: 22318199
references.citation: J Neurol Sci. 1994 May;123(1-2):6-10
references.pmid: 8064323
references.citation: Biol Blood Marrow Transplant. 2013 May;19(5):820-30
references.pmid: 23416852
references.citation: Blood. 2005 Aug 15;106(4):1460-5
references.pmid: 15860670
references.citation: Bone Marrow Transplant. 2005 Feb;35(4):345-52
references.pmid: 15592491
references.citation: Neuroradiology. 1981;22(2):61-5
references.pmid: 7301107
references.citation: Neuroradiology. 1994;36(2):155-6
references.pmid: 8183461
references.citation: Am J Hematol. 2014 Mar;89(3):267-72
references.pmid: 24779035
references.citation: Am J Dis Child. 1987 Jun;141(6):612-5
references.pmid: 3321974
references.citation: Blood. 2002 May 1;99(9):3144-50
references.pmid: 11964276
references.citation: Blood. 2000 Feb 15;95(4):1229-36
references.pmid: 10666195
references.citation: Case Rep Hematol. 2013;2013:516705
references.pmid: 23956892
references.citation: Biol Blood Marrow Transplant. 2012 Aug;18(8):1265-72
references.pmid: 22343376
references.citation: Blood. 2007 Oct 1;110(7):2749-56
references.pmid: 17606762
references.citation: J Pediatr. 1995 Jun;126(6):896-9
references.pmid: 7776091
references.citation: Blood. 2003 Jan 15;101(2):415-9
references.pmid: 12393443
references.citation: N Engl J Med. 2014 Aug 21;371(8):699-710
references.pmid: 25140956
references.citation: Acta Radiol. 2013 Dec;54(10):1182-90
references.pmid: 23858507
references.citation: Stroke. 2008 Jun;39(6):1898-900
references.pmid: 18420957
references.citation: Stroke. 2004 Feb;35(2):502-6
references.pmid: 14739410
references.citation: J Lab Clin Med. 1993 Jul;122(1):48-54
references.pmid: 8320490
references.citation: J Pediatr. 1992 Jun;120(6):882-5
references.pmid: 1593347
references.citation: Blood. 1997 Feb 1;89(3):739-61
references.pmid: 9028304
references.citation: Blood Cells Mol Dis. 2014 Sep;53(3):164-7
references.pmid: 24846580
references.citation: Hematology Am Soc Hematol Educ Program. 2011;2011:427-33
references.pmid: 22160069
references.citation: J Am Acad Nurse Pract. 2005 Jul;17(7):277-82
references.pmid: 15982248
references.citation: Stroke. 2005 Mar;36(3):567-77
references.pmid: 15692123
references.citation: Blood. 2012 Nov 22;120(22):4285-91
references.pmid: 22955919
references.citation: Clin Neuroradiol. 2016 Jun;26(2):209-13
references.pmid: 25293448
references.citation: J Pediatr. 1980 Feb;96(2):205-8
references.pmid: 7351580
references.citation: Pediatr Blood Cancer. 2010 Dec 15;55(7):1338-42
references.pmid: 20981690
references.citation: Blood. 1998 Jan 1;91(1):288-94
references.pmid: 9414296
references.citation: Dev Med Child Neurol. 2015 Feb;57(2):187-93
references.pmid: 25174812
references.citation: Am J Hematol. 2014 Jul;89(7):678-83
references.pmid: 24664847
references.citation: Am J Hematol. 2011 Oct;86(10):846-50
references.pmid: 21898530
references.citation: J Pediatr. 2010 Apr;156(4):645-50
references.pmid: 20022343
references.citation: Pediatrics. 2006 Nov;118(5):1916-24
references.pmid: 17079562
references.citation: Pediatr Blood Cancer. 2008 Nov;51(5):629-33
references.pmid: 18661498
references.citation: J Pediatr. 2007 Jul;151(1):67-72, 72.e1
references.pmid: 17586193
references.citation: N Engl J Med. 1996 Feb 22;334(8):494-500
references.pmid: 8559202