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Neurological Manifestations of Sickle Cell Disease and Their Impact on Allogeneic Hematopoietic Stem Cell Transplantation

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Sickle Cell Disease and Hematopoietic Stem Cell Transplantation

Abstract

While sickle cell disease (SCD) affects all organ systems, the brain has long been recognized as a major site of SCD-related morbidity. In fact, children with sickle cell anemia (SCA) historically have had higher stroke risk than any other group of children outside of the perinatal period. Overt strokes are widely accepted as an indication for curative allogeneic hematopoietic stem cell transplantation (HSCT). As we learn more about the broad spectrum of neurological disease due to SCA, from the well-described ischemic strokes and intracranial hemorrhage to the less understood neurocognitive deficits, the indications for and outcomes of allogeneic HSCT are in flux. In this chapter, we review the spectrum of neurological disease associated with SCA and the impact of pre-existing neurological conditions on HSCT complications and outcomes. We identify knowledge gaps and highlight recent developments. We also suggest a standardized clinical approach for assessing neurological complications of SCA before, during, and after HSCT.

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Abbreviations

AED:

Antiepileptic drug

ASCIEs:

Acute silent cerebral ischemic events

CNS:

Central nervous system

CSSCD:

Cooperative Study of Sickle Cell Disease

CSVT:

Cerebral sinovenous thrombosis

CT:

Computed tomography

EDAS:

Encephaloduroarteriosynangiosis

EEG:

Electroencephalogram

FLAIR:

Fluid-attenuated inversion recovery

GVHD:

Graft-versus-host disease

Hb:

Hemoglobin

HbS:

Hemoglobin S

HRQL:

Health-related quality of life

HSCT:

Hematopoietic stem cell transplantation

ICH:

Intracranial hemorrhage

IQ:

Intelligence quotient

MRA:

Magnetic resonance angiogram

MRI:

Magnetic resonance imaging

PRBC:

Packed red blood cell

PRES:

Posterior reversible encephalopathy syndrome

PSOM:

Pediatric Stroke Outcome Measure

SCA:

Sickle cell anemia—includes HbSS and HbS-β0 thalassemia

SCD:

Sickle cell disease—includes all sickle hemoglobinopathies

SCI:

Silent cerebral infarction

SCURT:

Sickle Cell Unrelated Transplant Trial

SIT:

Silent Infarct Transfusion Trial

SWiTCH:

Stroke With Transfusions Changing to Hydroxyurea

TCD:

Transcranial Doppler

TWiTCH:

TCD With Transfusions Changing to Hydroxyurea

US:

United States

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Authors and Affiliations

  1. Department of Neurology, Children’s National Medical Center, Washington, DC, USA

    Jessica L. Carpenter

  2. Division of Pediatric Hematology/Oncology, Washington University in St. Louis, St. Louis, MO, USA

    Monica L. Hulbert

Authors
  1. Jessica L. Carpenter
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  2. Monica L. Hulbert
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Correspondence to Monica L. Hulbert .

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Editors and Affiliations

  1. Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana, USA

    Emily Riehm Meier

  2. Division of Blood and Marrow Transplantation, Children’s National Medical Center, Washington, DC, USA

    Allistair Abraham

  3. Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA

    Ross M. Fasano

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Carpenter, J.L., Hulbert, M.L. (2018). Neurological Manifestations of Sickle Cell Disease and Their Impact on Allogeneic Hematopoietic Stem Cell Transplantation. In: Meier, E., Abraham, A., Fasano, R. (eds) Sickle Cell Disease and Hematopoietic Stem Cell Transplantation . Springer, Cham. https://doi.org/10.1007/978-3-319-62328-3_6

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  • DOI: https://doi.org/10.1007/978-3-319-62328-3_6

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